Outcome After Orthotopic Cardiac Transplantation in Adults With Congenital Heart Disease Jacqueline M. Lamour, MD; Linda J. Addonizio, MD; Mark E. Galantowicz, MD; Jan M. Quaegebeur, MD; Donna M. Mancini, MD; Maryanne R. Kichuk, MD; Ainat Beniaminovitz, MD; Robert E. Michler, MD; Alan Weinberg, MS; Daphne T. Hsu, MD Background—Advances in surgical and medical management have greatly improved long-term survival rates in patients with congenital heart disease (CHD). As these patients reach adulthood, myocardial dysfunction can occur, leading to cardiac transplantation. Methods and Results—We reviewed the pretransplantation and posttransplantation courses of 24 patients 18 years old (mean age, 26 years; range, 18 to 56 years) with CHD who received a transplant between January 1985 and September 1998. The relation between preoperative and perioperative risk factors for complications and death was assessed. Single ventricle was the pretransplantation diagnosis for 12 patients (50%), and d-transposition of the great vessels was the diagnosis for 4 patients (16%). Twenty-two patients had a mean of 2 previous operations. At cardiac transplantation, additional surgical procedures were required to correct extracardiac lesions in 18 patients (75%). Refractory heart failure was present in 22 patients, significant cyanosis was present in 7, and protein-losing enteropathy was present in 4. There were 5 early deaths due to bleeding (n=3) and infection (n=2). The Kaplan-Meier survival rate after cardiac transplantation was 79% at 1 year and 60% at 5 years . No anatomic or surgical risk factor was predictive of death. The outcome of patients with CHD who received a transplant was compared with that for patients without CHD (n=788). Mean bypass and ischemic times were significantly longer in patients with CHD than in patients without CHD. Survival rates after transplantation did not differ significantly between patients with and those without CHD (P=0.83). Conclusions—Successful cardiac transplantation is obtainable in adults with complex CHD, with an outcome similar to that of patients without CHD. A detailed assessment of cardiac anatomy and careful surgical planning are essential to the pretransplantation and posttransplantation management of these patients. (Circulation. 1999;100[suppl II]:II-200 –II-205.) Key Words: transplantation  heart defects, congenital  risk factors A dvances in surgical and medical management have greatly improved the long-term survival rates of patients with congenital heart disease (CHD); however, late myocar- dial dysfunction can occur after palliative or corrective surgery and is the most common cause of decline and death in patients with CHD. 1 Penkoske et al 2 estimated that of all patients with CHD, 10% to 20% will be potential candidates to receive a heart or heart-lung transplant at some time during their lifetime. This group of patients presents multiple unique surgical and medical challenges owing to their complex anatomy, prior palliative and corrective procedures, and overall debilitated condition. Elevated pulmonary vascular resistance due to years of long-standing congestive heart failure may further complicate cardiac transplantation, in- creasing the risk of donor right heart failure. 3 Although there have been multiple publications reporting the outcomes in children with CHD after cardiac transplantation, 4–6 few reports in the literature have focused on transplantation in the adult with complex CHD. 7 The purpose of our study was to describe the pretransplan- tation and posttransplantation courses of adults with CHD who undergo cardiac transplantation, to assess potential risk factors for a poor outcome, and to compare the posttransplan- tation outcome of adult patients with CHD with that of adult patients without CHD. Methods Patients Eight hundred two patients underwent primary orthotopic cardiac transplantation at our institution between January 1985 and Septem- ber 1998. Twenty-four (3%) were 18 years old and had CHD. Pretransplantation variables that were assessed included anatomic diagnosis, previous surgical and catheterization interventions, indi- cations for transplantation, United Network for Organ Sharing (UNOS) status, and pulmonary vascular resistance. Operative and From the Department of Pediatrics, Medicine and Surgery, College of Physicians and Surgeons, Columbia University, New York, NY. Dr Kichuk’s current address is Department of Pediatrics, Cleveland Clinic, Pediatric Cardiology Desk M-41, 9500 Euclid Ave, Cleveland, Ohio 44195. Dr Michler’s current address is Department of Surgery, Ohio State University, North Dorn Hall, 410 West 10th Ave, Columbus, Ohio 43210. Correspondence to Jacqueline M. Lamour, MD, BCH 2 North, 3959 Broadway, New York, NY 10032. E-mail jml14@columbia.edu © 1999 American Heart Association, Inc. Circulation is available at http://www.circulationaha.org II-200 Thoracic Transplantation and Ventricular Assist Devices by guest on April 8, 2017 http://circ.ahajournals.org/ Downloaded from