on July 17, 2006
Published online before print July 17, 2006, doi: 10.1161/CIRCULATIONAHA.105.548016
| |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Submitted on March 8, 2005
From Stanford University (D.B., C.C., P.G.), Stanford, Calif; The University of Alabama (D.N., J.K.K.), Birmingham; Columbia University (L.J.A., D.H.), New York, NY; Children’s Hospital Boston and Harvard Medical School (E.D.B.), Boston, Mass; Washington University (C.E.C.), St. Louis, Mo; and The University of Arkansas (W.R.M.), Little Rock. * To whom correspondence should be addressed. E-mail: danb{at}stanford.edu.
Background--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. Methods and Results--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. Conclusions--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.
Revised on March 14, 2006
Accepted on May 5, 2006
Outcome of Listing for Cardiac Transplantation for Failed Fontan. A Multi-Institutional Study
D. Bernstein MD*,
Key words: heart transplantation • heart failure, congestive • cardiomyopathies • protein-losing enteropathies • heart defects, congenital
This article has been cited by other articles:
 |
|
 |
|
  Y. Kotani, S. Kasahara, Y. Fujii, K. Yoshizumi, Y. Oshima, S.-i. Otsuki, T. Akagi, and S. Sano Clinical outcome of the Fontan operation in patients with impaired ventricular function Eur. J. Cardiothorac. Surg., October 1, 2009; 36(4): 683 - 687. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 D. T. Hsu and G. D. Pearson Heart Failure in Children: Part II: Diagnosis, Treatment, and Future Directions Circ Heart Fail, September 1, 2009; 2(5): 490 - 498. [Full Text] [PDF]
|
|
|
|
 |
|
 E. R. Griffiths, A. K. Kaza, M. C. Wyler von Ballmoos, H. Loyola, A. M. Valente, E. D. Blume, and P. del Nido Evaluating failing Fontans for heart transplantation: predictors of death. Ann. Thorac. Surg., August 1, 2009; 88(2): 558 - 563. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 J. M. Lamour, K. R. Kanter, D. C. Naftel, M. R. Chrisant, W. R. Morrow, B. S. Clemson, J. K. Kirklin, and CTRD (Cardiac Transplant Registry Database) and th The Effect of Age, Diagnosis, and Previous Surgery in Children and Adults Undergoing Heart Transplantation for Congenital Heart Disease. J. Am. Coll. Cardiol., July 7, 2009; 54(2): 160 - 165. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. J. Silka and Y. Bar-Cohen Should patients with congenital heart disease and a systemic ventricular ejection fraction less than 30% undergo prophylactic implantation of an ICD?: Patients With Congenital Heart Disease and a Systemic Ventricular Ejection Fraction Less Than 30% Should Undergo Prophylactic Implantation of an Implantable Cardioverter Defibrillator Circ Arrhythm Electrophysiol, October 1, 2008; 1(4): 298 - 306. [Full Text] [PDF]
|
|
|
|
|
|
S. A. Hunt and F. Haddad The Changing Face of Heart Transplantation J. Am. Coll. Cardiol., August 19, 2008; 52(8): 587 - 598. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
C. Mavroudis, B. J. Deal, C. L. Backer, R. D. Stewart, W. H. Franklin, S. Tsao, K. M. Ward, and R. A. DeFreitas 111 Fontan Conversions with Arrhythmia Surgery: Surgical Lessons and Outcomes Ann. Thorac. Surg., November 1, 2007; 84(5): 1457 - 1466. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
C. S.D. Almond, J. E. Mayer Jr, R. R. Thiagarajan, E. D. Blume, P. J. del Nido, and D. B. McElhinney Outcome After Fontan Failure and Takedown to an Intermediate Palliative Circulation Ann. Thorac. Surg., September 1, 2007; 84(3): 880 - 887. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
T. P. Graham Jr The Year in Congenital Heart Disease J. Am. Coll. Cardiol., July 24, 2007; 50(4): 368 - 377. [Full Text] [PDF]
|
|
|
|
 |
|
 C. E. Canter, R. E. Shaddy, D. Bernstein, D. T. Hsu, M. R.K. Chrisant, J. K. Kirklin, K. R. Kanter, R. S.D. Higgins, E. D. Blume, D. N. Rosenthal, et al. Indications for Heart Transplantation in Pediatric Heart Disease: A Scientific Statement From the American Heart Association Council on Cardiovascular Disease in the Young; the Councils on Clinical Cardiology, Cardiovascular Nursing, and Cardiovascular Surgery and Anesthesia; and the Quality of Care and Outcomes Research Interdisciplinary Working Group Circulation, February 6, 2007; 115(5): 658 - 676. [Abstract] [Full Text] [PDF]
|
|