An anomalous pulmonary venous return in which the right PULMONARY VEIN is not connected to the LEFT ATRIUM but to the INFERIOR VENA CAVA. Scimitar syndrome is named for the crescent- or Turkish sword-like shadow in the chest radiography and is often associated with hypoplasia of the right lung and right pulmonary artery, and dextroposition of the heart.
A developmental anomaly in which a mass of nonfunctioning lung tissue lacks normal connection with the tracheobroncheal tree and receives an anomalous blood supply originating from the descending thoracic or abdominal aorta. The mass may be extralobar, i.e., completely separated from normally connected lung, or intralobar, i.e., partly surrounded by normal lung.
A spectrum of congenital, inherited, or acquired abnormalities in BLOOD VESSELS that can adversely affect the normal blood flow in ARTERIES or VEINS. Most are congenital defects such as abnormal communications between blood vessels (fistula), shunting of arterial blood directly into veins bypassing the CAPILLARIES (arteriovenous malformations), formation of large dilated blood blood-filled vessels (cavernous angioma), and swollen capillaries (capillary telangiectases). In rare cases, vascular malformations can result from trauma or diseases.
The venous trunk which receives blood from the lower extremities and from the pelvic and abdominal organs.
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 characteristic symptom complex.
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.

Coil occlusion of aortopulmonary collateral arteries in an infant with scimitar syndrome. (1/31)

Scimitar syndrome in infancy is a rare condition, presenting with severe congestive heart failure and pulmonary hypertension. The presence of large systemic-pulmonary collateral arteries may play a role in the cause of heart failure and pulmonary hypertension. A 4-month-old infant underwent coil occlusion of large anomalous systemic arteries supplying the right lower pulmonary lobe. Symptoms of severe congestive heart failure and pulmonary hypertension improved dramatically with coil occlusion, and surgical correction was performed 3 months later without any complications. Coil occlusion of anomalous systemic arteries can improve symptoms of heart failure and pulmonary hypertension in infants and may bring about a good surgical result for this disease.  (+info)

Gadolinium-enhanced magnetic resonance angiography in scimitar syndrome: diagnosis and postoperative evaluation. (2/31)

We report the 1st magnetic resonance demonstration of both an anomalous pulmonary venous drainage and systemic arterial supply in a patient with scimitar syndrome. With its superior imaging capabilities, gadolinium-enhanced magnetic resonance angiography provides a powerful diagnostic tool for this complex congenital lesion and offers the possibility of surgical repair and follow-up without conventional cardiac catheterization.  (+info)

Retrospective prenatal diagnosis of scimitar syndrome aided by three-dimensional power Doppler imaging. (3/31)

Scimitar syndrome is a rare malformation of the arterial supply and venous drainage of the lung. We report the case of a fetus that presented with cardiac asymmetry and malposition of the fetal heart. Postnatally, scimitar syndrome was confirmed at cardiac catheterization. Retrospective reconstruction of three-dimensional power Doppler volumes, obtained during fetal life, allowed direct visualization of the abnormal aortopulmonary collateral vessel. This had not been seen on conventional scans. This case demonstrates many of the strengths of three-dimensional sonographic techniques for the delineation of complex vascular anatomy. It confirms that a prospective diagnosis of scimitar syndrome should be possible during fetal life.  (+info)

Meandering right pulmonary vein to the left atrium and inferior vena cava: the first case with associated anomalies. (4/31)

We report a case of a healthy, asymptomatic 6-year-old boy in whom an anomalous right pulmonary vein was noted to drain into both the inferior vena cava and left atrium in association with findings consistent with scimitar syndrome. The anomalous pulmonary vein took a very circuitous route through the lungs before draining into the left atrium, a condition previously termed "meandering pulmonary vein." To aid in the diagnosis, cardiovascular magnetic resonance imaging and magnetic resonance angiography were used to delineate this complex course and the connection of the anomalous pulmonary vein. To our knowledge, this is the 1st reported case of a meandering pulmonary vein with dual drainage to the inferior vena cava and left atrium in association with other anomalies.  (+info)

Magnetic resonance angiographic and three-dimensional computerized tomographic identification of scimitar syndrome in an 8-month-old infant. (5/31)

Scimitar syndrome is a rare congenital abnormality. Clear anatomic definition is an important issue before operation. We report definition of both abnormal pulmonary venous drainage and anomalous systemic arterial supply by gadolinium-enhanced magnetic resonance angiography and three-dimensional computerized tomography in an eight month-old infant with scimitar syndrome. The presented case study confirms that these noninvasive diagnostic tools can successfully be used to provide valuable information about vascular anatomy in infants with scimitar syndrome.  (+info)

Isolated and complex scimitar vein anomalies and their differentiation from the meandering right pulmonary vein. (6/31)

PURPOSE: Four pediatric patients with isolated (the adult form) and complex (the infantile form) scimitar vein anomalies were reviewed and compared with patients with meandering right pulmonary veins. MATERIALS AND METHODS: From January 1990 to December 2006, 4 female patients, aged 2 days to 3.5 years, with isolated and complex scimitar vein anomalies were retrospectively studied. The clinical features, chest radiographs, echocardiography, magnetic resonance imaging, magnetic resonance angiography, computer tomography, bronchography, cardiac catheterization with angiography, surgery, and autopsy were reviewed to substantiate the diagnosis of isolated and complex scimitar vein anomalies. RESULTS: Clinical manifestations were lung infections with radiographic scimitar signs in 4 patients (3 right; 1 left), respiratory distress, congestive heart failure, pulmonary hypertension and/or cyanosis in 3 patients, dextrocardia, heterotaxy, and/or right atrial isomerism in 2 patients, as well as dextroversion, right bronchial isomerism, bronchial stenosis, and/or sepsis in 1 patient. Two patients with right atrial isomerism expired. CONCLUSION: The clinical discrimination between scimitar vein anomaly with and without cardiovascular and bronchopulmonary malformations is crucial for the outcomes vary.  (+info)

Genesis stent implantation without using a long sheath in two children. (7/31)

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Scimitar syndrome. (8/31)

Scimitar syndrome or congenital pulmonary venolobar syndrome is a rare anomaly most commonly consisting of partial pulmonary venous drainage into the hepatic portion of the inferior vena cava, right lung hypoplasia, dextroposition of the heart, and anomalous systemic arterial supply from aorta or one of its branches to the right lung. We report a four-year-old girl with recurrent pneumonia and failure to thrive, who was diagnosed as having scimitar syndrome.  (+info)

Scimitar Syndrome, also known as "congenital venolobar syndrome," is a rare congenital heart defect characterized by the following features:

1. An anomalous pulmonary vein (or veins) that drains into the inferior vena cava or right atrium instead of the left atrium. This vein often has a curved, scimitar-like appearance on imaging studies, hence the name of the syndrome.
2. Hypoplasia (underdevelopment) of the right lung or part of the right lung, which is often associated with abnormalities of the pulmonary artery and bronchial tree in that area.
3. Cardiac shunting, either from left to right (resulting in increased blood flow to the lungs) or right to left (resulting in cyanosis).
4. Other congenital heart defects may also be present, such as atrial septal defect, ventricular septal defect, or patent ductus arteriosus.

Symptoms of Scimitar Syndrome can vary widely depending on the severity of the anomaly and associated cardiac shunting. Mild cases may be asymptomatic, while severe cases can present with respiratory distress, heart failure, or cyanosis in infancy or early childhood. Treatment typically involves surgical correction of the anomalous pulmonary vein and any associated cardiac defects.

Bronchopulmonary sequestration is a rare birth defect of the lungs, in which a mass of abnormal lung tissue develops that doesn't function and isn't connected to the tracheobronchial tree (the airways that lead to the lungs). This means that the abnormal tissue receives its blood supply from an anomalous systemic artery instead of the normal pulmonary circulation. The mass may be located within the lung (intralobar sequestration) or outside the lung (extralobar sequestration), and it can occur on either side of the chest.

Intralobar sequestrations are more common than extralobar sequestrations, accounting for about 75% of cases. They are usually found in adults and are located within a normal lung tissue. Extralobar sequestrations, on the other hand, are typically detected earlier in life (often as an incidental finding during prenatal ultrasound) and are surrounded by their own pleural lining, which can make them appear separate from the normal lung tissue.

Symptoms of bronchopulmonary sequestration may include recurrent respiratory infections, coughing up blood (hemoptysis), shortness of breath, or chest pain. Treatment usually involves surgical removal of the abnormal tissue to prevent complications such as infection, bleeding, or the development of malignancy.

Vascular malformations are abnormalities in the development and growth of blood vessels and lymphatic vessels that can occur anywhere in the body. They can be present at birth or develop later in life, and they can affect both the form and function of the affected tissues and organs. Vascular malformations can involve arteries, veins, capillaries, and/or lymphatic vessels, and they can range from simple, localized lesions to complex, multifocal disorders.

Vascular malformations are typically classified based on their location, size, flow characteristics, and the type of blood or lymphatic vessels involved. Some common types of vascular malformations include:

1. Capillary malformations (CMs): These are characterized by abnormal dilated capillaries that can cause red or pink discoloration of the skin, typically on the face or neck.
2. Venous malformations (VMs): These involve abnormal veins that can cause swelling, pain, and disfigurement in the affected area.
3. Lymphatic malformations (LMs): These involve abnormal lymphatic vessels that can cause swelling, infection, and other complications.
4. Arteriovenous malformations (AVMs): These involve a tangled mass of arteries and veins that can cause high-flow lesions, bleeding, and other serious complications.
5. Combined vascular malformations: These involve a combination of different types of blood or lymphatic vessels, such as capillary-lymphatic-venous malformations (CLVMs) or arteriovenous-lymphatic malformations (AVLMs).

The exact cause of vascular malformations is not fully understood, but they are believed to result from genetic mutations that affect the development and growth of blood vessels and lymphatic vessels. Treatment options for vascular malformations depend on the type, size, location, and severity of the lesion, as well as the patient's age and overall health. Treatment may include medication, compression garments, sclerotherapy, surgery, or a combination of these approaches.

The inferior vena cava (IVC) is the largest vein in the human body that carries deoxygenated blood from the lower extremities, pelvis, and abdomen to the right atrium of the heart. It is formed by the union of the left and right common iliac veins at the level of the fifth lumbar vertebra. The inferior vena cava is a retroperitoneal structure, meaning it lies behind the peritoneum, the lining that covers the abdominal cavity. It ascends through the posterior abdominal wall and passes through the central tendon of the diaphragm to enter the thoracic cavity.

The inferior vena cava is composed of three parts:

1. The infrarenal portion, which lies below the renal veins
2. The renal portion, which receives blood from the renal veins
3. The suprahepatic portion, which lies above the liver and receives blood from the hepatic veins before draining into the right atrium of the heart.

The inferior vena cava plays a crucial role in maintaining venous return to the heart and contributing to cardiovascular function.

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.

A syndrome, in medical terms, is a set of symptoms that collectively indicate or characterize a disease, disorder, or underlying pathological process. It's essentially a collection of signs and/or symptoms that frequently occur together and can suggest a particular cause or condition, even though the exact physiological mechanisms might not be fully understood.

For example, Down syndrome is characterized by specific physical features, cognitive delays, and other developmental issues resulting from an extra copy of chromosome 21. Similarly, metabolic syndromes like diabetes mellitus type 2 involve a group of risk factors such as obesity, high blood pressure, high blood sugar, and abnormal cholesterol or triglyceride levels that collectively increase the risk of heart disease, stroke, and diabetes.

It's important to note that a syndrome is not a specific diagnosis; rather, it's a pattern of symptoms that can help guide further diagnostic evaluation and management.

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.

Scimitar syndrome, or congenital pulmonary venolobar syndrome, is a rare congenital heart defect characterized by anomalous ... The syndrome associated with PAPVR is more commonly known as Scimitar syndrome after the curvilinear pattern created on a chest ... Scimitar syndrome was first described by George Cooper and Raoul Chassinat in 1836 regarding a rare syndrome that has an ... "Scimitar vein draining to the left atrium and a historical review of the scimitar syndrome". Pediatric Radiology. 34 (5): 409- ...
Scimitar syndrome is a rare condition characterised by partial or complete anomalous pulmonary venous drainage to the inferior ...
Scimitar syndrome. A constant feature of Scimitar syndrome is aplasia of one or more lobes of the right lung. Variable features ... Scimitar syndrome. When symptoms of scimitar syndrome are related to anomalous pulmonary venous return (to the inferior vena ... Scimitar syndrome. The constant feature of this syndrome is partial or total anomalous pulmonary venous return to the inferior ... Potter syndrome (with bilateral renal agenesis or dysplasia), abnormalities of the thoracic cage, Scimitar syndrome (right- ...
Living with Scimitar syndrome has reinforced the importance of mental health care and support. Madhumita discusses her ... What is Scimitar Syndrome?. Also known as hypogenetic lung syndrome, Scimitar syndrome is a rare congenital heart defect which ... Home » Scimitar syndrome » Rare Community Profiles: How Madhumitas Scimitar Syndrome Diagnosis Empowered Her to Understand the ... Home » Scimitar syndrome » Rare Community Profiles: How Madhumitas Scimitar Syndrome Diagnosis Empowered Her to Understand the ...
Josué V. CASTRO NETO0; Rubens J. CARVALHO0; Francisco Carlos C. SOUSA0; Marco Aurélio B. AGUIAR0; E. Régis Jucá0 DOI: 10.1590/S0102-76381998000300014. ...
pediatric cardiac surgery, scimitar syndrome, atrial septal defect, pulmonary sequestration, vascular ring ... Case report: A unique quadruple coexisting anomaly-scimitar syndrome, atrial septal defect, vascular ring, and pulmonary ...
Re-Sheathing the Scimitar Syndrome (Articles) Michael Michail, Kaleab N. Asrress, Abhishek Joshi, John A. Giles, Muhammed Z. ...
Chemin A, Bonnet D, Le Bourgeois M, Levy M, Delacourt C. Respiratory outcome in children with scimitar syndrome. J Pediatr. ... Shinohara G, Morita K, Yamashiro M, Hashimoto K. Scimitar syndrome in an infant with right lung hypoplasia, ventricular septa ... Deli T, Kovacs T. Pena Shokei syndrome type1 - combination of polyhydramnios and pulmonary hypoplasia in fetal akinesia. Orv ... for the prevention of mortality and morbidity in paediatric acute lung injury and acute respiratory distress syndrome. Cochrane ...
Repair Scimitar Syndrome. 2600. Valve Insufficiencies. 2610. Tricuspid Valve (see 2312-2314) ...
Schwartz-Jampel syndrome 1 Schwartz-Lelek Syndrome Schweitzer Kemink Graham Syndrome scimitar syndrome + ... urofacial syndrome + A syndrome that is characterized by inverted facial expressions in association with a severe and early- ... Cerebellar Vermis Aplasia with Associated Features suggesting Smith-Lemli-Opitz Syndrome and Meckel Syndrome ... urofacial syndrome (DOID:0050816). Annotations: Rat: (2) Mouse: (2) Human: (2) Chinchilla: (2) Bonobo: (2) Dog: (2) Squirrel: ( ...
... right subclavian artery-esophageal fistula and severe gastrointestinal bleeding after surgical correction of scimitar syndrome ... A new recessive syndrome with VATER-like defects, pulmonary hypertension, abnormal ears, blue sclera, laryngeal webs, and ...
Persistent left superior vena cava - Total anomalous pulmonary venous connection - Scimitar syndrome. ... aortic valves (stenosis, insufficiency, bicuspid) - mitral valves (stenosis, regurgitation) - Hypoplastic left heart syndrome. ... Persistent truncus arteriosus - Double outlet right ventricle (Taussig-Bing syndrome) - Transposition of the great vessels ( ... Ventricular septal defect - Atrial septal defect (Lutembachers syndrome) - Atrioventricular septal defect (Ostium primum) - ...
Lastinger, A., El Yaman, M., Gustafson, R., Yossuck, P. (2013, October). "Scimitar Syndrome and H-Type Tracheoesophageal ...
Atrial Septal Defect, Partial Anomalous Pulmonary Venous Connection, and Scimitar Syndrome. Backer, C. L., Tannous, P. & ... Early myoclonic encephalopathy (ohtahara syndrome). Purohit, R. & Laux, L., Jun 1 2023, Handbook of Pediatric Epilepsy Case ...
Scimitar syndrome. A constant feature of Scimitar syndrome is aplasia of one or more lobes of the right lung. Variable features ... Scimitar syndrome. When symptoms of scimitar syndrome are related to anomalous pulmonary venous return, this return can be ... Scimitar syndrome. The constant feature of this syndrome is partial or total anomalous pulmonary venous return to the inferior ... Potter syndrome (with bilateral renal agenesis or dysplasia), abnormalities of the thoracic cage, Scimitar syndrome (right- ...
... persistent left superior vena cava and scimitar syndrome incidentally found in a CT-scan performed on a female complaining of ... Título: Indirect MR venography for evaluation and therapy planning of patients with Klippel-Trenaunay syndrome ... Título: Circulating osteoprotegerin is increased in metabolic syndrome and associates with subclinical atherosclersosis and ... In elderly patients there were 35 events (15 deaths, 10 acute coronary syndromes, and 10 revascularizations). Patients with ...
Coloboma, Congenital Heart Disease, Ichthyosiform Dermatosis, Impaired Intellectual Development, And Ear Anomalies Syndrome. ... Scimitar Syndrome. Pulmonary artery hypoplasia, Atrial septal defect, Single ventricle, Patent ductus arteriosus, Pa.... ORPHA: ...
Recurrent episodes of lung infections in the first months of life - dont forget Scimitar syndrome. Source: Annual Congress ... Asthma: a complex syndrome of many diseases?. Source: International Congress 2014 - ME09 Asthma: a complex syndrome of many ... Patient testimony: hyper-IgE ?syndrome and associated lung ?infections. Breathe, 15 (4) e131; 10.1183/20734735.0331-2019 You ... Is widal syndrome usually a severe disease?. Source: Eur Respir J 2004; 24: Suppl. 48, 47s. Year: 2004. ...
Hypoplastic left heart associated with scimitar syndrome. McBride, M. E., Huddleston, C. B., Balzer, D. T., Goel, D. & Gazit, A ... Noninvasive Electrocardiographic Imaging (ECGI) of a univentricular heart with Wolff-Parkinson-White syndrome. Ghosh, S., Avari ... Dismal Lung Transplant Outcomes in Children With Tetralogy of Fallot With Pulmonary Atresia Compared to Eisenmenger Syndrome or ... Cardiac memory in patients with Wolff-Parkinson-White syndrome: Noninvasive imaging of activation and repolarization before and ...
Scimitar Syndrome: Definition, Diagnosis, Management and Outcomes. Vladimiro Vida. Padova, Italy. Atrial Septal Defects in ...
In reality, bronchogenic cysts and extrapulmonary sequestrations have been found in association with Scimitar syndrome herbs ... This syndrome is observed in patients who reveal a state of generalized leaky capillaries following shock syndromes, low-move ... Such hereditary most cancers predisposition syndromes embody Fanconi’s anaemia* and Li Fraumeni* syndrome*. Arises from ... The code is identical and the reference for this syndrome is Social and Physical Disability the same difficult asthma ...
Scimitar syndrome: Heart surgeons perform life-changing surgery. Hippocratic Post ,. Healthy diet linked to better mental ...
Surgical technique decision for the treatment of the scimitar syndrome Josué V. CASTRO NETO; Rubens J. CARVALHO; Francisco ...
Scimitar Syndrome - Preferred Concept UI. M0019527. Scope note. An anomalous pulmonary venous return in which the right ... Syndrome du cimeterre Entry term(s):. Anomalous Pulmonary Venous Return. Anomaly, Scimitar. Pulmonary Venous Return Anomaly. ... Scimitar syndrome is named for the crescent- or Turkish sword-like shadow in the chest radiography and is often associated with ... Scimitar syndrome is named for the crescent- or Turkish sword-like shadow in the chest radiography and is often associated with ...
Persistent left superior vena cava - Total anomalous pulmonary venous connection - Scimitar syndrome ... Q87.1) Congenital malformation syndromes predominantly associated with short stature *Aarskog syndrome. *Cockayne syndrome ( ... Q98.1) Klinefelters syndrome, male with more than two X chromosomes. *(Q98.2) Klinefelters syndrome, male with 46,XX ... Pseudohermaphroditism · True hermaphroditism · Mixed gonadal dysgenesis · Swyer syndrome · Persistent Mullerian duct syndrome ...
Ladds procedure in functional single ventricle and heterotaxy syndrome: Does timing affect outcome?. Sharma, M. S., Guleserian ... Management and outcomes of heterotaxy syndrome associated with pulmonary atresia or pulmonary stenosis. Sebastian, V. A., ... Optimization of preoperative status in hypoplastic left heart syndrome with intact atrial septum by left atrial decompression ...
Acute Interstitial Pneumonia (Hamman-Rich Syndrome) in Lung Transplantation: A Case Series. Shepherd, H. M., Terada, Y., ...
Anomalous origin of left coronary artery from right pulmonary artery in association with scimitar syndrome ... Late cardiovascular manifestations of Gregg syndrome: Importance of multimodal imaging approach to optimize diagnosis ...
Three-dimensional echocardiography images showing anomalous pulmonary venous return in an adult with scimitar syndrome. Palios ...

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