Complete Ectopia Cordis: Successful Two-Stage Repair

Objectives: Ectopia cordis (EC) is an extremely rare congenital defect with the estimated prevalence of 5.5 to 7.9 per million live births. The heart is, either partially or completely, positioned outside of the thoracic cavity. EC can appear sporadic or as part of the pentalogy of Cantrell. Prognosis of EC is very poor: most children die in utero or short after birth. Herein we report a case of complete EC that was associated with sternum aplasia, a secundum atrial septal defect (ASDII), a wide open persisting ductus arteriosus (PDA) and a persisting left superior vena cava (PLSVC). No visceral abnormalities could be found.

Methods: A female neonate was born after 36 weeks of gestation with an antenatal diagnosis of a thoracic malformation. The EC was immediately covered and the child was transferred to pediatric cardiac intensive care unit. At the age of 2 days, cardiac surgery was performed with the aim to create space for the heart and to cover and protect it. Finally, a thick 1-mm PTFE membrane was fixed to the subcutaneous tissue to close the thorax ventrally. The postoperative wound care included repetitive sterile dressing changes. We noticed that the heart, which was approximately 3 cm over the skin level at birth, sank slowly into the chest. Nine weeks later second operation was performed. The tissue was carefully mobilized until the fascia could be sutured, a thick 1-mm PTFE membrane was connected to the subcuticular layer to protect the heart and to add stability to the thoracic wall and the skin was closed.

Result: The postoperative course was uneventful. Patient was discharged home 13 weeks after the first surgery, in good condition without any cardiac medication supported only by high-flow without oxygen support. The chest was plane and the wound closed.

Conclusion: Our first priority was to protect the ectopic heart from infection and systemic and pulmonary blood flow compromise due to external pressure or torsion of the heart. With the time, the body created space for the heart, which sank into the chest by itself without hemodynamic or respiratory compromise. Finally the thoracic fascia could be closed and the skin was adapted which will allow the patient to grow. In future we expect to perform at least one more surgery to perform a thoracic reconstruction plasty to protect the heart and to achieve a nearly normal chest wall. The success of this simple and reproducible treatment is rooted in the stepwise adaptation of the mediastinal cavity to contain the heart with no compression by using synthetic membranes which function as a complementing container, as well as a barrier.