Heart Transplantation in Adults With Congenital Heart Disease: 100% Survival With Operations Performed by a Surgeon Specializing in Congenital Heart Disease in an Adult Hospital.

BACKGROUND

Cardiac transplantation in adult patients with congenital heart disease poses numerous challenges. The optimal operative and postoperative management strategies remain unclear. The purpose of our study was to (1) characterize the adult patient with a congenital heart condition undergoing transplantation, the operation, and the postoperative course; (2) report the survival after heart transplantation at our center; and (3) discuss issues surrounding the unique setting we provide for the operative and postoperative care of this complex patient cohort.

METHODS

We performed a retrospective cohort study of 12 consecutive adult patients with a prior history of congenital heart disease who underwent heart transplantation at a single, large, academic center between September 1, 2005, and September 1, 2013. The operations were performed by a surgeon specializing in congenital heart disease in an adult hospital. Postoperative care was provided jointly by that surgeon and the adult cardiac transplantation team.

RESULTS

At operation, the median age and weight were 41 years (range, 16 to 72 years) and 65 kg (range, 45 to 104 kg), and 100% of patients had undergone previous operations. The median donor ischemic time was 197 minutes (range, 137 to 282 minutes). The median cardiopulmonary bypass time was 210 minutes (range, 175 to 457 minutes), and the median total operating time was 582 minutes (range, 389 to 968 minutes). Three patients required mechanical support to be weaned from cardiopulmonary bypass. Postoperatively, 3 patients required the addition of mechanical support in the intensive care unit, and 3 patients required tracheostomy for prolonged ventilation. The majority of patients had a complicated postoperative course (66%). The median number of noncardiac consultants required to help care for these patients was four (range, two to 12). The mortality was 0%.

CONCLUSIONS

Cardiac transplantation in adults with congenital cardiac disease is challenging, is fraught with adverse events, and requires meticulous care and teamwork for success. A surgeon specializing in congenital heart conditions may be best to handle the operative challenges, and an adult hospital with access to certain technology and consultant services may be best to handle the postoperative challenges in this difficult patient population.