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Outcome of patients with ectopia cordis and significant intracardiac defects
L K Hornberger1, S D Colan, J E Lock
1Department of Pediatrics, Hospital for Sick Children, Toronto, Ontario, Canada.
Insights
Ectopia cordis with significant heart defects has a poor prognosis, but survival is possible with early intervention. Modern surgical techniques offer improved outcomes for selected patients with thoracic or thoracoabdominal ectopia cordis.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Surgery
- Neonatal Medicine
Background:
- Ectopia cordis, a rare congenital anomaly, is often associated with severe heart defects and a historically poor prognosis.
- Previous reports highlight the challenges in managing patients with ectopia cordis and significant intracardiac abnormalities.
Purpose of the Study:
- To evaluate the outcomes of patients with ectopia cordis and significant congenital heart disease in a contemporary medical and surgical setting.
- To identify factors influencing survival and successful management in this complex patient population.
Main Methods:
- Retrospective review of clinical courses, echocardiograms, catheterization data, and operative reports for 13 patients with ectopia cordis and conotruncal defects (1982-present).
- Analysis included patients with thoracic ectopia cordis (TEC) and thoracoabdominal ectopia cordis (TAEC).
- Assessment of postnatal management, surgical interventions, and long-term follow-up.
Main Results:
- Ten patients were managed postnatally; 5 survived beyond infancy (4/8 TAEC, 1/2 TEC).
- Survivors underwent initial skin coverage procedures and, in some cases, cardiac repair or single-ventricle palliation.
- Deaths were associated with significant extracardiac defects like large omphaloceles and pulmonary hypoplasia.
Conclusions:
- Patients with thoracic or thoracoabdominal ectopia cordis and significant intracardiac defects, without major extracardiac anomalies, can achieve long-term survival.
- Successful cardiac repair or definitive single-ventricle palliation is feasible for selected survivors.
- Early management focusing on defect closure and cardiac intervention improves prognosis.
Background:
Previous reports suggest a poor prognosis for patients with ectopia cordis and significant congenital heart disease.
Methods And Results:
To determine the outcome of affected patients in a recent medical and surgical era, we reviewed the clinical course, echocardiograms, catheterization data, and operative reports of all patients with ectopia cordis and significant heart disease encountered at Children's Hospital from 1982 to the present. We identified 13 patients with conotruncal defects and either thoracic (TEC, n = 4) or thoracoabdominal (TAEC, n = 9) ectopia cordis. Diagnosis was established in utero in 6, with termination of pregnancy in 2 and death before transfer to Children's Hospital in 1. Of 10 patients postnatally managed at Children's Hospital, 4 of 8 with TAEC and 1 of 2 with TEC survived beyond infancy, with an age at most recent follow-up of 3.5 to 9.8 years. All 5 survivors had procedures as neonates to provide skin coverage over the midline defect and heart, with concomitant repair of a small omphalocele in 4. Three underwent successful surgical repair of the cardiac defect, including ventricular septal defect closure for double-outlet right ventricle (9 months), repair of tetralogy of Fallot with pulmonary atresia (2.6 years), and repair of double-outlet right ventricle, pulmonary stenosis, and total anomalous pulmonary venous connection to the coronary sinus (3.3 years). Two other patients with TAEC, conoventricular defects, and hypoplastic left ventricle underwent single-ventricle palliation. One patient, at 4 years of age underwent a right cavopulmonary anastomosis and intra-atrial coronary sinus-to-right superior vena cava baffle. The second patient had a modified Fontan procedure. None of the 5 survivors had significant extrathoracic defects, whereas all 3 who died by 3 weeks of age had both a large omphalocele and pulmonary hypoplasia. Two infants with pentalogy of Cantrell, 1 with a large omphalocele, died of fungal sepsis by 5 weeks of age, and 1 other died intraoperatively after repair of pulmonary venous confluence obstruction and right Blalock-Taussig shunt placement.
Conclusions:
Our experience indicates that patients with TEC and TAEC and significant intracardiac defects, in the absence of significant extracardiac defects, can survive beyond early infancy and undergo successful cardiac repair or more definitive palliation for single-ventricle physiology.