Ectopia Cordis
Pentalogy of Cantrell
Commotio Cordis
Thoracic ectopia cordis with anatomically normal heart. (1/8)
Ectopia cordis is a rare congenital malformation, which is commonly associated with other intracardiac defects. At two-day-old full-term baby girl was admitted to Santa Casade Misericordia Hospital Montes Claros, NG, Brazil, with thoracic ectopia cordis. A transthoracic echocardiographic study did not identify any associated congenital heart diseases. The infant underwent surgical treatment using a rib graft to create a neo-sternum. She was discharged after presenting a good outcome on the 20th postoperative day. (+info)Fontan operation through a right lateral thoracotomy to treat Cantrell syndrome with severe ectopia cordis. (2/8)
(+info)Prenatal diagnosis of ectopia cordis: case report. (3/8)
Ectopia cordis is a rare and impressive malformation presenting as an isolated lesion or as part of the Cantrell's pentology syndrom. It is defined as an anomaly in which the fetal heart lies outside the thoracic cavity. THE AIM of the study is to report the prenatal diagnostic features and management of ectopia cordis. CASE REPORT: We report a prenatal diagnosis case of ectopia cordis using two-dimensional ultrasound at 19 weeks of gestation. Multiple congenital anomalies were found. The most important one was the presence of a ventral thoracoabdominal wall defect with exstrophy of the heart, liver, stomach and intestines. Histopathological examination confirmed the ultrasound findings. Due to severity of the malformations, termination of pregnancy was made. (+info)PHACES syndrome and ectopia cordis. (4/8)
(+info)Rhombencephalosynapsis: a hindbrain malformation associated with incomplete separation of midbrain and forebrain, hydrocephalus and a broad spectrum of severity. (5/8)
(+info)Ectopia cordis in a first-trimester sonographic screening program for aneuploidy. (6/8)
(+info)Fontan operation for the Cantrell syndrome using a clamshell incision. (7/8)
(+info)Ectopia cordis. (8/8)
(+info)Ectopia Cordis is a rare congenital condition in which the heart or a portion of it is located outside the chest wall during fetal development. It is caused by the failure of the anterior chest wall and ventricular septum to close properly, resulting in the heart being exposed on the exterior of the body or covered only by a thin layer of skin. This condition is often associated with other congenital defects, such as cardiac abnormalities, chromosomal anomalies, and genetic syndromes. The severity of ectopia cordis can vary widely, from mild cases where the heart is partially outside the chest to severe cases where it is completely exposed. Treatment typically involves surgical correction, which can be complex due to the presence of other associated defects.
Pentalogy of Cantrell is a rare congenital anomaly that affects the development of the anterior chest wall and abdominal wall, as well as the heart and diaphragm. It is characterized by the presence of five distinct malformations:
1. Midline supraumbilical abdominal wall defect (omphalocele or gastroschisis)
2. Lower sternal cleft
3. Diaphragmatic hernia
4. Ectopia cordis (congenital heart defect with the heart partially or completely outside of the chest)
5. Absence of pericardium (the sac surrounding the heart)
The condition is thought to arise from a failure of lateral fusions during embryonic development, leading to the characteristic pattern of malformations. Pentalogy of Cantrell is often associated with other congenital anomalies and syndromes, and it carries a high mortality rate due to the severity of the cardiac defects.
Ectopia lentis is a medical term that refers to the displacement or malpositioning of the lens in the eye. The lens, which is normally located behind the iris and held in place by tiny fibers called zonules, can become dislocated due to various reasons such as genetic disorders like Marfan syndrome, trauma, or other ocular diseases.
When the lens becomes displaced, it can cause a variety of symptoms including blurry vision, double vision, sensitivity to light, and distorted images. In some cases, ectopia lentis may be asymptomatic and only discovered during a routine eye examination. Treatment for ectopia lentis depends on the severity of the displacement and any associated symptoms. In mild cases, no treatment may be necessary, while in more severe cases, surgery may be required to reposition or remove the lens and replace it with an artificial one.
Commotio cordis is a medical condition that results from a sudden, violent blow to the chest, which can cause the heart to stop beating (cardiac arrest). It usually occurs in young, healthy individuals during sports activities. The impact disrupts the electrical activity of the heart without causing any structural damage to the organ. This disruption can lead to ventricular fibrillation, an abnormal heart rhythm that prevents the heart from pumping blood effectively. If not treated promptly with defibrillation, commotio cordis can result in death within minutes.
'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.
Ectopia cordis
Pentalogy of Cantrell
Situs inversus
List of fetal abnormalities
List of diseases (E)
List of congenital disorders
Ectopia (medicine)
Development of the digestive system
Body cavity
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List of ICD-9 codes 740-759: congenital anomalies
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Omphalocele3
- citation needed] Defects more commonly associated with ectopia cordis include: Intracardiac defects Atrial septal defect Ventricular septal defect Tetralogy of Fallot Tricuspid atresia Double outlet right ventricle Non-cardiac malformations Pentalogy of Cantrell Omphalocele Anterior diaphragmatic hernia Cleft palate The Diagnosis of ectopia cordis is found with a routine ultrasound as early as the first trimester or the beginning of the second trimester. (wikipedia.org)
- Ventral abdominal body wall defects comprise a group of congenital malformations that includes gastroschisis and omphalocele, which are relatively common, and ectopia cordis, bladder exstrophy, and cloacal exstrophy, which are extremely rare. (medscape.com)
- The most severe expression of pentalogy of Cantrell presents at birth with ectopia cordis and omphalocele. (rarediseases.org)
Congenital3
- Ectopia cordis (Greek: "away / out of place" + Latin: "heart") or ectopic heart is a congenital malformation in which the heart is abnormally located either partially or totally outside of the thorax. (wikipedia.org)
- Many cases of ectopia cordis have associated congenital heart defects, in which the heart has failed to properly form. (wikipedia.org)
- Sternal agenesis as well as ectopia cordis are extremely rare congenital malformations. (bvsalud.org)
Fetal1
- The multidisciplinary, expert fetal team from CHOC and UCI gives infants, like Damian with ectopia cordis, the best chance at survival. (choc.org)
Diagnosis1
- In November 2014, Kieran's family received a prenatal diagnosis, learning their little girl would be born with ectopia cordis. (veritysvillage.com)
Thoracic3
- The prognosis of ectopia cordis depends on classification according to three factors: Location of the defect Cervical Thoracic Thoracoabdominal Abdominal Extent of the cardiac displacement Presence or absence of intracardiac defects Some studies have suggested a better prognosis with surgery in cases of thoracoabdominal ectopia cordis or less severe pentalogy of Cantrell. (wikipedia.org)
- It is typically classified according to location of the ectopic heart, which includes: Cervical Thoracic Thoracoabdominal Abdominal Thoracic and thoraco-abdominal ectopia cordis constitute the vast majority of known cases. (wikipedia.org)
- Ectopia cordis is a severe condition in which the heart is completely or partially displaced outside of the thoracic cavity and therefore not protected by the chest wall. (rarediseases.org)
Pentalogy1
- Ectopia cordis is frequently, but not always associated with pentalogy of Cantrell. (rarediseases.org)
Births1
- The occurrence of ectopia cordis is 8 per million births. (wikipedia.org)
Chest2
- Ectopia cordis results from a failure of proper maturation of midline mesoderm and ventral body wall (chest) formation during embryonic development. (wikipedia.org)
- Marcela initially had the first of two surgical procedures in September 2021 at Boston Children's Hospital to correct ectopia cordis - a rare, usually fatal disorder in which babies are born with their hearts outside their chest. (foodforthepoor.org)
Case2
- Is it a case of ectopia cordis? (contemporaryobgyn.net)
- No. 31 was probably a case of ectopia cordis. (jhngg.com)
Condition1
- In humans, this condition is called ectopia cordis, but in veterinary medicine there is no name for the life-threatening genetic defect. (crystalinks.com)
Treatment1
- Due to the rarity and rapid postpartum mortality of ectopia cordis, limited treatment options have been developed. (wikipedia.org)
Baby1
- Born with ectopia cordis, baby prepares for multiple corrective surgeries by a large team of CHOC experts. (choc.org)
Diaphragmatic2
- citation needed] Defects more commonly associated with ectopia cordis include: Intracardiac defects Atrial septal defect Ventricular septal defect Tetralogy of Fallot Tricuspid atresia Double outlet right ventricle Non-cardiac malformations Pentalogy of Cantrell Omphalocele Anterior diaphragmatic hernia Cleft palate The Diagnosis of ectopia cordis is found with a routine ultrasound as early as the first trimester or the beginning of the second trimester. (wikipedia.org)
- The syndrome includes five features: 1) a defect in sternal fusion, resulting in ectopia cordis, 2) a diaphragmatic hernia, 3) a weakened abdominal wall, oresulting in an omphalocele, 4) a missing pericardium, and 5) structural and valvular defects in the heart. (nih.gov)
Thoracoabdominal3
- The prognosis of ectopia cordis depends on classification according to three factors: Location of the defect Cervical Thoracic Thoracoabdominal Abdominal Extent of the cardiac displacement Presence or absence of intracardiac defects Some studies have suggested a better prognosis with surgery in cases of thoracoabdominal ectopia cordis or less severe pentalogy of Cantrell. (wikipedia.org)
- It is typically classified according to location of the ectopic heart, which includes: Cervical Thoracic Thoracoabdominal Abdominal Thoracic and thoraco-abdominal ectopia cordis constitute the vast majority of known cases. (wikipedia.org)
- Khaled S, Olivier G, Dominique M, Bernard K. Ectopia cordis: A successful single stage thoracoabdominal repair. (jiaps.com)
Cloacal exstrophy1
- Ventral abdominal body wall defects comprise a group of congenital malformations that includes gastroschisis and omphalocele, which are relatively common, and ectopia cordis, bladder exstrophy, and cloacal exstrophy, which are extremely rare. (medscape.com)
Sternal1
- PHACES is an acronym denoting major features of the disorder: Posterior fossa malformations (such as Dandy-Walker cysts), hemangioma of the cervicofacial region, arterial anomalies (usually head or neck), cardiac anomalies (such as Coarctation of the aorta), ocular anomalies, and sternal or abdominal clefting or ectopia cordis. (eyewiki.org)
Extrathoracic1
- One fetus afflicted with extrathoracic ectopia cordis was recovered from a dam exposed to 8,000 ppm acetonitrile. (nih.gov)
Ventral2
- Ectopia cordis results from a failure of proper maturation of midline mesoderm and ventral body wall (chest) formation during embryonic development. (wikipedia.org)
- Dr. Adelstein and colleagues first studied mutations in MYH10 that lead to strikingly abnormal heart development in the mouse-the heart develops outside of the body (ectopia cordis) due to a failure of ventral wall closure. (nih.gov)
Cleft1
- Ectopia cordis thoracalis with facial cleft. (jiaps.com)
Partial1
- A case of partial ectopia cordis and umbilical hernia. (nih.gov)
Cervical1
- Shows theoretical and practical importance: Acardia total lack of heartlack of closing the pericardial sac, diplocardia double heart multiplicitas cordis multiple cordsdextrocardia heart on the right side of the mediastinumcardiac ectopia presence of heart in the cervical region, pectoral or abdominaletc. (asspub.ro)
Heart1
- In Guatemala, the chances of survival were slim for a sweet baby named Marcela because of ectopia cordis, a rare condition in which a baby is born with a heart partially or entirely outside the chest cavity. (foodforthepoor.org)
Term1
- Haller first described the term ectopia cordis in 1706. (jiaps.com)
Cases2
- In general, the prognosis for ectopia cordis is poor-most cases result in death shortly after birth due to infection, hypoxemia, or cardiac failure. (wikipedia.org)
- Ectopia Cordis: Two Different Cases with Literature Review', J Card Crit Care TSS. (yashodahealthcare.com)
Found1
- Ectopia cordis (EC) is often found in fetuses with POC. (globalgenes.org)
Results1
- Your search for Ectopia cordis did not return any results. (nih.gov)