A congenital heart defect characterized by the narrowing or complete absence of the opening between the RIGHT VENTRICLE and the PULMONARY ARTERY. Lacking a normal PULMONARY VALVE, unoxygenated blood in the right ventricle can not be effectively pumped into the lung for oxygenation. Clinical features include rapid breathing, CYANOSIS, right ventricle atrophy, and abnormal heart sounds (HEART MURMURS).
Developmental abnormalities in any portion of the VENTRICULAR SEPTUM resulting in abnormal communications between the two lower chambers of the heart. Classification of ventricular septal defects is based on location of the communication, such as perimembranous, inlet, outlet (infundibular), central muscular, marginal muscular, or apical muscular defect.
A valve situated at the entrance to the pulmonary trunk from the right ventricle.
Progressive destruction or the absence of all or part of the extrahepatic BILE DUCTS, resulting in the complete obstruction of BILE flow. Usually, biliary atresia is found in infants and accounts for one third of the neonatal cholestatic JAUNDICE.
The degeneration and resorption of an OVARIAN FOLLICLE before it reaches maturity and ruptures.
Congenital obliteration of the lumen of the intestine, with the ILEUM involved in 50% of the cases and the JEJUNUM and DUODENUM following in frequency. It is the most frequent cause of INTESTINAL OBSTRUCTION in NEWBORNS. (From Stedman, 25th ed)
Congenital abnormality characterized by the lack of full development of the ESOPHAGUS that commonly occurs with TRACHEOESOPHAGEAL FISTULA. Symptoms include excessive SALIVATION; GAGGING; CYANOSIS; and DYSPNEA.
A combination of congenital heart defects consisting of four key features including VENTRICULAR SEPTAL DEFECTS; PULMONARY STENOSIS; RIGHT VENTRICULAR HYPERTROPHY; and a dextro-positioned AORTA. In this condition, blood from both ventricles (oxygen-rich and oxygen-poor) is pumped into the body often causing CYANOSIS.
The pathologic narrowing of the orifice of the PULMONARY VALVE. This lesion restricts blood outflow from the RIGHT VENTRICLE to the PULMONARY ARTERY. When the trileaflet valve is fused into an imperforate membrane, the blockage is complete.
The short wide vessel arising from the conus arteriosus of the right ventricle and conveying unaerated blood to the lungs.
This structure includes the thin muscular atrial septum between the two HEART ATRIA, and the thick muscular ventricular septum between the two HEART VENTRICLES.
Developmental abnormalities involving structures of the heart. These defects are present at birth but may be discovered later in life.
The muscular structure separating the right and the left lower chambers (HEART VENTRICLES) of the heart. The ventricular septum consists of a very small membranous portion just beneath the AORTIC VALVE, and a large thick muscular portion consisting of three sections including the inlet septum, the trabecular septum, and the outlet septum.
'Abnormalities, Multiple' is a broad term referring to the presence of two or more structural or functional anomalies in an individual, which may be genetic or environmental in origin, and can affect various systems and organs of the body.
An infant during the first month after birth.
The valve consisting of three cusps situated between the right atrium and right ventricle of the heart.
Abnormalities in any part of the HEART SEPTUM resulting in abnormal communication between the left and the right chambers of the heart. The abnormal blood flow inside the heart may be caused by defects in the ATRIAL SEPTUM, the VENTRICULAR SEPTUM, or both.
Maintenance of blood flow to an organ despite obstruction of a principal vessel. Blood flow is maintained through small vessels.
A congenital abnormality that is characterized by a blocked CHOANAE, the opening between the nose and the NASOPHARYNX. Blockage can be unilateral or bilateral; bony or membranous.
A congenital heart defect characterized by downward or apical displacement of the TRICUSPID VALVE, usually with the septal and posterior leaflets being attached to the wall of the RIGHT VENTRICLE. It is characterized by a huge RIGHT ATRIUM and a small and less effective right ventricle.
Pathophysiological conditions of the FETUS in the UTERUS. Some fetal diseases may be treated with FETAL THERAPIES.
Absence of the orifice between the RIGHT ATRIUM and RIGHT VENTRICLE, with the presence of an atrial defect through which all the systemic venous return reaches the left heart. As a result, there is left ventricular hypertrophy (HYPERTROPHY, LEFT VENTRICULAR) because the right ventricle is absent or not functional.
A fetal blood vessel connecting the pulmonary artery with the descending aorta.
Motion pictures of the passage of contrast medium through blood vessels.
Use or insertion of a tubular device into a duct, blood vessel, hollow organ, or body cavity for injecting or withdrawing fluids for diagnostic or therapeutic purposes. It differs from INTUBATION in that the tube here is used to restore or maintain patency in obstructions.
Abnormal passage between the ESOPHAGUS and the TRACHEA, acquired or congenital, often associated with ESOPHAGEAL ATRESIA.
A congenital anomaly caused by the failed development of TRUNCUS ARTERIOSUS into separate AORTA and PULMONARY ARTERY. It is characterized by a single arterial trunk that forms the outlet for both HEART VENTRICLES and gives rise to the systemic, pulmonary, and coronary arteries. It is always accompanied by a ventricular septal defect.
Malformations of CORONARY VESSELS, either arteries or veins. Included are anomalous origins of coronary arteries; ARTERIOVENOUS FISTULA; CORONARY ANEURYSM; MYOCARDIAL BRIDGING; and others.
The arterial trunk arising from the fetal heart. During development, it divides into AORTA and the PULMONARY ARTERY.
The visualization of tissues during pregnancy through recording of the echoes of ultrasonic waves directed into the body. The procedure may be applied with reference to the mother or the fetus and with reference to organs or the detection of maternal or fetal disease.
The circulation of the BLOOD through the LUNGS.
A developmental abnormality in which the spiral (aortopulmonary) septum failed to completely divide the TRUNCUS ARTERIOSUS into ASCENDING AORTA and PULMONARY ARTERY. This abnormal communication between the two major vessels usually lies above their respective valves (AORTIC VALVE; PULMONARY VALVE).
A procedure in which total right atrial or total caval blood flow is channeled directly into the pulmonary artery or into a small right ventricle that serves only as a conduit. The principal congenital malformations for which this operation is useful are TRICUSPID ATRESIA and single ventricle with pulmonary stenosis.
The lower right and left chambers of the heart. The right ventricle pumps venous BLOOD into the LUNGS and the left ventricle pumps oxygenated blood into the systemic arterial circulation.
A congenital cardiovascular malformation in which the AORTA arises entirely from the RIGHT VENTRICLE, and the PULMONARY ARTERY arises from the LEFT VENTRICLE. Consequently, the pulmonary and the systemic circulations are parallel and not sequential, so that the venous return from the peripheral circulation is re-circulated by the right ventricle via aorta to the systemic circulation without being oxygenated in the lungs. This is a potentially lethal form of heart disease in newborns and infants.
Backflow of blood from the RIGHT VENTRICLE into the RIGHT ATRIUM due to imperfect closure of the TRICUSPID VALVE.
A specific pair of GROUP G CHROMOSOMES of the human chromosome classification.
Procedures in which placement of CARDIAC CATHETERS is performed for therapeutic or diagnostic procedures.
Surgery performed on the heart.
The heart of the fetus of any viviparous animal. It refers to the heart in the postembryonic period and is differentiated from the embryonic heart (HEART/embryology) only on the basis of time.
A congenital heart defect characterized by the persistent opening of fetal DUCTUS ARTERIOSUS that connects the PULMONARY ARTERY to the descending aorta (AORTA, DESCENDING) allowing unoxygenated blood to bypass the lung and flow to the PLACENTA. Normally, the ductus is closed shortly after birth.
A congenital abnormality characterized by the persistence of the anal membrane, resulting in a thin membrane covering the normal ANAL CANAL. Imperforation is not always complete and is treated by surgery in infancy. This defect is often associated with NEURAL TUBE DEFECTS; MENTAL RETARDATION; and DOWN SYNDROME.
Ultrasonic recording of the size, motion, and composition of the heart and surrounding tissues. The standard approach is transthoracic.
Surgical union or shunt between ducts, tubes or vessels. It may be end-to-end, end-to-side, side-to-end, or side-to-side.
Radiographic visualization of the aorta and its branches by injection of contrast media, using percutaneous puncture or catheterization procedures.
Radiography of blood vessels after injection of a contrast medium.
Care alleviating symptoms without curing the underlying disease. (Stedman, 25th ed)
The portion of the descending aorta proceeding from the arch of the aorta and extending to the DIAPHRAGM, eventually connecting to the ABDOMINAL AORTA.
The status during which female mammals carry their developing young (EMBRYOS or FETUSES) in utero before birth, beginning from FERTILIZATION to BIRTH.
Evaluation undertaken to assess the results or consequences of management and procedures used in combating disease in order to determine the efficacy, effectiveness, safety, and practicability of these interventions in individual cases or series.
Studies in which individuals or populations are followed to assess the outcome of exposures, procedures, or effects of a characteristic, e.g., occurrence of disease.

Coanda effect on ductal flow in the pulmonary artery. (1/120)

The Coanda effect (the tendency of a jet stream to adhere to a boundary wall), and the relevant anatomy, may explain the location of ductal jets within the main pulmonary artery. With the usual insertion of the duct close to the left pulmonary artery, during right ventricular ejection, the ductal jet adheres to the left wall of the main pulmonary artery. When right ventricular ejection is absent in pulmonary atresia, the ductal jet streams down the right wall of the pulmonary artery to the pulmonary valve, reverses, and maintains a parallel column back toward the bifurcation. If the reversed flow is mistaken for ejection from the right ventricle, the diagnosis of pulmonary atresia may be missed.  (+info)

Prenatal diagnosis of right ventricular outflow tract obstruction with intact ventricular septum, and detection of ventriculocoronary connections. (2/120)

OBJECTIVES: To determine the accuracy of prenatal diagnosis of pulmonary atresia and intact ventricular septum (PAIVS), and pulmonary stenosis, including prenatal detection of ventriculocoronary connections, to evaluate heart size during the prenatal period, and to evaluate the outcome. DESIGN AND PATIENTS: Medical records of 20 cases with prenatally diagnosed PAIVS and pulmonary stenosis were reviewed retrospectively. Prenatal and postnatal echocardiography were also reviewed and dimensions of the ventricles and vessels were measured retrespectively. RESULTS: Of 20 prenatal diagnoses (15 PAIVS and five pulmonary stenosis), 16 were confirmed as correct. One critical pulmonary stenosis case had been diagnosed as PAIVS prenatally; three had no confirmation. Eight pregnancies were terminated, three had no active treatment, and nine were treated; all survived. Of 13 assessed with ventriculocoronary connections prenatally, seven were diagnosed correctly (four with, three without ventriculocoronary connections), but one was falsely positive; five had no confirmation. The more prominent hypoplasia of the main pulmonary artery and the tricuspid valve annulus, and the sigmoid shape of the ductus arteriosus, seemed to be associated with the presence of ventriculocoronary connections. CONCLUSIONS: Current prenatal echocardiography can accurately diagnose right ventricular outflow tract obstruction and ventriculocoronary connections. Prenatal detection of this constellation of abnormalities aids in family counselling and decisions on postnatal management.  (+info)

Persistent pulmonary hypertension of the newborn associated with pulmonary atresia and intact interventricular septum. (3/120)

Neonates with pulmonary atresia and intact interventricular septum (PAIVS) do not have pulmonary vascular disease secondary to their heart abnormality. Persistent pulmonary hypertension of the newborn has not been described in association with this condition. The case is reported of a female neonate born with PAIVS, who preoperatively had no clinical evidence or any risk factors for persistent pulmonary hypertension of the newborn, but whose postoperative course was highly suggestive of persistent pulmonary hypertension; necropsy confirmed the features of pulmonary vascular disease.  (+info)

Pulmonary ventricular outflow reconstruction with a size-reduced cryopreserved pulmonary valve allograft: mid-term follow-up. (4/120)

Surgical reduction of pulmonary allografts is being performed because of the shortage of allografts of suitable size for pediatric use. However, the outcome of size-reduced pulmonary allografts for pulmonary conduits is unknown. In the present study, cryopreserved pulmonary allografts harvested from adults at the time of kidney donation were size-reduced and used in 4 children, 2 with pulmonary atresia and ventricular septal defect and 2 with atrioventricular discordance, pulmonary atresia and ventricular septal defect. They all had undergone right and/or left modified Blalock-Taussig shunt operations with a 5-mm synthetic graft prior to the reparative operations. They underwent definitive repair with a size-reduced cryopreserved pulmonary allograft valved conduit and were followed up for 2-5 years. Postoperative echocardiographic and cineangiographic assessments revealed excellent function of the pulmonary bicuspidalized valves with a minimal pressure gradient and no, or only trivial, regurgitation. Although the long-term result of a cryopreserved bicuspid pulmonary valved conduit remains unknown, the remodeled bicuspid pulmonary allograft conduits showed excellent hemodynamic characteristics in mid-term follow-up and appear to be a reasonable alternative to other types of conduits when an appropriate-sized allograft is not available.  (+info)

Pulmonary atresia with intact ventricular septum percutaneous radiofrequency-assisted valvotomy and balloon dilation versus surgical valvotomy and Blalock Taussig shunt. (5/120)

OBJECTIVE: We compared the result of radiofrequency (RF)-assisted valvotomy and balloon dilation with closed surgical valvotomy and Blalock Taussig (BT) shunt as primary treatment in selected patients with pulmonary atresia and intact ventricular septum (PA-IVS). BACKGROUND: Patients with PA-IVS who have mild to moderate hypoplasia of the right ventricle (RV) and patent infundibulum have the greatest potential for complete biventricular circulation. The use of RF or laser wires to perforate the atretic valve followed by balloon dilation provides an alternative to surgery. METHODS: Between May 1990 and March 1998, 33 selected patients underwent either percutaneous RF valvotomy and balloon dilation (group 1, n = 21; two crossed over to group 2) or surgical valvotomy with concomitant BT shunt (group 2, n = 14). Second RV decompression by balloon dilation or right ventricular outflow tract (RVOT) reconstruction were performed if necessary. Patients who remained cyanosed were subjected to transcatheter trial closure of the interatrial communication. Partial biventricular repair was offered to those with inadequate growth of the RV. RESULTS: The primary procedure was successful in 19 patients in group 1. There was one in-hospital death and two late deaths. Of the remaining 16 survivors, 12 achieved complete biventricular circulation, 7 of whom required no further interventions. Two patients required repeat balloon dilation, 1 RVOT reconstruction and 2 transcatheter closure of interatrial communication. Two patients underwent partial biventricular repair. In group 2, there were 3 in-hospital deaths after the primary procedure and 1 patient died four months later. All survivors (n = 10) required a second RV decompression, 8 by balloon dilation and 2 by RVOT reconstruction, after which, two patients died. Of the final 8 survivors, 7 achieved complete biventricular circulation, 5 after coil occlusion of the BT shunt and 2 after closure of interatrial communication. CONCLUSIONS: Radiofrequency valvotomy and balloon dilation is more efficacious and safe compared with closed pulmonary valvotomy and BT shunt in selected patients with PA-IVS.  (+info)

Effect of Blalock Taussig shunt on clinical parameters, left ventricular function and pulmonary arteries. (6/120)

Twenty children (mean age 3.25 years) with congenital cyanotic heart disease undergoing modified left Blalock-Taussig (BT) shunt were studied. The mean follow-up period was 9.5 months (range 6 months to 1 year). The shunt was performed for cyanotic spells in 15 (75%) and hypoplastic pulmonary arteries in 5 (25%) patients. There were no immediate or late complications. None had cyanotic spell after the shunt. The mean arterial oxygen saturation improved from 66.47 +/- 11.9 to 76.97 +/- 8.16% (p = 0.0003) and mean hematocrit decreased from 51.55 +/- 9.5 to 46.5 +/- 9.7 (p = 0.002) after the shunt. The left atrial systolic volume and left ventricular diastolic volume also increased significantly following the shunt (from 15.82 +/- 6.37 to 20.83 +/- 8.91 ml p = 0.006 and from 36.13 +/- 16.08 to 41.08 +/- 20.07 ml (p = 0.01) respectively. There was significant growth of main, right and left pulmonary arteries and pulmonary valve annulus after the procedure.  (+info)

Early and intermediate outcomes after repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: experience with 85 patients. (7/120)

BACKGROUND: Pulmonary atresia with ventricular septal defect (VSD) and major aortopulmonary collaterals (MAPCAs) is a complex lesion with marked heterogeneity of pulmonary blood supply. Traditional management has involved staged unifocalization of pulmonary blood supply. Our approach has been to perform early 1-stage complete unifocalization in almost all patients. METHODS AND RESULTS: Since 1992, 85 patients with pulmonary atresia, VSD, and MAPCAs have undergone unifocalization (median age, 7 months). Complete 1-stage unifocalization and intracardiac repair were performed through a midline approach in 56 patients, whereas 23 underwent unifocalization in a single stage with the VSD left open, and 6 underwent staged unifocalization through sequential thoracotomies. There were 9 early deaths. During follow-up (1 to 69 months), there were 7 late deaths. Actuarial survival was 80% at 3 years. Among early survivors, actuarial survival with complete repair was 88% at 2 years. Reintervention on the neo-pulmonary arteries was performed in 24 patients. CONCLUSIONS: Early 1-stage complete unifocalization can be performed in >90% of patients with pulmonary atresia and MAPCAs, even those with absent true pulmonary arteries, and yields good functional results. Complete repair during the same operation is achieved in two thirds of patients. There remains room for improvement; actuarial survival 3 years after surgery is 80%, and there is a significant rate of reintervention. These results must be appreciated within the context of the natural history of this lesion: 65% of patients survive to 1 year of age and slightly >50% survive to 2 years even with surgical intervention.  (+info)

Heat shock protein 72 expression in the right ventricle of patients undergoing congenital cardiac surgery. (8/120)

While heat shock protein (HSP) 72 is known as a stress protein, there have been no reports of HSP 72 expression in patients who have undergone surgery for congenital heart disease. Fourteen patients (7 males and 7 females) who had undergone surgery for congenital heart disease were studied. The ages of the patients ranged from 2 months to 43 years old (mean 6.5 +/- 10.8 years old; median 3.0 years old). The diagnoses were Tetralogy of Fallot in seven, pulmonary atresia with ventricular septal defect (VSD) in three, complex anomalies in three, and VSD in one patient. Histological study and HSP analysis using Western blots and immunostaining with anti-HSP 72 monoclonal antibody were performed for right ventricular muscle samples resected during the surgery. The histological findings showed hypertrophic changes of ventricular cardiomyocytes in all samples studied. Western blots detected HSP 72 expression of various degrees in all specimens. Immunostaining using monoclonal antibody against HSP 72 showed that the protein was present in the nuclei and cytoplasm of cardiomyocytes. In conclusion, although it is difficult to determine the cause of the "stress" that triggers HSP 72 expression in cardiomyocytes, low O2 saturation and pressure overload might act as a "stress", and the only common factor that induced HSP 72 in every sample was hypertrophy.  (+info)

Pulmonary atresia is a congenital heart defect where the pulmonary valve, which controls blood flow from the right ventricle to the lungs, doesn't form properly and instead of being open, there is a membranous obstruction or atresia. This results in an absence of communication between the right ventricle and the pulmonary artery.

The right ventricle is often small and underdeveloped due to this condition, and blood flow to the lungs can be severely limited. In some cases, there may be additional heart defects present, such as a ventricular septal defect (a hole between the two lower chambers of the heart) or patent ductus arteriosus (an abnormal connection between the pulmonary artery and the aorta).

Pulmonary atresia can range from mild to severe, and treatment options depend on the specific anatomy and physiology of each individual case. Treatment may include medications, catheter-based procedures, or open-heart surgery, and in some cases, a heart transplant may be necessary.

A ventricular septal defect (VSD) is a type of congenital heart defect that involves a hole in the wall separating the two lower chambers of the heart, the ventricles. This defect allows oxygenated blood from the left ventricle to mix with deoxygenated blood in the right ventricle, leading to inefficient oxygenation of the body's tissues. The size and location of the hole can vary, and symptoms may range from none to severe, depending on the size of the defect and the amount of blood that is able to shunt between the ventricles. Small VSDs may close on their own over time, while larger defects usually require medical intervention, such as medication or surgery, to prevent complications like pulmonary hypertension and heart failure.

The pulmonary valve, also known as the pulmonic valve, is a semilunar valve located at the exit of the right ventricle of the heart and the beginning of the pulmonary artery. It has three cusps or leaflets that prevent the backflow of blood from the pulmonary artery into the right ventricle during ventricular diastole, ensuring unidirectional flow of blood towards the lungs for oxygenation.

Biliary atresia is a rare, progressive liver disease in infants and children, characterized by the inflammation, fibrosis, and obstruction of the bile ducts. This results in the impaired flow of bile from the liver to the intestine, leading to cholestasis (accumulation of bile in the liver), jaundice (yellowing of the skin and eyes), and eventually liver cirrhosis and failure if left untreated.

The exact cause of biliary atresia is not known, but it is believed to be a combination of genetic and environmental factors. It can occur as an isolated condition or in association with other congenital anomalies. The diagnosis of biliary atresia is typically made through imaging studies, such as ultrasound and cholangiography, and confirmed by liver biopsy.

The standard treatment for biliary atresia is a surgical procedure called the Kasai portoenterostomy, which aims to restore bile flow from the liver to the intestine. In this procedure, the damaged bile ducts are removed and replaced with a loop of intestine that is connected directly to the liver. The success of the Kasai procedure depends on several factors, including the age at diagnosis and surgery, the extent of liver damage, and the skill and experience of the surgeon.

Despite successful Kasai surgery, many children with biliary atresia will eventually develop cirrhosis and require liver transplantation. The prognosis for children with biliary atresia has improved significantly over the past few decades due to earlier diagnosis, advances in surgical techniques, and better postoperative care. However, it remains a challenging condition that requires close monitoring and multidisciplinary management by pediatric hepatologists, surgeons, and other healthcare professionals.

Follicular atresia is a physiological process that occurs in the ovary, where follicles (fluid-filled sacs containing immature eggs or oocytes) undergo degeneration and disappearance. This process begins after the primordial follicle stage and continues throughout a woman's reproductive years. At birth, a female has approximately 1 to 2 million primordial follicles, but only about 400 of these will mature and release an egg during her lifetime. The rest undergo atresia, which is a natural process that helps regulate the number of available eggs and maintain hormonal balance within the body.

The exact mechanisms that trigger follicular atresia are not fully understood, but it is believed to be influenced by various factors such as hormonal imbalances, oxidative stress, and apoptosis (programmed cell death). In some cases, accelerated or excessive follicular atresia can lead to infertility or early menopause.

Intestinal atresia is a congenital condition characterized by the absence or complete closure of a portion of the intestine, preventing the passage of digested food from the stomach to the remaining part of the intestines. This results in a blockage in the digestive system, which can be life-threatening if not treated promptly after birth. The condition can occur anywhere along the small or large intestine and may affect either a single segment or multiple segments of the intestine.

There are several types of intestinal atresia, including:

1. Jejunal atresia: A closure or absence in the jejunum, a part of the small intestine located between the duodenum and ileum.
2. Ileal atresia: A closure or absence in the ileum, the lower portion of the small intestine that connects to the large intestine (cecum).
3. Colonic atresia: A closure or absence in the colon, a part of the large intestine responsible for storing and eliminating waste.
4. Duodenal atresia: A closure or absence in the duodenum, the uppermost portion of the small intestine that receives chyme (partially digested food) from the stomach.
5. Multiple atresias: When more than one segment of the intestines is affected by atresia.

The exact cause of intestinal atresia remains unclear, but it is believed to be related to disruptions in fetal development during pregnancy. Treatment typically involves surgical correction to reconnect the affected segments of the intestine and restore normal digestive function. The prognosis for infants with intestinal atresia depends on the severity and location of the atresia, as well as any associated conditions or complications.

Esophageal atresia is a congenital condition in which the esophagus, the tube that connects the throat to the stomach, does not develop properly. In most cases, the upper esophagus ends in a pouch instead of connecting to the lower esophagus and stomach. This condition prevents food and liquids from reaching the stomach, leading to difficulty swallowing and feeding problems in newborn infants. Esophageal atresia often occurs together with a congenital defect called tracheoesophageal fistula, in which there is an abnormal connection between the esophagus and the windpipe (trachea).

The medical definition of 'Esophageal Atresia' is:

A congenital anomaly characterized by the absence of a normal connection between the upper esophagus and the stomach, resulting in the separation of the proximal and distal esophageal segments. The proximal segment usually ends in a blind pouch, while the distal segment may communicate with the trachea through a tracheoesophageal fistula. Esophageal atresia is often associated with other congenital anomalies and can cause serious complications if not diagnosed and treated promptly after birth.

Tetralogy of Fallot is a congenital heart defect that consists of four components: ventricular septal defect (a hole between the lower chambers of the heart), pulmonary stenosis (narrowing of the pulmonary valve and outflow tract), overriding aorta (the aorta lies directly over the ventricular septal defect), and right ventricular hypertrophy (thickening of the right ventricular muscle). This condition results in insufficient oxygenation of the blood, leading to cyanosis (bluish discoloration of the skin and mucous membranes) and other symptoms such as shortness of breath, fatigue, and poor growth. Treatment typically involves surgical repair, which is usually performed during infancy or early childhood.

Pulmonary Valve Stenosis is a cardiac condition where the pulmonary valve, located between the right ventricle and the pulmonary artery, has a narrowed opening. This stenosis (narrowing) can cause obstruction of blood flow from the right ventricle to the lungs. The narrowing can be caused by a fusion of the valve leaflets, thickened or calcified valve leaflets, or rarely, a dysplastic valve.

The severity of Pulmonary Valve Stenosis is classified based on the gradient pressure across the valve, which is measured during an echocardiogram. A mild stenosis has a gradient of less than 30 mmHg, moderate stenosis has a gradient between 30-59 mmHg, and severe stenosis has a gradient of 60 mmHg or higher.

Mild Pulmonary Valve Stenosis may not require treatment, while more severe cases may need to be treated with balloon valvuloplasty or surgical valve replacement. If left untreated, Pulmonary Valve Stenosis can lead to right ventricular hypertrophy, heart failure, and other complications.

The pulmonary artery is a large blood vessel that carries deoxygenated blood from the right ventricle of the heart to the lungs for oxygenation. It divides into two main branches, the right and left pulmonary arteries, which further divide into smaller vessels called arterioles, and then into a vast network of capillaries in the lungs where gas exchange occurs. The thin walls of these capillaries allow oxygen to diffuse into the blood and carbon dioxide to diffuse out, making the blood oxygen-rich before it is pumped back to the left side of the heart through the pulmonary veins. This process is crucial for maintaining proper oxygenation of the body's tissues and organs.

The heart septum is the thick, muscular wall that divides the right and left sides of the heart. It consists of two main parts: the atrial septum, which separates the right and left atria (the upper chambers of the heart), and the ventricular septum, which separates the right and left ventricles (the lower chambers of the heart). A normal heart septum ensures that oxygen-rich blood from the lungs does not mix with oxygen-poor blood from the body. Any defect or abnormality in the heart septum is called a septal defect, which can lead to various congenital heart diseases.

Congenital heart defects (CHDs) are structural abnormalities in the heart that are present at birth. They can affect any part of the heart's structure, including the walls of the heart, the valves inside the heart, and the major blood vessels that lead to and from the heart.

Congenital heart defects can range from mild to severe and can cause various symptoms depending on the type and severity of the defect. Some common symptoms of CHDs include cyanosis (a bluish tint to the skin, lips, and fingernails), shortness of breath, fatigue, poor feeding, and slow growth in infants and children.

There are many different types of congenital heart defects, including:

1. Septal defects: These are holes in the walls that separate the four chambers of the heart. The two most common septal defects are atrial septal defect (ASD) and ventricular septal defect (VSD).
2. Valve abnormalities: These include narrowed or leaky valves, which can affect blood flow through the heart.
3. Obstruction defects: These occur when blood flow is blocked or restricted due to narrowing or absence of a part of the heart's structure. Examples include pulmonary stenosis and coarctation of the aorta.
4. Cyanotic heart defects: These cause a lack of oxygen in the blood, leading to cyanosis. Examples include tetralogy of Fallot and transposition of the great arteries.

The causes of congenital heart defects are not fully understood, but genetic factors and environmental influences during pregnancy may play a role. Some CHDs can be detected before birth through prenatal testing, while others may not be diagnosed until after birth or later in childhood. Treatment for CHDs may include medication, surgery, or other interventions to improve blood flow and oxygenation of the body's tissues.

The ventricular septum is the thick, muscular wall that separates the left and right ventricles, which are the lower chambers of the heart. Its main function is to prevent the oxygen-rich blood in the left ventricle from mixing with the oxygen-poor blood in the right ventricle.

A congenital heart defect called a ventricular septal defect (VSD) can occur when there is an abnormal opening or hole in the ventricular septum, allowing blood to flow between the two ventricles. This can result in various symptoms and complications, depending on the size of the defect and the amount of blood that passes through it. VSDs are typically diagnosed and treated by pediatric cardiologists or cardiac surgeons.

'Abnormalities, Multiple' is a broad term that refers to the presence of two or more structural or functional anomalies in an individual. These abnormalities can be present at birth (congenital) or can develop later in life (acquired). They can affect various organs and systems of the body and can vary greatly in severity and impact on a person's health and well-being.

Multiple abnormalities can occur due to genetic factors, environmental influences, or a combination of both. Chromosomal abnormalities, gene mutations, exposure to teratogens (substances that cause birth defects), and maternal infections during pregnancy are some of the common causes of multiple congenital abnormalities.

Examples of multiple congenital abnormalities include Down syndrome, Turner syndrome, and VATER/VACTERL association. Acquired multiple abnormalities can result from conditions such as trauma, infection, degenerative diseases, or cancer.

The medical evaluation and management of individuals with multiple abnormalities depend on the specific abnormalities present and their impact on the individual's health and functioning. A multidisciplinary team of healthcare professionals is often involved in the care of these individuals to address their complex needs.

A newborn infant is a baby who is within the first 28 days of life. This period is also referred to as the neonatal period. Newborns require specialized care and attention due to their immature bodily systems and increased vulnerability to various health issues. They are closely monitored for signs of well-being, growth, and development during this critical time.

The tricuspid valve is the heart valve that separates the right atrium and the right ventricle in the human heart. It is called "tricuspid" because it has three leaflets or cusps, which are also referred to as flaps or segments. These cusps are named anterior, posterior, and septal. The tricuspid valve's function is to prevent the backflow of blood from the ventricle into the atrium during systole, ensuring unidirectional flow of blood through the heart.

A heart septal defect is a type of congenital heart defect, which means it is present at birth. It involves an abnormal opening in the septum, the wall that separates the two sides of the heart. This opening allows oxygen-rich blood to leak into the oxygen-poor blood chambers in the heart.

There are several types of heart septal defects, including:

1. Atrial Septal Defect (ASD): A hole in the atrial septum, the wall between the two upper chambers of the heart (the right and left atria).
2. Ventricular Septal Defect (VSD): A hole in the ventricular septum, the wall between the two lower chambers of the heart (the right and left ventricles).
3. Atrioventricular Septal Defect (AVSD): A combination of an ASD and a VSD, often accompanied by malformation of the mitral and/or tricuspid valves.

The severity of a heart septal defect depends on the size of the opening and its location in the septum. Small defects may cause no symptoms and may close on their own over time. Larger defects can lead to complications, such as heart failure, pulmonary hypertension, or infective endocarditis, and may require medical or surgical intervention.

Collateral circulation refers to the alternate blood supply routes that bypass an obstructed or narrowed vessel and reconnect with the main vascular system. These collateral vessels can develop over time as a result of the body's natural adaptation to chronic ischemia (reduced blood flow) caused by various conditions such as atherosclerosis, thromboembolism, or vasculitis.

The development of collateral circulation helps maintain adequate blood flow and oxygenation to affected tissues, minimizing the risk of tissue damage and necrosis. In some cases, well-developed collateral circulations can help compensate for significant blockages in major vessels, reducing symptoms and potentially preventing the need for invasive interventions like revascularization procedures. However, the extent and effectiveness of collateral circulation vary from person to person and depend on factors such as age, overall health status, and the presence of comorbidities.

Choanal atresia is a medical condition where the back of the nasal passage (choana) is blocked or narrowed, usually by bone, membrane, or a combination of both. This blockage can be present at birth (congenital) or acquired later in life due to various reasons such as infection, injury, or tumor.

Congenital choanal atresia is more common and occurs during fetal development when the nasal passages fail to open properly. It can affect one or both sides of the nasal passage and can be unilateral (affecting one side) or bilateral (affecting both sides). Bilateral choanal atresia can cause breathing difficulties in newborns, as they are obligate nose breathers and cannot breathe through their mouth yet.

Treatment for choanal atresia typically involves surgical intervention to open up the nasal passage and restore normal breathing. The specific type of surgery may depend on the location and extent of the blockage. In some cases, follow-up surgeries or additional treatments may be necessary to ensure proper functioning of the nasal passage.

Ebstein anomaly is a congenital heart defect that affects the tricuspid valve, which is the valve between the right atrium and right ventricle of the heart. In Ebstein anomaly, the tricuspid valve is abnormally formed and positioned, causing it to leak blood back into the right atrium. This can lead to various symptoms such as shortness of breath, fatigue, and cyanosis (bluish discoloration of the skin). Treatment for Ebstein anomaly may include medication, surgery, or a combination of both. It is important to note that the severity of the condition can vary widely among individuals, and some people with Ebstein anomaly may require more intensive treatment than others.

Fetal diseases are medical conditions or abnormalities that affect a fetus during pregnancy. These diseases can be caused by genetic factors, environmental influences, or a combination of both. They can range from mild to severe and may impact various organ systems in the developing fetus. Examples of fetal diseases include congenital heart defects, neural tube defects, chromosomal abnormalities such as Down syndrome, and infectious diseases such as toxoplasmosis or rubella. Fetal diseases can be diagnosed through prenatal testing, including ultrasound, amniocentesis, and chorionic villus sampling. Treatment options may include medication, surgery, or delivery of the fetus, depending on the nature and severity of the disease.

Tricuspid atresia is a congenital heart defect where the tricuspid valve, which regulates blood flow between the right atrium and right ventricle, fails to develop properly. As a result, there is no direct pathway for blood to move from the right atrium to the right ventricle and then to the lungs for oxygenation.

In this condition, blood from the body returning to the heart enters the right atrium but cannot flow through the tricuspid valve into the right ventricle. Instead, it flows through an opening in the interatrial septum (atrial septal defect) into the left atrium and then into the left ventricle. The left ventricle pumps this blood to the body and a portion of it goes to the lungs via a patent ductus arteriosus or other collateral vessels.

Tricuspid atresia is often associated with other heart defects, such as transposition of the great arteries, pulmonary stenosis, or total anomalous pulmonary venous return. Symptoms can vary depending on the severity and associated defects but may include cyanosis (bluish discoloration of the skin), shortness of breath, fatigue, and poor growth. Treatment typically involves surgical interventions to create a path for blood to flow to the lungs and establish proper oxygenation.

The Ductus Arteriosus is a fetal blood vessel that connects the pulmonary trunk (the artery that carries blood from the heart to the lungs) and the aorta (the largest artery in the body, which carries oxygenated blood from the heart to the rest of the body). This vessel allows most of the blood from the right ventricle of the fetal heart to bypass the lungs, as the fetus receives oxygen through the placenta rather than breathing air.

After birth, with the first breaths, the blood oxygen level increases and the pressure in the lungs rises. As a result, the circulation in the newborn's body changes, and the Ductus Arteriosus is no longer needed. Within the first few days or weeks of life, this vessel usually closes spontaneously, turning into a fibrous cord called the Ligamentum Arteriosum.

Persistent Patency of the Ductus Arteriosus (PDA) occurs when the Ductus Arteriosus does not close after birth, which can lead to various complications such as heart failure and pulmonary hypertension. This condition is often seen in premature infants and may require medical intervention or surgical closure of the vessel.

Cineangiography is a medical imaging technique used to visualize the blood flow in the heart and cardiovascular system. It involves the injection of a contrast agent into the bloodstream while X-ray images are taken in quick succession, creating a movie-like sequence that shows the movement of the contrast through the blood vessels and chambers of the heart. This technique is often used to diagnose and evaluate various heart conditions, such as coronary artery disease, valvular heart disease, and congenital heart defects.

The procedure typically involves threading a catheter through a blood vessel in the arm or leg and guiding it to the heart. Once in place, the contrast agent is injected, and X-ray images are taken using a specialized X-ray machine called a fluoroscope. The images captured during cineangiography can help doctors identify areas of narrowing or blockage in the coronary arteries, abnormalities in heart valves, and other cardiovascular problems.

Cineangiography is an invasive procedure that carries some risks, such as bleeding, infection, and reactions to the contrast agent. However, it can provide valuable information for diagnosing and treating heart conditions, and may be recommended when other diagnostic tests have been inconclusive.

Catheterization is a medical procedure in which a catheter (a flexible tube) is inserted into the body to treat various medical conditions or for diagnostic purposes. The specific definition can vary depending on the area of medicine and the particular procedure being discussed. Here are some common types of catheterization:

1. Urinary catheterization: This involves inserting a catheter through the urethra into the bladder to drain urine. It is often performed to manage urinary retention, monitor urine output in critically ill patients, or assist with surgical procedures.
2. Cardiac catheterization: A procedure where a catheter is inserted into a blood vessel, usually in the groin or arm, and guided to the heart. This allows for various diagnostic tests and treatments, such as measuring pressures within the heart chambers, assessing blood flow, or performing angioplasty and stenting of narrowed coronary arteries.
3. Central venous catheterization: A catheter is inserted into a large vein, typically in the neck, chest, or groin, to administer medications, fluids, or nutrition, or to monitor central venous pressure.
4. Peritoneal dialysis catheterization: A catheter is placed into the abdominal cavity for individuals undergoing peritoneal dialysis, a type of kidney replacement therapy.
5. Neurological catheterization: In some cases, a catheter may be inserted into the cerebrospinal fluid space (lumbar puncture) or the brain's ventricular system (ventriculostomy) to diagnose or treat various neurological conditions.

These are just a few examples of catheterization procedures in medicine. The specific definition and purpose will depend on the medical context and the particular organ or body system involved.

A tracheoesophageal fistula (TEF) is an abnormal connection between the trachea (windpipe) and the esophagus (tube that carries food from the mouth to the stomach). This congenital anomaly is usually present at birth and can vary in size and location. It can cause complications such as respiratory distress, feeding difficulties, and recurrent lung infections. TEF is often treated surgically to separate the trachea and esophagus and restore their normal functions.

Persistent Truncus Arteriosus is a rare congenital heart defect that is characterized by the failure of the truncus arteriosus to divide into the separate pulmonary artery and aorta during fetal development. This results in a single large vessel, the truncus arteriosus, which gives rise to both the systemic and pulmonary circulations.

The truncus arteriosus contains a single semilunar valve, instead of the two separate semilunar valves (pulmonary and aortic) found in a normal heart. Additionally, there is often a ventricular septal defect (VSD), a hole in the wall between the two lower chambers of the heart, present.

This condition leads to mixing of oxygenated and deoxygenated blood within the truncus arteriosus, resulting in cyanosis (bluish discoloration of the skin and mucous membranes) and decreased oxygen delivery to the body. Symptoms typically appear soon after birth and may include difficulty breathing, poor feeding, rapid heart rate, and failure to thrive.

Persistent truncus arteriosus is usually treated with surgical repair in infancy or early childhood to separate the pulmonary and systemic circulations, close the VSD, and reconstruct the great vessels as needed.

Coronary vessel anomalies refer to abnormalities in the structure, origin, or course of the coronary arteries or veins. These vessels are responsible for delivering oxygenated blood to the heart muscle. Some common types of coronary vessel anomalies include:

1. Anomalous Origin of the Coronary Artery (AOCA): This occurs when one or both of the coronary arteries originate from an abnormal location in the aorta. The left coronary artery may arise from the right sinus of Valsalva, while the right coronary artery may arise from the left sinus of Valsalva. This can lead to ischemia (reduced blood flow) and potentially life-threatening complications such as sudden cardiac death.
2. Coronary Artery Fistula: A fistula is an abnormal connection between a coronary artery and another chamber or vessel in the heart. Blood flows directly from the high-pressure coronary artery into a low-pressure chamber, bypassing the capillaries and leading to a steal phenomenon where oxygenated blood is diverted away from the heart muscle.
3. Coronary Artery Aneurysm: An aneurysm is a localized dilation or bulging of the coronary artery wall. This can lead to complications such as thrombosis (blood clot formation), embolism (blockage caused by a clot that travels to another location), or rupture, which can be life-threatening.
4. Myocardial Bridge: In this condition, a segment of the coronary artery passes between the muscle fibers of the heart, instead of running along its surface. This can cause compression of the artery during systole (contraction) and lead to ischemia.
5. Kawasaki Disease: Although not strictly an anomaly, Kawasaki disease is a pediatric illness that can result in coronary artery aneurysms and other complications if left untreated.

Coronary vessel anomalies may be asymptomatic or present with symptoms such as chest pain, shortness of breath, palpitations, or syncope (fainting). Diagnosis typically involves imaging techniques such as coronary angiography, computed tomography (CT) angiography, or magnetic resonance angiography. Treatment depends on the specific anomaly and may involve medications, percutaneous interventions, or surgical correction.

Truncus Arteriosus is a congenital heart defect where a single large vessel arises from the heart, instead of separate pulmonary and aortic trunks. This results in the mixing of oxygenated and deoxygenated blood within the truncus. The truncus then divides into systemic, coronary, and pulmonary arteries. It's usually associated with a ventricular septal defect. This condition is often diagnosed early in life due to cyanosis (bluish discoloration of the skin) and heart murmurs. Surgical correction is required for survival.

Prenatal ultrasonography, also known as obstetric ultrasound, is a medical diagnostic procedure that uses high-frequency sound waves to create images of the developing fetus, placenta, and amniotic fluid inside the uterus. It is a non-invasive and painless test that is widely used during pregnancy to monitor the growth and development of the fetus, detect any potential abnormalities or complications, and determine the due date.

During the procedure, a transducer (a small handheld device) is placed on the mother's abdomen and moved around to capture images from different angles. The sound waves travel through the mother's body and bounce back off the fetus, producing echoes that are then converted into electrical signals and displayed as images on a screen.

Prenatal ultrasonography can be performed at various stages of pregnancy, including early pregnancy to confirm the pregnancy and detect the number of fetuses, mid-pregnancy to assess the growth and development of the fetus, and late pregnancy to evaluate the position of the fetus and determine if it is head down or breech. It can also be used to guide invasive procedures such as amniocentesis or chorionic villus sampling.

Overall, prenatal ultrasonography is a valuable tool in modern obstetrics that helps ensure the health and well-being of both the mother and the developing fetus.

Pulmonary circulation refers to the process of blood flow through the lungs, where blood picks up oxygen and releases carbon dioxide. This is a vital part of the overall circulatory system, which delivers nutrients and oxygen to the body's cells while removing waste products like carbon dioxide.

In pulmonary circulation, deoxygenated blood from the systemic circulation returns to the right atrium of the heart via the superior and inferior vena cava. The blood then moves into the right ventricle through the tricuspid valve and gets pumped into the pulmonary artery when the right ventricle contracts.

The pulmonary artery divides into smaller vessels called arterioles, which further branch into a vast network of tiny capillaries in the lungs. Here, oxygen from the alveoli diffuses into the blood, binding to hemoglobin in red blood cells, while carbon dioxide leaves the blood and is exhaled through the nose or mouth.

The now oxygenated blood collects in venules, which merge to form pulmonary veins. These veins transport the oxygen-rich blood back to the left atrium of the heart, where it enters the systemic circulation once again. This continuous cycle enables the body's cells to receive the necessary oxygen and nutrients for proper functioning while disposing of waste products.

An aortopulmonary septal defect, also known as a congenital heart defect, is a type of ventricular septal defect (VSD). It is a hole in the wall that separates the two lower chambers of the heart - the left and right ventricles. In an aortopulmonary septal defect, the hole is located in the part of the ventricular septum that is next to the pulmonary valve and the aortic valve. This defect allows oxygenated blood from the left ventricle to mix with deoxygenated blood from the right ventricle, resulting in inefficient oxygenation of the blood and increased workload on the heart.

The severity of an aortopulmonary septal defect can vary depending on the size of the hole and the presence of other cardiac abnormalities. Symptoms may include difficulty breathing, poor feeding, and failure to thrive in infants. If left untreated, this condition can lead to serious complications such as heart failure, pulmonary hypertension, and infective endocarditis. Treatment typically involves surgical closure of the defect, which is usually performed during the first year of life.

The Fontan procedure is a type of open-heart surgery used to treat specific types of complex congenital (present at birth) heart defects. It's typically performed on children with single ventricle hearts, where one of the heart's lower chambers (the right or left ventricle) is underdeveloped or missing.

In a normal heart, oxygen-poor (blue) blood returns from the body to the right atrium, then flows through the tricuspid valve into the right ventricle. The right ventricle pumps the blue blood to the lungs, where it picks up oxygen and turns red. Oxygen-rich (red) blood then returns from the lungs to the left atrium, flows through the mitral valve into the left ventricle, and the left ventricle pumps it out to the body through the aorta.

However, in a single ventricle heart, the underdeveloped or missing ventricle cannot effectively pump blood to the lungs and the body simultaneously. The Fontan procedure aims to separate the blue and red blood circulation to improve oxygenation of the body's tissues.

The Fontan procedure involves two stages:

1. In the first stage, usually performed in infancy, a shunt or a band is placed around the pulmonary artery (the blood vessel that carries blood from the heart to the lungs) to control the amount of blood flowing into the lungs. This helps prevent lung congestion due to excessive blood flow.
2. The second stage, the Fontan procedure itself, takes place when the child is between 18 months and 4 years old. During this surgery, the surgeon creates a connection between the inferior vena cava (the large vein that returns blue blood from the lower body to the heart) and the pulmonary artery. This allows oxygen-poor blood to flow directly into the lungs without passing through the underdeveloped ventricle.

The Fontan procedure significantly improves the quality of life for many children with single ventricle hearts, although they may still face long-term complications such as heart failure, arrhythmias, and protein-losing enteropathy (a condition where the body loses too much protein in the stool). Regular follow-up care with a pediatric cardiologist is essential to monitor their health and manage any potential issues.

The heart ventricles are the two lower chambers of the heart that receive blood from the atria and pump it to the lungs or the rest of the body. The right ventricle pumps deoxygenated blood to the lungs, while the left ventricle pumps oxygenated blood to the rest of the body. Both ventricles have thick, muscular walls to generate the pressure necessary to pump blood through the circulatory system.

Transposition of the Great Vessels is a congenital heart defect in which the two main vessels that carry blood from the heart to the rest of the body are switched in position. Normally, the aorta arises from the left ventricle and carries oxygenated blood to the body, while the pulmonary artery arises from the right ventricle and carries deoxygenated blood to the lungs. In transposition of the great vessels, the aorta arises from the right ventricle and the pulmonary artery arises from the left ventricle. This results in oxygen-poor blood being pumped to the body and oxygen-rich blood being recirculated back to the lungs, which can lead to serious health problems and is often fatal if not corrected through surgery soon after birth.

Tricuspid valve insufficiency, also known as tricuspid regurgitation, is a cardiac condition in which the tricuspid valve located between the right atrium and right ventricle of the heart does not close properly, allowing blood to flow back into the right atrium during contraction of the right ventricle. This results in a portion of the blood being pumped inefficiently, which can lead to volume overload of the right side of the heart and potentially result in symptoms such as fatigue, weakness, shortness of breath, and fluid retention. The condition can be congenital or acquired, with common causes including dilated cardiomyopathy, infective endocarditis, rheumatic heart disease, and trauma.

Human chromosome pair 22 consists of two rod-shaped structures present in the nucleus of each cell in the human body. Each chromosome is made up of DNA tightly coiled around histone proteins, forming a complex structure called a chromatin.

Chromosome pair 22 is one of the 22 autosomal pairs of human chromosomes, meaning they are not sex chromosomes (X or Y). Chromosome 22 is the second smallest human chromosome, with each arm of the chromosome designated as p and q. The short arm is labeled "p," and the long arm is labeled "q."

Chromosome 22 contains several genes that are associated with various genetic disorders, including DiGeorge syndrome, velocardiofacial syndrome, and cat-eye syndrome, which result from deletions or duplications of specific regions on the chromosome. Additionally, chromosome 22 is the location of the NRXN1 gene, which has been associated with an increased risk for autism spectrum disorder (ASD) and schizophrenia when deleted or disrupted.

Understanding the genetic makeup of human chromosome pair 22 can provide valuable insights into human genetics, evolution, and disease susceptibility, as well as inform medical diagnoses, treatments, and research.

Cardiac catheterization is a medical procedure used to diagnose and treat cardiovascular conditions. In this procedure, a thin, flexible tube called a catheter is inserted into a blood vessel in the arm or leg and threaded up to the heart. The catheter can be used to perform various diagnostic tests, such as measuring the pressure inside the heart chambers and assessing the function of the heart valves.

Cardiac catheterization can also be used to treat certain cardiovascular conditions, such as narrowed or blocked arteries. In these cases, a balloon or stent may be inserted through the catheter to open up the blood vessel and improve blood flow. This procedure is known as angioplasty or percutaneous coronary intervention (PCI).

Cardiac catheterization is typically performed in a hospital cardiac catheterization laboratory by a team of healthcare professionals, including cardiologists, radiologists, and nurses. The procedure may be done under local anesthesia with sedation or general anesthesia, depending on the individual patient's needs and preferences.

Overall, cardiac catheterization is a valuable tool in the diagnosis and treatment of various heart conditions, and it can help improve symptoms, reduce complications, and prolong life for many patients.

Cardiac surgical procedures are operations that are performed on the heart or great vessels (the aorta and vena cava) by cardiothoracic surgeons. These surgeries are often complex and require a high level of skill and expertise. Some common reasons for cardiac surgical procedures include:

1. Coronary artery bypass grafting (CABG): This is a surgery to improve blood flow to the heart in patients with coronary artery disease. During the procedure, a healthy blood vessel from another part of the body is used to create a detour around the blocked or narrowed portion of the coronary artery.
2. Valve repair or replacement: The heart has four valves that control blood flow through and out of the heart. If one or more of these valves become damaged or diseased, they may need to be repaired or replaced. This can be done using artificial valves or valves from animal or human donors.
3. Aneurysm repair: An aneurysm is a weakened area in the wall of an artery that can bulge out and potentially rupture. If an aneurysm occurs in the aorta, it may require surgical repair to prevent rupture.
4. Heart transplantation: In some cases, heart failure may be so severe that a heart transplant is necessary. This involves removing the diseased heart and replacing it with a healthy donor heart.
5. Arrhythmia surgery: Certain types of abnormal heart rhythms (arrhythmias) may require surgical treatment. One such procedure is called the Maze procedure, which involves creating a pattern of scar tissue in the heart to disrupt the abnormal electrical signals that cause the arrhythmia.
6. Congenital heart defect repair: Some people are born with structural problems in their hearts that require surgical correction. These may include holes between the chambers of the heart or abnormal blood vessels.

Cardiac surgical procedures carry risks, including bleeding, infection, stroke, and death. However, for many patients, these surgeries can significantly improve their quality of life and longevity.

The fetal heart is the cardiovascular organ that develops in the growing fetus during pregnancy. It starts to form around 22 days after conception and continues to develop throughout the first trimester. By the end of the eighth week of gestation, the fetal heart has developed enough to pump blood throughout the body.

The fetal heart is similar in structure to the adult heart but has some differences. It is smaller and more compact, with a four-chambered structure that includes two atria and two ventricles. The fetal heart also has unique features such as the foramen ovale, which is a hole between the right and left atria that allows blood to bypass the lungs, and the ductus arteriosus, a blood vessel that connects the pulmonary artery to the aorta and diverts blood away from the lungs.

The fetal heart is responsible for pumping oxygenated blood from the placenta to the rest of the body and returning deoxygenated blood back to the placenta for re-oxygenation. The rate of the fetal heartbeat is faster than that of an adult, typically ranging from 120 to 160 beats per minute. Fetal heart rate monitoring is a common method used during pregnancy and childbirth to assess the health and well-being of the developing fetus.

Patent Ductus Arteriosus (PDA) is a congenital heart defect in which the ductus arteriosus, a normal fetal blood vessel that connects the pulmonary artery and the aorta, fails to close after birth. The ductus arteriosus allows blood to bypass the lungs while the fetus is still in the womb, but it should close shortly after birth as the newborn begins to breathe and oxygenate their own blood.

If the ductus arteriosus remains open or "patent," it can result in abnormal blood flow between the pulmonary artery and aorta. This can lead to various cardiovascular complications, such as:

1. Pulmonary hypertension (high blood pressure in the lungs)
2. Congestive heart failure
3. Increased risk of respiratory infections

The severity of the symptoms and the need for treatment depend on the size of the PDA and the amount of blood flow that is shunted from the aorta to the pulmonary artery. Small PDAs may close on their own over time, while larger PDAs typically require medical intervention, such as medication or surgical closure.

Imperforate anus is a congenital condition in which the opening of the anus is absent or abnormally closed or narrowed, preventing the normal passage of stool. This results in a blockage in the digestive tract and can lead to serious health complications if not treated promptly.

The anus is the external opening of the rectum, which is the lower end of the digestive tract. During fetal development, the rectum and anus normally connect through a canal called the anal canal or the recto-anal canal. In imperforate anus, this canal may be completely closed or narrowed, or it may not form properly.

Imperforate anus can occur as an isolated condition or as part of a genetic syndrome or other congenital abnormalities. The exact cause is not fully understood, but it is believed to result from a combination of genetic and environmental factors.

Treatment for imperforate anus typically involves surgery to create an opening in the anus and restore normal bowel function. In some cases, additional procedures may be necessary to correct related abnormalities or complications. The prognosis for individuals with imperforate anus depends on the severity of the condition and any associated abnormalities. With prompt and appropriate treatment, most people with imperforate anus can lead normal lives.

Echocardiography is a medical procedure that uses sound waves to produce detailed images of the heart's structure, function, and motion. It is a non-invasive test that can help diagnose various heart conditions, such as valve problems, heart muscle damage, blood clots, and congenital heart defects.

During an echocardiogram, a transducer (a device that sends and receives sound waves) is placed on the chest or passed through the esophagus to obtain images of the heart. The sound waves produced by the transducer bounce off the heart structures and return to the transducer, which then converts them into electrical signals that are processed to create images of the heart.

There are several types of echocardiograms, including:

* Transthoracic echocardiography (TTE): This is the most common type of echocardiogram and involves placing the transducer on the chest.
* Transesophageal echocardiography (TEE): This type of echocardiogram involves passing a specialized transducer through the esophagus to obtain images of the heart from a closer proximity.
* Stress echocardiography: This type of echocardiogram is performed during exercise or medication-induced stress to assess how the heart functions under stress.
* Doppler echocardiography: This type of echocardiogram uses sound waves to measure blood flow and velocity in the heart and blood vessels.

Echocardiography is a valuable tool for diagnosing and managing various heart conditions, as it provides detailed information about the structure and function of the heart. It is generally safe, non-invasive, and painless, making it a popular choice for doctors and patients alike.

Surgical anastomosis is a medical procedure that involves the connection of two tubular structures, such as blood vessels or intestines, to create a continuous passage. This technique is commonly used in various types of surgeries, including vascular, gastrointestinal, and orthopedic procedures.

During a surgical anastomosis, the ends of the two tubular structures are carefully prepared by removing any damaged or diseased tissue. The ends are then aligned and joined together using sutures, staples, or other devices. The connection must be secure and leak-free to ensure proper function and healing.

The success of a surgical anastomosis depends on several factors, including the patient's overall health, the location and condition of the structures being joined, and the skill and experience of the surgeon. Complications such as infection, bleeding, or leakage can occur, which may require additional medical intervention or surgery.

Proper postoperative care is also essential to ensure the success of a surgical anastomosis. This may include monitoring for signs of complications, administering medications to prevent infection and promote healing, and providing adequate nutrition and hydration.

Aortography is a medical procedure that involves taking X-ray images of the aorta, which is the largest blood vessel in the body. The procedure is usually performed to diagnose or assess various conditions related to the aorta, such as aneurysms, dissections, or blockages.

To perform an aortography, a contrast dye is injected into the aorta through a catheter that is inserted into an artery, typically in the leg or arm. The contrast dye makes the aorta visible on X-ray images, allowing doctors to see its structure and any abnormalities that may be present.

The procedure is usually performed in a hospital or outpatient setting and may require sedation or anesthesia. While aortography can provide valuable diagnostic information, it also carries some risks, such as allergic reactions to the contrast dye, damage to blood vessels, or infection. Therefore, it is typically reserved for situations where other diagnostic tests have been inconclusive or where more invasive treatment may be required.

Angiography is a medical procedure in which an x-ray image is taken to visualize the internal structure of blood vessels, arteries, or veins. This is done by injecting a radiopaque contrast agent (dye) into the blood vessel using a thin, flexible catheter. The dye makes the blood vessels visible on an x-ray image, allowing doctors to diagnose and treat various medical conditions such as blockages, narrowing, or malformations of the blood vessels.

There are several types of angiography, including:

* Cardiac angiography (also called coronary angiography) - used to examine the blood vessels of the heart
* Cerebral angiography - used to examine the blood vessels of the brain
* Peripheral angiography - used to examine the blood vessels in the limbs or other parts of the body.

Angiography is typically performed by a radiologist, cardiologist, or vascular surgeon in a hospital setting. It can help diagnose conditions such as coronary artery disease, aneurysms, and peripheral arterial disease, among others.

Palliative care is a type of medical care that focuses on relieving the pain, symptoms, and stress of serious illnesses. The goal is to improve quality of life for both the patient and their family. It is provided by a team of doctors, nurses, and other specialists who work together to address the physical, emotional, social, and spiritual needs of the patient. Palliative care can be provided at any stage of an illness, alongside curative treatments, and is not dependent on prognosis.

The World Health Organization (WHO) defines palliative care as: "an approach that improves the quality of life of patients and their families facing the problems associated with life-threatening illness, through the prevention and relief of suffering by means of early identification and impeccable assessment and treatment of pain and other problems, physical, psychological and spiritual."

The thoracic aorta is the segment of the largest artery in the human body (the aorta) that runs through the chest region (thorax). The thoracic aorta begins at the aortic arch, where it branches off from the ascending aorta, and extends down to the diaphragm, where it becomes the abdominal aorta.

The thoracic aorta is divided into three parts: the ascending aorta, the aortic arch, and the descending aorta. The ascending aorta rises from the left ventricle of the heart and is about 2 inches (5 centimeters) long. The aortic arch curves backward and to the left, giving rise to the brachiocephalic trunk, the left common carotid artery, and the left subclavian artery. The descending thoracic aorta runs downward through the chest, passing through the diaphragm to become the abdominal aorta.

The thoracic aorta supplies oxygenated blood to the upper body, including the head, neck, arms, and chest. It plays a critical role in maintaining blood flow and pressure throughout the body.

Pregnancy is a physiological state or condition where a fertilized egg (zygote) successfully implants and grows in the uterus of a woman, leading to the development of an embryo and finally a fetus. This process typically spans approximately 40 weeks, divided into three trimesters, and culminates in childbirth. Throughout this period, numerous hormonal and physical changes occur to support the growing offspring, including uterine enlargement, breast development, and various maternal adaptations to ensure the fetus's optimal growth and well-being.

Treatment outcome is a term used to describe the result or effect of medical treatment on a patient's health status. It can be measured in various ways, such as through symptoms improvement, disease remission, reduced disability, improved quality of life, or survival rates. The treatment outcome helps healthcare providers evaluate the effectiveness of a particular treatment plan and make informed decisions about future care. It is also used in clinical research to compare the efficacy of different treatments and improve patient care.

Follow-up studies are a type of longitudinal research that involve repeated observations or measurements of the same variables over a period of time, in order to understand their long-term effects or outcomes. In medical context, follow-up studies are often used to evaluate the safety and efficacy of medical treatments, interventions, or procedures.

In a typical follow-up study, a group of individuals (called a cohort) who have received a particular treatment or intervention are identified and then followed over time through periodic assessments or data collection. The data collected may include information on clinical outcomes, adverse events, changes in symptoms or functional status, and other relevant measures.

The results of follow-up studies can provide important insights into the long-term benefits and risks of medical interventions, as well as help to identify factors that may influence treatment effectiveness or patient outcomes. However, it is important to note that follow-up studies can be subject to various biases and limitations, such as loss to follow-up, recall bias, and changes in clinical practice over time, which must be carefully considered when interpreting the results.

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... is a rare birth defect characterized by pulmonary valve atresia occurring ... "Pulmonary atresia with ventricular septal defect". Orphanet. Retrieved 2022-09-08. "Pulmonary atresia". GOSH Hospital site. ... "Pulmonary atresia". Middlesex Health. Retrieved 2022-09-08. "Pulmonary atresia with ventricular septal defect". www.mayoclinic. ... "Pulmonary Atresia with Ventricular Septal Defect (VSD)". www.childrensmercy.org. Retrieved 2022-09-08. "Pulmonary atresia ...
Pulmonary atresia, malformation of the pulmonary valve in which the valve orifice fails to develop. Renal agenesis, only having ... "Pulmonary atresia". PubMed Health. Retrieved 11 September 2012. "Tricuspid atresia". PubMed Health. Retrieved 11 September 2012 ... Atresia is a condition in which an orifice or passage in the body is (usually abnormally) closed or absent. Examples of atresia ... Congenital bronchial atresia, a rare congenital abnormality Choanal atresia, blockage of the back of the nasal passage, usually ...
Somerville, J (1970). "Management of pulmonary atresia". Br Heart J. 32 (5): 641-51. doi:10.1136/hrt.32.5.641. PMC 487386. PMID ... Ross, D (1966). "Correction of Pulmonary Atresia with a Homograft Aortic Valve". The Lancet. 288 (7479): 1446-1447. doi:10.1016 ... the first report of the use of a homograft aortic valve to repair pulmonary atresia. In 1975, Somerville, "always feisty and ...
"Hybrid Ventricular Decompression in Pulmonary Atresia with Intact Septum". The Annals of Thoracic Surgery. 88 (2): 688-689. doi ... which allowed patients with pulmonary valve disease to have their valves replaced without surgery. Burke performed the first ... heart team used a 3D printed model of a child's heart and lungs to design a novel repair for complex total anomalous pulmonary ... partnered with cardiac teams in Boston and New York in the first US trial of the Medtronic Melody Transcatheter Pulmonary Valve ...
... pulmonary atresia; or pulmonary stenosis; or subpulmonary stenosis. The Rastelli procedure is typically performed between one ... The pulmonary valve is surgically closed. From the right ventricle to the pulmonary bifurcation, a synthetic conduit and a ... using a pulmonary or aortic homograft conduit to relieve pulmonary obstruction in double outlet right ventricle with pulmonary ...
"Repaired oesophageal atresia: respiratory morbidity and pulmonary function in adults". The European Respiratory Journal. 36 (5 ... Congenital esophageal atresia (EA) represents a failure of the esophagus to develop as a continuous passage. Instead, it ends ... Esophageal atresia is a congenital medical condition (birth defect) that affects the alimentary tract. It causes the esophagus ... The upper neck pouch sign is another sign that helps in the antenatal diagnosis of esophageal atresia and it may be detected ...
Pulmonary atresia is the complete closure of the pulmonary valve. Ebstein's anomaly is the displacement of the septal leaflet ... Less common CHD's are tricuspid and pulmonary atresia, and Ebstein's anomaly. Tricuspid atresia is the complete absence of the ... the pressure in the pulmonary artery will close the pulmonary valve. The closure of the pulmonary valve contributes the P2 ... The aortic and pulmonary valves are located at the base of the aorta and the pulmonary trunk respectively. These are also ...
These abnormalities include micrognathia, macroglossia, anal atresia, and pulmonary hypoplasia. The most commonly associated ...
Heart valve Pulmonary atresia Interior of right side of heart. Front of thorax, showing surface relations of bones, lungs ( ... the pressure in the pulmonary artery closes the pulmonary valve. The closure of the pulmonary valve contributes to the P2 ... Each cusp forms pocket like dilatation called pulmonary sinus at the initial portion of pulmonary trunk.[citation needed] The ... Pulmonary valves Pulmonary valves Stradins, P (September 2004). "Comparison of biomechanical and structural properties between ...
Asynchronous pulmonary hyperplasia associated with tracheal atresia: pathologic and prenatal sonographic findings. Pediatr ... Pulmonary hyperplasia is a serious pathological event which occurs in neonatal medicine. It leads to pulmonary hypertension and ... "Pulmonary hyperplasia and the two sides of PKC". Cardiovascular Research. 78 (3): 409-410. doi:10.1093/cvr/cvn089. PMID ...
Additional symptoms include cryptorchidism, anal stenosis, anal atresia, pulmonary hypoplasia, and congenital heart defects. ...
Other heart birth defects include ventricular septal defect, pulmonary atresia, and tetralogy of Fallot. An abdominal pregnancy ... Pulmonary resistance is reduced dramatically, prompting more blood to move into the pulmonary arteries from the right atrium ... The blood from the lungs travels through the pulmonary veins to the left atrium, producing an increase in pressure that pushes ... In the fetus, there is a special connection between the pulmonary artery and the aorta, called the ductus arteriosus, which ...
Though there is no clear pattern, the most common conditions include pulmonary stenosis and atresia. In addition, though ... Some research has shown that females may experience vaginal atresia or haematocolpos. The autosomal recessive form of the ... Balci, Sevim; Beksaç, Sinan; Haliloglu, Mithat; Ercis, Murat; Eryilmaz, Muzaffer (1998). "Robinow syndrome, vaginal atresia, ...
"Deletion of chromosome 22q11.2 and outcome in patients with pulmonary atresia and ventricular septal defect". The Annals of ... aortic atresia-severe stenosis with ventricular septal defect (AA/VSD); and aortic atresia with interrupted aortic arch and ... This allows for blood to flow from the left ventricle to the pulmonary valve. After creating this tunnel, the surgeon connects ... The surgeon then transects the pulmonary artery and aorta and frees them from surrounding tissue, then makes an incision into ...
The indications mainly include patients with pulmonary diseases such as pulmonary valve stenosis, atresia or insufficiency. ... for pulmonary valve replacement in congenital heart disease. Compared to crypreserved homograft, decellularized pulmonary ... The pulmonary valve then needs to be replaced by a heart valve prosthesis. A drawback of this method is that it can frequently ... and the pulmonary valve prosthesis, often a conventional cryopreserved homograft, is subject to the same rate of degeneration ...
By 1945, this operation had been performed on a total of three infants with pulmonary stenosis and pulmonary atresia. As Alfred ... "The Surgical Treatment of Malformations of the Heart in Which There Is Pulmonary Stenosis or Pulmonary Atresia". Journal of the ... "Heretofore there has been no satisfactory treatment for pulmonary stenosis and pulmonary atresia. A "blue" baby with a ... pulmonary atresia, and more rare and complex abnormalities. At the time of Taussig's death, tens of thousands of children's ...
"The surgical treatment of malformations of the heart in which there is pulmonary stenosis or pulmonary atresia". JAMA. 128: 189 ... Pulmonary reserve is measured by spirometry. If there is no evidence of undue shortness of breath or diffuse parenchymal lung ... In addition, people who are very ill with a poor performance status or who have inadequate pulmonary reserve would be unlikely ... In 1948, Russell Brock, probably unaware of Sellor's work, used a specially designed dilator in three cases of pulmonary ...
... namely pulmonary or aortic atresia. Myocardial sinusoids is considered not an accurate term as endothelium lines the ... Cardiac Abnormalities of the origin of the left coronary artery Pulmonary atresia Stenosis Right or Left ventricle obstruction ...
HRHS is known for the pulmonary atresia valves, the tricuspid valve, right ventricle, and the pulmonary artery all failing to ... The proximal pulmonary artery is connected to the aortic arch, while the narrowed segment of the pulmonary trunk is repaired. ... An aortopulmonary shunt is created to connect the aorta to the main pulmonary artery to provide pulmonary blood flow to the ... "Prenatal diagnosis of an isochromosome 5p in a fetus with increased nuchal translucency thickness and pulmonary atresia with ...
Pulmonary atresia with ventricular septal defect (or Tetralogy of Fallot with pulmonary atresia) will result in the development ... Interactive videogame graphic showing repair of tetralogy of Fallot with pulmonary atresia - from Lucile Packard Children's ... Management of Tetralogy of Fallot with pulmonary atresia - contains angiographic images of MAPCAs. Maeda, Eriko; Masaaki ... associated with congenital heart diseases with decreased pulmonary blood flow like tetralogy of Fallot or pulmonary atresia it ...
... such as pulmonary atresia, Tetralogy of Fallot, or persistent truncus arteriosus. PPVI can also be used to replace ... Percutaneous pulmonary valve implantation (PPVI), also known as transcatheter pulmonary valve replacement (TPVR), is the ... October 2000). "Percutaneous replacement of pulmonary valve in a right-ventricle to pulmonary-artery prosthetic conduit with ... less invasive procedure in comparison to open heart surgery and is commonly used to treat conditions such as pulmonary atresia ...
The Burying Beetle and The Bower Bird chronicle the story of Gussie, a 12-year-old girl who suffers from pulmonary atresia, a ...
... tricuspid atresia, and anomalous pulmonary venous connection. Aortopulmonary septal defect This article incorporates text in ... It divides the distal part of the truncus into two vessels, the aorta and pulmonary artery, which lie side by side above, but ... and actively separates the aorta and pulmonary arteries and fuses with the interventricular septum within the heart during ... near the heart the pulmonary artery is in front of the aorta. Four endocardial cushions appear in the proximal part of the ...
... such as using fetal cardiac catheterization to correct pulmonary atresia with intact ventricular septum. Spina Bifida is ...
Tricuspid atresia Interrupted aortic arch Coarctation of aorta Pulmonary atresia (PA) Pulmonary stenosis (critical) Atrial ... valve stenosis Pulmonary valve stenosis Mitral insufficiency/regurgitation Tricuspid insufficiency/regurgitation Pulmonary ... Total anomalous pulmonary venous connection Hypoplastic left heart syndrome (HLHS) Transposition of the great arteries (d-TGA) ...
Tricuspid atresia Interrupted aortic arch Pulmonary atresia (PA) Pulmonary stenosis (critical) Eisenmenger syndrome (reversal ... The pulmonary pressure resistance in the cavopulmonary connection is increased, and these models permit clear analyses of the ... Tetralogy of Fallot (ToF) Total anomalous pulmonary venous connection Hypoplastic left heart syndrome (HLHS) Transposition of ... or any condition which increases pulmonary vascular resistance. The result may be the development of collateral circulation. ...
... or the pulmonary artery. Pulmonary atresia Pulmonary valve stenosis Hypoplastic right heart syndrome Tetralogy of Fallot A left ...
... surgical procedure used to increase blood flow to the lungs in some forms of congenital heart disease such as pulmonary atresia ... Some centers now use a shunt directly from the right ventricle to the pulmonary artery, a Sano shunt. This is done to avoid the ... The operation involved the joining of the subclavian artery to the pulmonary artery. After meeting with Taussig, the two men ... The procedure involves connecting a branch of the subclavian artery or carotid artery to the pulmonary artery. In modern ...
Pulmonary atresia with an intact ventricular septum: This type of pulmonary atresia is associated with complete and intact ... Pulmonary atresia with a ventricular septal defect: This type of pulmonary atresia happens when a ventricular septal defect ... "Facts about Pulmonary Atresia: Types of Pulmonary Atresia". CDC. USA.gov. Retrieved August 22, 2017. Rao, P. Syamasundar (2019- ... Pulmonary atresia happens in 7-8 per 100,000 births and is characterized by the aorta branching out of the right ventricle. ...
  • In the case of pulmonary atresia with ventricular septal defect, decreased pulmonary blood flow may cause associated defects such as: Tricuspid atresia Tetralogy of Fallot Double outlet right ventricle In terms of the cause of pulmonary atresia, there is uncertainty as to what instigates this congenital heart defect. (wikipedia.org)
  • Pulmonary atresia with intact ventricular septum (PA-IVS) is a rare congenital malformation. (wikipedia.org)
  • Pulmonary atresia with ventricular septal defect (PA-VSD) is identified by underdevelopment of the right ventricle. (wikipedia.org)
  • Pulmonary atresia may occur with or without a ventricular septal defect (VSD) . (medlineplus.gov)
  • If the person does not have a VSD, the condition is called pulmonary atresia with intact ventricular septum (PA/IVS). (medlineplus.gov)
  • In this form of pulmonary atresia, a ventricular septal defect (VSD) allows blood to flow into and out of the right ventricle (RV). (cdc.gov)
  • Therefore, blood flowing into the RV can help the ventricle develop during pregnancy, so it is typically not as small as in pulmonary atresia with an intact ventricular septum. (cdc.gov)
  • Pulmonary atresia with ventricular septal defect (PA-VSD) is a cyanotic congenital heart disease characterized by underdevelopment of the right ventricular (RV) outflow tract (ie, subpulmonary infundibulum) with atresia of the pulmonary valve, a large ventricular septal defect (VSD), and overriding of the aorta. (medscape.com)
  • Pulmonary atresia with ventricular septal defect demonstrates a wide spectrum of severity, depending on the degree of pulmonary artery development. (medscape.com)
  • Pathologically, pulmonary atresia with ventricular septal defect is frequently considered the most severe end of the spectrum of tetralogy of Fallot (TOF) , but whether pulmonary atresia with ventricular septal defect and TOF should be treated as two distinct entities is controversial. (medscape.com)
  • In patients with the standard type of TOF with pulmonary atresia, pulmonary arteries are usually normal in size with normal peripheral pulmonary arborization, which is unlike pulmonary atresia with ventricular septal defect. (medscape.com)
  • In addition, systemic-to-pulmonary collateral vessels are not as well developed in patients with TOF with pulmonary atresia as they are in patients with pulmonary atresia with ventricular septal defect. (medscape.com)
  • In pulmonary atresia with ventricular septal defect, the extent of pulmonary artery development determines the clinical presentation and the surgical options available. (medscape.com)
  • Many children with pulmonary atresia also have a ventricular septal defect (VSD) , a hole in the tissue between the lower chambers of the heart. (chop.edu)
  • In children with pulmonary atresia and ventricular septal defect (VSD), surgery will be necessary to close the VSD. (chop.edu)
  • It was then we found out Ivy had a very rare heart condition: pulmonary atresia with an intact ventricular septum. (tinytickers.org)
  • Pulmonary atresia, intact ventricular septum, and Ebstein anomaly of the tricuspid valve. (unipd.it)
  • Pulmonary atresia with intact ventricular septum is a disorder that involves the whole right ventricle. (unipd.it)
  • Ebstein anomaly of the tricuspid valve further complicates surgical management and outcome of pulmonary atresia and intact ventricular septum. (unipd.it)
  • Objective: We and others have observed significant hyperinflation and airflow obstruction after the surgical repair of pulmonary atresia and ventricular septal defect. (elsevierpure.com)
  • Methods: We performed a prospective study of children and young adults with pulmonary atresia and ventricular septal defect between June 1996 and December 1998. (elsevierpure.com)
  • Conclusions: We have identified an extremely strong association between pulmonary atresia and ventricular septal defect and persistent airway hyperresponsiveness. (elsevierpure.com)
  • These results provide a basis to anticipate persistent respiratory difficulties after operations in patients with pulmonary atresia and ventricular septal defect. (elsevierpure.com)
  • Causes of primary diastolic dysfunction include an anatomic obstruction that prevents ventricular filling (eg, pulmonary venous obstruction), a primary reduction in ventricular compliance (eg, cardiomyopathy, transplant rejection), external constraints (eg, pericardial effusion), and poor hemodynamics after the Fontan procedure (eg, elevated pulmonary vascular resistance). (medscape.com)
  • Background: We advocate one-stage unifocalization concomitant with palliative right ventricular outflow tract reconstruction (pRVOTR) for patients with major aortopulmonary collateral arteries (MAPCA) in pulmonary atresia with ventricular septal defects. (jspccs.jp)
  • Definitive repair was performed in two patients, and staged repair with pRVOTR (right ventricular to pulmonary artery conduits with a diameter of 75% of the normal pulmonary annulus diameter) was performed in 13 patients. (jspccs.jp)
  • Eitoku, T , Kasahara, S , Baba, K & Kotani, Y 2021, ' Impact of decompression of the right ventricle on the sinusoidal communications in pulmonary atresia and intact ventricular septum ', Journal of Thoracic and Cardiovascular Surgery . (elsevierpure.com)
  • That day he was diagnosed with Pulmonary Atresia with Intact Ventricular Septum. (irishcentral.com)
  • Tricuspid atresia is the most common cause of cyanosis with left ventricular hypertrophy. (medscape.com)
  • Persistent truncus arteriosus occurs when, during fetal development, the primitive truncus does not divide into the pulmonary artery and aorta, resulting in a single, large, arterial trunk that overlies a large, malalignment type ventricular septal defect. (msdmanuals.com)
  • Atresia of the tricuspid valve and total anomalous supracardiac pulmonary venous return. (bvsalud.org)
  • However, in tetralogy of Fallot, the pulmonary valve (PV) does form, although it is small and blood has trouble flowing through it - this is called pulmonary valve stenosis. (cdc.gov)
  • If collaterals are multiple and the ductus is congenitally absent, abnormal intrapulmonary arborization (ie, stenosis of unbranched and intrapulmonary arteries) and pulmonary hypertension are present. (medscape.com)
  • Other associations include tricuspid atresia or stenosis, complete atrioventricular (AV) canal, complete or corrected transposition of the great arteries, left superior vena cava, anomalies of the coronary sinus, dextrocardia, and asplenia or polysplenia syndrome. (medscape.com)
  • The pathologic, clinical, and electrocardiographic features of tricuspid stenosis and atresia are similar. (medscape.com)
  • [ 19 ] Therefore, the fact that isolated congenital tricuspid stenosis belongs to the group of tricuspid atresia defects and that their embryologic developments are similar is no surprise. (medscape.com)
  • Thus, the tricuspid valve stenosis, tricuspid atresia with well formed but fused valve leaflets, and the muscular type of tricuspid atresia represent a spectrum of morphologic abnormalities. (medscape.com)
  • Modified technique for dilatation of pulmonary valve stenosis (nearly atresia) in a neonate]. (bvsalud.org)
  • Because of this lack of blood flowing through the right side of the heart, the structures on that side, such as the pulmonary valve and the tricuspid valve, are abnormally small. (wikipedia.org)
  • Pulmonary atresia also usually means that the patient will have an inadequately formed tricuspid valve and an undersized right ventricle. (drug-attorneys.com)
  • Tricuspid atresia may be defined as congenital absence or agenesis of the tricuspid valve. (medscape.com)
  • Whether a muscular type of tricuspid atresia develops or whether well-formed but fused tricuspid-valve leaflets develop depends on the stage of development when the embryologic aberration takes place. (medscape.com)
  • Pulmonary circulation may be supplied by a patent ductus arteriosus (PDA), systemic-to-pulmonary collaterals, or plexuses of bronchial and pleural arteries. (medscape.com)
  • Pulmonary atresia often occurs paired alongside another congenital heart condition named "Patent Ductus Arteriosus, or PDA. (drug-attorneys.com)
  • This combination is often called tetralogy of Fallot with pulmonary atresia. (chop.edu)
  • In this interactive 3-D animation, our doctors compare a healthy heart to one with a complex congenital condition called tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries (MAPCAs) . (stanfordchildrens.org)
  • As with most congenital heart diseases, there is no known cause of pulmonary atresia. (medlineplus.gov)
  • A 6-year-old female (height: 115 cm, weight: 22 kg) patient with pediatric living donor LT for biliary atresia (cirrhosis with portal hypertension) with a pulmonary AVF, HPS, was admitted to an intensive care unit (ICU) transplantation. (transpopmed.org)
  • The cardiac analysis confirmed dextrocardia, associated with patent foramen ovale and atrial septal defect, without pulmonary arterial hypertension. (clinicalcasereportsjournal.com)
  • Persistent pulmonary hypertension of the newborn (PPHN) is defined as the failure of the normal circulatory transition that occurs after birth. (medscape.com)
  • It is a syndrome characterized by marked pulmonary hypertension that causes hypoxemia secondary to right-to-left shunting of blood at the foramen ovale and ductus arteriosus. (medscape.com)
  • Idiopathic persistent pulmonary hypertension of the newborn can present without signs of acute perinatal distress. (medscape.com)
  • In contrast to adult primary pulmonary hypertension, the newborn syndrome is not defined by a specific pressure of the pulmonary circulation. (medscape.com)
  • can include pulmonary fibrosis, bullae, and emphysematous changes that can contribute to pulmonary hypertension and cor pulmonale in 5% of cases. (medscape.com)
  • Potential risk factors that can cause this congenital heart defect are those the pregnant mother may come in contact with, such as: Certain medications Diet Smoking In regards to the diagnosis of pulmonary atresia the body requires oxygenated blood for survival. (wikipedia.org)
  • medical citation needed] The diagnosis of pulmonary atresia can be done via the following exams/methods: an echocardiogram, chest x-ray, EKG and an exam to measure the amount of O 2in the body. (wikipedia.org)
  • Absence of the left pulmonary veins and the presence of collaterals draining to the innominate vein confirmed the diagnosis of acquired UPVA. (mcmaster.ca)
  • The diagnosis is confirmed regardless of the pulmonary arterial pressure, as long as it is accompanied by a right-to-left shunt and absence of congenital heart disease. (medscape.com)
  • While unilateral pulmonary venous atresia (UPVA) most commonly presents as an extremely rare late embryological defect resulting in complete occlusion or absence of the PV pathway, it may also be an acquired pathology. (mcmaster.ca)
  • We describe a case of a newborn presenting type III esophageal atresia associated with unilateral pulmonary atresia and dextrocardia. (clinicalcasereportsjournal.com)
  • To our knowledge, this is the first reported case of EA with TEF and unilateral pulmonary atresia in a patient with dextrocardia in situs solitus. (clinicalcasereportsjournal.com)
  • Type B (multifocal, with confluent but hypoplastic pulmonary arteries supplied by major aortopulmonary collateral arteries [MAPCAs]): Pulmonary blood flow is supplied by native pulmonary arteries and by MAPCAs. (medscape.com)
  • In congenital heart defects such as pulmonary atresia, structural abnormalities can include the valves of the heart, and the walls and arteries/veins near the heart muscle. (wikipedia.org)
  • The right and left pulmonary arteries may communicate freely (ie, confluence) or may not communicate (ie, nonconfluence). (medscape.com)
  • The pathology of intrapulmonary arteries depends on the pulmonary blood flow and the patency of the ductus. (medscape.com)
  • If the ductus is large and supplies confluent pulmonary arteries, the blood flow and the intrapulmonary arteries of both lungs are normal. (medscape.com)
  • Type A (unifocal with confluent, good-sized pulmonary arteries): Pulmonary blood flow is provided by native pulmonary arteries. (medscape.com)
  • Sometimes the pulmonary arteries are very small. (chop.edu)
  • The central pulmonary arteries were created by using an autologous pericardium roll/patch. (jspccs.jp)
  • Bilateral equal pulmonary blood flow was obtained and led to the growth of pulmonary arteries with this procedure. (jspccs.jp)
  • The right chambers are responsible for pumping blood to the lungs through the pulmonary arteries. (vejthani.com)
  • Before profound heart failure develops, peripheral pulses will be bounding because of the large run off from the proximal aorta to the pulmonary arteries. (msdmanuals.com)
  • Type I: The main pulmonary artery arises from the truncus and then divides into the right and left pulmonary arteries. (msdmanuals.com)
  • Type II: The right and left pulmonary arteries arise separately (but adjacent to each other) from the posterior aspect of the truncus. (msdmanuals.com)
  • Type III: The right and left pulmonary arteries arise from the lateral aspects of the truncal root reasonably distant from each other. (msdmanuals.com)
  • Type IV: Both pulmonary arteries are supplied by collateral vessels from the descending aorta. (msdmanuals.com)
  • Neonatal echocardiographic investigations revealed normal mediastinal anatomy and pulmonary vasculature with a bicuspid aortic valve. (mcmaster.ca)
  • The symptoms/signs of pulmonary atresia that will occur in babies are consistent with cyanosis, some fatigue and some shortness of breath (eating may be a problem as well). (wikipedia.org)
  • What are the signs and symptoms of Pediatric Pulmonary Atresia? (childrens.com)
  • Basically, Pulmonary Atresia can occur in a variety of ways with differing symptoms. (drug-attorneys.com)
  • In pulmonary atresia, since blood cannot directly flow from the right ventricle of the heart out to the pulmonary artery, blood must use other routes to bypass the unformed pulmonary valve. (cdc.gov)
  • Tricuspid atresia: association with persistent truncus arteriosus. (medscape.com)
  • This particular type appears to be associated with absent pulmonary valve leaflets. (medscape.com)
  • The treatment of pulmonary atresia consists of: an IV medication called prostaglandin E1, which is used for treatment of pulmonary atresia, as it stops the ductus arteriosus from closing, allowing mixing of the pulmonary and systemic circulations, but prostaglandin E1 can be dangerous as it can cause apnea. (wikipedia.org)
  • The systemic or pulmonary circulation may depend on the patency of the ductus arteriosus, especially in patients presenting in the first few days of life. (medscape.com)
  • Surgeons may open the pulmonary valve by patching across it, or they may place a shunt (a narrow tube) from the aorta to the pulmonary artery (vessel to the lung) to be sure there is enough blood flow to the lungs. (chop.edu)
  • A shunt can be created between the aorta and the pulmonary artery to help increase blood flow to the lungs. (wikipedia.org)
  • Because a baby with pulmonary atresia may need surgery or other procedures soon after birth, this birth defect is considered a critical congenital heart defect (critical CHD). (cdc.gov)
  • In a baby without a congenital heart defect, the right side of the heart pumps oxygen-poor blood from the heart to the lungs through the pulmonary artery. (cdc.gov)
  • A congenital heart defect characterized by the narrowing or complete absence of the opening between the RIGHT VENTRICLE and the PULMONARY ARTERY. (bvsalud.org)
  • Furthermore, Pulmonary Atresia can take place with or without a septal defect as well. (drug-attorneys.com)
  • If the constriction occurs right at the valve, it is called pulmonary valvular atresia. (aboutkidshealth.ca)
  • The type of surgery recommended depends on the size of the right ventricle and the pulmonary artery, if the right ventricle is small and unable to act as a pump, the surgery performed would be the Fontan procedure. (wikipedia.org)
  • In other cases, open heart surgery is required to repair pulmonary atresia. (chop.edu)
  • Why might my child need surgery for pulmonary atresia without VSD? (orovillehospital.com)
  • In some cases, healthcare providers are able to treat milder forms (pulmonary atresia) without surgery. (orovillehospital.com)
  • What are the risks of surgery for pulmonary atresia without VSD for a child? (orovillehospital.com)
  • How can I help my child get ready for surgery for pulmonary atresia without VSD? (orovillehospital.com)
  • The surgery chosen depends on the size of the pulmonary artery and right ventricle. (epnet.com)
  • Justin was only 2 days old when he had open heart surgery at Children's Hospital of Philadelphia to treat his pulmonary atresia. (chop.edu)
  • Surgery was performed on day 1 to cure the atresia and close the tracheoesophageal fistula, with success. (clinicalcasereportsjournal.com)
  • The pulmonary valve is located on the right side of the heart between the right ventricle and pulmonary artery. (wikipedia.org)
  • The pulmonary valve is an opening on the right side of the heart that regulates blood flow from the right ventricle (right side pumping chamber) to the lungs. (medlineplus.gov)
  • Pulmonary atresia is a birth defect of the pulmonary valve, which is the valve that controls blood flow from the right ventricle (lower right chamber of the heart) to the main pulmonary artery (the blood vessel that carries blood from the heart to the lungs). (cdc.gov)
  • Pulmonary atresia is when this valve didn't form at all, and no blood can go from the right ventricle of the heart out to the lungs. (cdc.gov)
  • In babies with pulmonary atresia, the pulmonary valve that usually controls the blood flowing through the pulmonary artery is not formed, so blood is unable to get directly from the right ventricle to the lungs. (cdc.gov)
  • The artery which usually carries blood out of the right ventricle, the main pulmonary artery (MPA), remains very small, since the pulmonary valve (PV) doesn't form. (cdc.gov)
  • In some cases of pulmonary atresia, the right ventricle is poorly developed and cannot function adequately as a pump. (chop.edu)
  • The right ventricle pumps blood that's low in oxygen through the pulmonary artery to the lungs. (orovillehospital.com)
  • Between the right ventricle and the pulmonary artery lies the pulmonary valve, one of the heart's four valves. (orovillehospital.com)
  • So there is no connection between the right ventricle and the pulmonary artery. (orovillehospital.com)
  • For children who have pulmonary atresia without VSD, treatments vary based on if the right ventricle is working as it should. (orovillehospital.com)
  • As a rule, the pulmonary valve is the cavity on the right side of the heart that controls the blood stream between the right ventricle and the lungs. (drug-attorneys.com)
  • a condition involving the pulmonary valve, between the right ventricle and pulmonary artery, not forming completely. (aboutkidshealth.ca)
  • With this condition, the pulmonary valve, between the right ventricle and the pulmonary artery, is not formed completely. (aboutkidshealth.ca)
  • With pulmonary atresia, the pulmonary valve cannot open properly, meaning blood can't flow from the right ventricle to the lungs as it normally would. (aboutkidshealth.ca)
  • In pulmonary atresia, the valve cannot open properly, which means the blood cannot move from the right ventricle to the lungs. (aboutkidshealth.ca)
  • Lacking a normal PULMONARY VALVE, unoxygenated blood in the right ventricle can not be effectively pumped into the lung for oxygenation. (bvsalud.org)
  • Then the oxygenated blood travels through pulmonary veins to back to the left side heart chamber. (vejthani.com)
  • Occurs when some or all pulmonary veins are connected to a wrong area or areas of the heart. (vejthani.com)
  • Our patient, a 6-year-old girl, underwent a porto-enterostomy (Kasai) operation on the 45 th day of delivery due to biliary atresia. (transpopmed.org)
  • We present a 6-year-old female patient with successful anesthetic management of pediatric living donor LT with HPS and pulmonary arteriovenous fistula (AVF) due to biliary atresia. (transpopmed.org)
  • Herein, we present the successful anesthetic management of a pediatric living donor LT for biliary atresia with a pulmonary AVF and HPS in the light literature. (transpopmed.org)
  • She had a Kasai operation on the 45 th day after birth to treat biliary atresia. (transpopmed.org)
  • In a healthy heart, the right side of the heart pumps blood into the lungs through the pulmonary artery. (childrens.com)
  • Very young children with elevated pulmonary vascular resistance may not able to undergo the Fontan procedure. (wikipedia.org)
  • Multiple treatments are possible, but which one is selected depends on the extent of the heart abnormalities that accompany the pulmonary valve defect. (medlineplus.gov)
  • Pulmonary atresia is a birth defect (pronounced PULL-mun-airy ah-TREE-sha) of the heart where the valve that controls blood flow from the heart to the lungs doesn't form at all. (cdc.gov)
  • Pulmonary atresia is a birth defect where the pulmonary valve that carries blood from the heart to the lungs doesn't form during development and often requires medical attention soon after birth. (childrens.com)
  • Ivy's heart defect, pulmonary atresia, wasn't detected until she was critically ill. (tinytickers.org)
  • If you took Lexapro (or other SSRI) and had a child born with Pulmonary Atresia (PA) or other serious heart or birth defect, call the Willis Law Firm today for a Free & Confidential Lexapro Lawsuit Consultation because you and your child may be eligable to monetary compensation for damages and injuries through a Lexapro Pulmonary Atresia (PA) Lawsuit. (drug-attorneys.com)
  • Furthermore, Lexapro Pulmonary Atresia (PA) and birth defect cases are taken on a contingency fee basis, and we are currently accepting Lexapro Birth Defect Lawsuits Nationwide. (drug-attorneys.com)
  • Pulmonary atresia (PA) is a rare heart defect. (epnet.com)
  • Little more than 3 decades ago, the terminology for this defect (eg, tricuspid atresia, univentricular heart, univentricular atrioventricular connection) was intensely debated. (medscape.com)
  • Children who have had surgical repair of pulmonary atresia require lifelong care by a cardiologist. (chop.edu)
  • Before the operation, the surgical strategy for unifocalization of MAPCA and pulmonary reconstruction was planned using accurate spatial information from the differential color imaging provided by 3D CT angiography. (jspccs.jp)
  • Surgical exploration and complementary exams showed: a type III esophageal atresia, a dextrocardia, and a severe right pulmonary atresia associated with right pulmonary artery hypoplasia. (clinicalcasereportsjournal.com)
  • Two patients were managed by pulmonary valvotomy, three by systemic-pulmonary artery shunt, and two had a combination of the two. (unipd.it)
  • Consequently, a mixture of oxygenated and deoxygenated blood enters systemic, pulmonary, and coronary circulations. (msdmanuals.com)
  • In pulmonary atresia, the valve leaflets are fused. (medlineplus.gov)
  • [ 17 , 18 ] The classic muscular form of tricuspid atresia develops if the embryologic insult occurs early in gestation, and fused valve leaflets occur if the embryologic abnormality occurs slightly later than this in gestation. (medscape.com)
  • The pulmonary arterial systolic pressure was 35.3±14.1 mmHg before definitive repair and decreased to 29.3±2.2 mmHg after definitive repair. (jspccs.jp)
  • In this three-stage procedure, the right atrium is disconnected from the pulmonary circulation. (wikipedia.org)
  • PA occurs when the pulmonary valve does not appropriately shape during the fetal heart maturation phase. (drug-attorneys.com)
  • When pulmonary atresia occurs, a hard piece of tissue develops right where the valve needs to be. (drug-attorneys.com)
  • Pulmonary atresia occurs in about one baby per 14,000 births, and is the tenth most common type of heart disease in newborns. (aboutkidshealth.ca)
  • Informez-vous sur l'atrésie pulmonaire, un trouble qui fait en sorte que la valvule pulmonaire, entre le ventricule droit et l'artère pulmonaire, ne se forme pas complètement. (aboutkidshealth.ca)
  • Thus, pulmonary atresia with a VSD is like a very severe form of tetralogy of Fallot. (cdc.gov)
  • In a normal functioning heart, the opening to the pulmonary valve has three flaps that open and close. (wikipedia.org)
  • PA-IVS involves complete blockage of the pulmonary valve located on the right side of the heart. (wikipedia.org)
  • Pulmonary atresia is a form of heart disease in which the pulmonary valve does not form properly. (medlineplus.gov)
  • The causes of heart defects, such as pulmonary atresia, among most babies are unknown. (cdc.gov)
  • The pulmonary valve is an opening on the right side of the heart that helps prevent blood from leaking back into the heart between beats. (chop.edu)
  • In the congenital heart disease pulmonary atresia, the pulmonary valve has not formed correctly: it is sealed and can't open. (chop.edu)
  • If your baby is diagnosed with pulmonary atresia before birth, the Fetal Heart Program at Children's Hospital can prepare a plan for delivery and care immediately after birth. (chop.edu)
  • The exact treatment for pulmonary atresia depends on each child's heart anatomy. (chop.edu)
  • Pulmonary atresia is a condition where the pulmonary valve didn't form in the heart. (orovillehospital.com)
  • Normally the pulmonary valve opens when the heart contracts. (orovillehospital.com)
  • Pulmonary atresia (PA) is one of several kinds of congenital heart disease. (drug-attorneys.com)
  • Current medical research suggests that women taking SSRI's, like Lexapro, during all or any part of pregnancy results in the elevated risk of congenital heart defects, including Pulmonary Atresia. (drug-attorneys.com)
  • Other SSRIs associated with Pulmonary Atresia and other congenital heart defects include: Zoloft, Celexa, Paxil, and Prozac. (drug-attorneys.com)
  • It is when the heart does not have a pulmonary valve. (epnet.com)
  • Atresia" refers to a missing heart structure. (aboutkidshealth.ca)
  • It basically means that the right side of Adam's heart never developed because he has no pulmonary valve and the blood couldn't pump from his heart into his lungs," explains Lisa, a waitress. (irishcentral.com)
  • Sometimes a shunt can be placed between the aorta and pulmonary artery. (epnet.com)
  • Patency of the ductus maintains the pulmonary circulation. (medscape.com)
  • In this form of pulmonary atresia, the wall, or septum, between the ventricles remains complete and intact. (cdc.gov)

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