OUTCOME OF PATIENTS WITH ECTOPIA CORDIS AND SIGNIFICANT INTRACARDIAC DEFECTS

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 medi cal and surgical era, we reviewed the clinical course, echocardiograms , catheterization data, and operative reports of all patients with ect opia cordis and significant heart disease encountered al Children's Ho spital from 1982 to the present. We identified 13 patients with conotr uncal defects and either thoracic (TEC, n=4) or thoracoabdominal (TAEC , n=9) ectopia cordis. Diagnosis was established in utero in 6, with t ermination of pregnancy in 2 and death before transfer to Children's H ospital in 1. Of 10 patients postnatally managed at Children's Hospita l, 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 h ad procedures as neonates to provide skin coverage over the midline de fect and heart, with concomitant repair of a small omphalocele in 4. T hree underwent successful surgical repair of the cardiac defect, inclu ding ventricular septal defect closure for double-outlet right ventric le (9 months), repair of tetralogy of Fallot with pulmonary atresia (2 .6 years), and repair of double-outlet right ventricle, pulmonary sten osis, and total anomalous pulmonary venous connection to the coronary sinus (3.3 years). Two other patients with TAEC, conoventricular defec ts, and hypoplastic left ventricle underwent single-ventricle palliati on. One patient, at 4 years of age, underwent a right cavopulmonary an astomosis and intra-atrial coronary sinus-to-right superior vena cava baffle. The second patient had a modified Fontan procedure. None of th e 5 survivors had significant extrathoracic defects, whereas all 3 who died by 3 weeks of age had both a large omphalocele and pulmonary hyp oplasia. Two infants with pentalogy of Cantrell, 1 with a large omphal ocele, died of fungal sepsis by 5 weeks of age, and 1 other died intra operatively after repair of pulmonary venous confluence obstruction an d right Blalock-Taussig shunt placement. Conclusions Our experience in dicates that patients with TEC and TAEC and significant intracardiac d efects, in the absence of significant extracardiac defects, can surviv e beyond early infancy and undergo successful cardiac repair or more d efinitive palliation for single-ventricle physiology.