Ectopia Cordis

A rare developmental defect in which the heart is abnormally located partially or totally outside the THORAX. It is the result of defective fusion of the anterior chest wall. Depending on the location of the heart, ectopia cordis can be thoracic, thoracoabdominal, abdominal, and cervical.

Also Known As:

Networked: 45 relevant articles (0 outcomes, 1 trials/studies)

Disease Context: Research Results

Related Diseases

1.	Pentalogy of Cantrell
2.	Umbilical Hernia (Omphalocele)
3.	Gastroschisis
4.	CHARGE Syndrome
5.	Tetralogy of Fallot (Fallot Tetralogy)

Experts

1.	Tyl, R W: 1 article (11/2014)
2.	Trimmer, G W: 1 article (11/2014)
3.	Schreiner, C A: 1 article (11/2014)
4.	Marr, M C: 1 article (11/2014)
5.	Roberts, L G: 1 article (11/2014)
6.	Hoffman, G M: 1 article (11/2014)
7.	Gray, T M: 1 article (11/2014)
8.	Clark, C R: 1 article (11/2014)
9.	Murray, F J: 1 article (11/2014)
10.	Cerqua, Amanda: 1 article (07/2014)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Ectopia Cordis:

1.	Verapamil (Calan)FDA LinkGeneric 06/01/1990 - "Ectopia cordis was observed during a study on the effects of a calcium antagonist (verapamil) on chicken embryo heart development. "
2.	CalciumIBA 06/01/1990 - "Ectopia cordis was observed during a study on the effects of a calcium antagonist (verapamil) on chicken embryo heart development. "
3.	Sternal cleftIBA 01/01/2009 - "The authors report a clinical case of a full-term female neonate with congenital sternal cleft and partial ectopia cordis. " 01/01/2009 - "Congenital medium sternal cleft with partial ectopia cordis repair." 05/01/2007 - "Congenital sternal cleft in an adult male not associated with cardiac defects or ectopia cordis." 02/01/1999 - "Sternal cleft and ectopia cordis: a case report." 10/01/1998 - "This article reviews 8 cases of sternal cleft not associated with ectopia cordis in patients presenting between October 1979 and November 1997. "
4.	Dihydrotachysterol (AT 10)IBA 08/01/1997 - "We report here the earliest prenatal diagnosis to date of a case of ectopia cordis using both two-dimensional and three-dimensional ultrasound at 10 weeks of gestation. " 08/01/1997 - "Prenatal diagnosis of ectopia cordis at 10 weeks of gestation using two-dimensional and three-dimensional ultrasonography." 06/01/2011 - "A case of ectopia cordis (EC) with gastroschisis in a 27-year-old primigravida was diagnosed at 10(3/7) weeks of gestation. " 02/01/2009 - "In case 1, sonographic evaluation revealed ectopia cordis, omphalocele, and cystic hygroma at 10 weeks' gestation. " 11/01/2014 - "G/MTBE exposure did not produce statistically significant maternal or developmental effects; however, two uncommon ventral wall closure defects occurred: gastroschisis (1 fetus at 10,000mg/m(3)) and ectopia cordis (1 fetus at 2000mg/m(3); 2 fetuses/1 litter at 10,000mg/m(3)). "
5.	AminopropionitrileIBA 10/01/1997 - "The characteristic features of the Cantrell-sequence--anterior thoraco-abdominal wall defect with ectopia cordis and diaphragm, sternum, pericardium, and heart defects--have been observed in animals following maternal administration of beta-aminopropionitrile, a toxic amino-acid derivative. " 04/01/1972 - "Ectopia cordis (ectocardia) and gastroschisis induced in rats by maternal administration of the lathyrogen, beta-aminopropionitrile (BAPN)."
6.	EctrodactylyIBA 03/15/1991 - "The malformations include a first and second branchial arch sequence, ectopia cordis with congenital heart defect, caudal "regression" sequence with absent sacrum and hypoplastic right femur, ectrodactyly, left radial abnormality, islet cell hyperplasia, and skin lesions. " 11/01/1998 - "An infant presented with multiple congenital anomalies including a midline thoracoabdominal defect, absence of the sternum, ectopia cordis, right diaphragmatic hernia, right anophthalmia, left microphthalmia, incomplete bilateral cleft lip, and various limb defects including ectrodactyly of the right hand and left foot, and phocomelia of the right lower extremity. "
7.	SmokeIBA 07/01/2009 - "Exposure of smoke solutions from CNG-powered four-stroke auto-rickshaws induces distressed embryonic movements, embryonic hemorrhaging and ectopia cordis."
8.	PseudocholinesteraseIBA 03/01/1986 - "The analysis of 340 amniotic fluids of normal pregnancies indicates that the normal value for AChE is 5.17 +/- 2.63 mU/ml (97% confidence interval for the mean 4.84-5.49 mU/ml. A group of 27 abnormal pregnancies provides evidence that fetal vomiting and regurgitation, fetal demise, multiple cysts syndrome, idiopathic IUGR, arthrogryposis multiplex, hydrocephaly (stenosis of aqueductus), trisomy 21, trisomy 18, hydronephrosis, pyloric stenosis, heart malformation, ectopia cordis and multiple gestation produce elevated levels of pseudocholinesterase (PChE) in amniotic fluid. "
9.	Polytetrafluoroethylene (Teflon)FDA Link 02/01/1995 - "We present a case report of a patient with thoracic ectopia cordis repaired in a single stage using polytetrafluoroethylene membrane and skin for coverage and closure of the heart and thoracic defect."
10.	Polyglycolic Acid (Polyglycolide)IBA 06/01/2012 - "We present a case of a 10-month-old male infant with thoracoabdominal ectopia cordis, as part of Cantrell pentad, repaired using a poly-L-lactic-polyglycolic acid plate, a resorbable plating system widely used in craniomaxillofacial reconstruction. " 06/01/2012 - "Repair of ectopia cordis using a resorbable poly-L-lactic-polyglycolic acid plate in a patient with pentalogy of Cantrell."

Therapies and Procedures

1.	Fontan Procedure (Fontan Operation) 10/01/2013 - "We report a patient with ectopia cordis, functionally single ventricle and bilateral superior vena cava, who underwent a staged Fontan procedure through a clamshell incision and the sternothoracotomy approach. " 02/01/2001 - "Thus, ectopia cordis is not a contraindication for a Fontan operation." 02/01/2001 - "Successful staged-Fontan operation in a patient with ectopia cordis." 04/01/2008 - "Fontan operation through a right lateral thoracotomy to treat Cantrell syndrome with severe ectopia cordis."
2.	Sternotomy 10/01/2013 - "A median sternotomy could be difficult for a child with ectopia cordis and complex congenital cardiac anomalies. " 04/01/2008 - "A median sternotomy would be very difficult for Cantrell syndrome with severe ectopia cordis. "
3.	Blalock-Taussig Procedure 04/01/2008 - "For Cantrell syndrome and tricuspid atresia after left modified Blalock-Taussig shunt with severe ectopia cordis, defect in the middle and inferior portion of the sternum, and the closing of ventriculo-peritoneal shunt, we performed extracardiac total cavopulmonary connection through a right lateral thoracotomy after establishing right modified Blalock-Taussig shunt and performing coil embolization of left modified Blalock-Taussig shunt by cardiologists."
4.	Ventriculoperitoneal Shunt 04/01/2008 - "For Cantrell syndrome and tricuspid atresia after left modified Blalock-Taussig shunt with severe ectopia cordis, defect in the middle and inferior portion of the sternum, and the closing of ventriculo-peritoneal shunt, we performed extracardiac total cavopulmonary connection through a right lateral thoracotomy after establishing right modified Blalock-Taussig shunt and performing coil embolization of left modified Blalock-Taussig shunt by cardiologists."
5.	Tissue Expansion 01/01/1995 - "Repair of thoracoabdominal ectopia cordis with myocutaneous flaps and intraoperative tissue expansion."