Outcome of listing for cardiac transplantation for failed fontan : A multi-institutional study

• Published in: Circulation (New York, N.Y.) Vol. 114; no. 4; pp. 273 - 280
• Main Authors: BERNSTEIN, D, NAFTEL, D, CHIN, C, ADDONIZIO, L. J, GAMBERG, P, BLUME, E. D, HSU, D, CANTER, C. E, KIRKLIN, J. K, MORROW, W. R
• Format: Journal Article
• Language: English
• Published: Hagerstown, MD Lippincott Williams & Wilkins 25.07.2006
• Subjects: Adolescent; Biological and medical sciences; Blood and lymphatic vessels; Cardiology. Vascular system; Cardiovascular system; Cause of Death; Child; Child, Preschool; Diseases of the peripheral vessels. Diseases of the vena cava. Miscellaneous; Fontan Procedure; Heart; Heart Diseases - complications; Heart Diseases - congenital; Heart Diseases - mortality; Heart Diseases - surgery; Heart failure, cardiogenic pulmonary edema, cardiac enlargement; Heart Transplantation - adverse effects; Heart Transplantation - mortality; Humans; Infant; Medical sciences; Pharmacology. Drug treatments; Protein-Losing Enteropathies - etiology; Respiration, Artificial; Retrospective Studies; Salvage Therapy - adverse effects; Salvage Therapy - methods; Salvage Therapy - mortality; Survival Rate; Treatment Failure; Treatment Outcome; Vasodilator agents. Cerebral vasodilators; Heart failure; Prognosis; protein-losing enteropathies; Cardiomyopathy; heart transplantation; Cardiovascular disease; Myocardial disease; Protein losing enteropathy; heart failure, congestive; Intestinal malabsorption; Digestive diseases; Intestinal disease; heart defects, congenital; Congenital cardiopathy; cardiomyopathies
• ISSN: 0009-7322; 1524-4539
• DOI: 10.1161/CIRCULATIONAHA.105.548016

The Fontan procedure is a successful palliation for children with single-ventricle physiology; however, many will eventually require heart transplantation. The purpose of this study was to determine risk factors for death awaiting transplantation and to examine results after transplantation in Fontan patients. A retrospective, multi-institutional review was performed of 97 Fontan patients <18 years of age listed at 17 Pediatric Heart Transplant Study centers from 1993 to 2001. Mean age at listing was 9.7 years (0.5 to 17.9 years); 25% were <4 years old; 53% were United Network for Organ Sharing status 1; 18% required ventilator support. Pretransplantation survival was 78% at 6 months and 74% at 12 months and was similar to 243 children with other congenital heart disease (CHD) and 747 children without congenital heart disease (No-CHD), who were also awaiting transplantation. Patients who were younger, status 1, had shorter interval since Fontan, or were on a ventilator were more likely to die while waiting. At 6 months, the probability of receiving a transplant was similar for status 1 and 2 (65% versus 68%); however, the probability of death was higher for status 1 (22% versus 5%). Seventy patients underwent transplantation. Survival was 76% at 1 year, 70% at 3 years, and 68% at 5 years, slightly less than CHD and No-CHD patients. Causes of death included infection (30%), graft failure (17%), rejection (13%), sudden death (13%), and graft coronary artery disease (9%). Protein-losing enteropathy (present in 34 patients) resolved in all who survived >30 days after transplantation. Heart transplantation is an effective therapy for pediatric patients with a failed Fontan. Although early posttransplantation survival is slightly lower than other patients with CHD, long-term results are encouraging, and protein-losing enteropathy can be expected to resolve.