Abstract
A 9-month-old female infant was diagnosed with incomplete Cantrell syndrome with complete ectopia cordis accompanied by congenital heart defects including tetralogy of Fallot, cor triatriatum, and an unroofed coronary sinus. She was provided coverage for her heart with split thickness skin grafts after birth, and she underwent intracardiac repair and chest wall reconstruction using musculocutaneous flaps 9 months later. The long-term use of cardiopulmonary bypass for dissecting adhesions of the heart to the chest wall and repairing the complex congenital heart defects in the malposition contributed to coagulopathy and myocardial edema. The latter caused cardiac ventricular diastolic dysfunction, leading to decreased cardiac output on covering the sternal defect with a polytetrafluoroethylene sheet. Continuous epinephrine infusion and release of a part of the coverage preserved the cardiac output. Reconstruction of the chest wall was accomplished postoperatively using musculocutaneous flaps on day 11 after improving the myocardial edema with continuous hemodiafiltration.
