Outcomes of Pediatric Patients With Hypertrophic Cardiomyopathy Listed for Transplant

Gajarski, Robert; Naftel, David C.; Pahl, Elfriede; Alejos, Juan; Pearce, F. Bennett; Kirklin, James K.; Zamberlan, Mary; Dipchand, Anne I.; Pediatric Heart Transplant Study Investigators,

doi:10.1016/j.healun.2009.05.028

« Previous Next »The Journal of Heart and Lung Transplantation
Volume 28, Issue 12 , Pages 1329-1334, December 2009

Outcomes of Pediatric Patients With Hypertrophic Cardiomyopathy Listed for Transplant

Robert Gajarski, MD
- Division of Pediatric Cardiology, C.S. Mott Children's Hospital, Ann Arbor, Michigan
- Reprint requests: Robert J. Gajarski, MD, University of Michigan, Division of Pediatric Cardiology, C.S. Mott Children's Hospital, L1242 Women's, Box 0204, 1500 E. Medical Center Dr, Ann Arbor, MI 48109. Telephone: 734-615-2369. Fax: 734-936-9470
David C. Naftel, PhD
- Cardiothoracic Surgery, University of Alabama at Birmingham; Birmingham, Alabama
Elfriede Pahl, MD
- Children's Memorial Hospital, Feinberg Northwestern School of Medicine, Chicago, Illinois
Juan Alejos, MD
- Mattel Children's Hospital, University of California–Los Angeles Medical Center, Los Angeles, California
F. Bennett Pearce, MD
- Pediatric Cardiology, University of Alabama at Birmingham; Birmingham, Alabama
James K. Kirklin, MD
- Cardiothoracic Surgery, University of Alabama at Birmingham; Birmingham, Alabama
Mary Zamberlan, CPNP
- Division of Pediatric Cardiology, C.S. Mott Children's Hospital, Ann Arbor, Michigan
Anne I. Dipchand, MD
- Hospital for Sick Children, Toronto, Ontario, Canada
Pediatric Heart Transplant Study Investigators

Received 1 April 2009; received in revised form 28 May 2009; accepted 30 May 2009. published online 28 September 2009.

Background

The course of pediatric hypertrophic cardiomyopathy (HCM) is variable, and the indications for heart transplantation (HTx) are ill defined. This study investigated outcomes and risk factors for adverse outcomes after listing for HTx.

Methods

A multicenter, event-driven data registry of patients aged < 18 years listed for HTx between January 1993 and December 2007 was used.

Results

During the study period, 3,147 children were listed for HTx (mean age, 7.6 ± 0.8 years). Of l,320 with CM at listing, 77 (6%) had HCM (61% boys; 79% white); 59% were United Network of Organ Sharing (UNOS) status I, 30% were receiving inotropes, 27% were ventilated, and 8% required extracorporeal membrane oxygenation. Arrhythmia had occurred in 27%, and 14% had failure to thrive. Within 1 year, 65% underwent HTx. Overall, 25 patients died after listing: 11 (14%) while waiting and 14 of 49 (29%) after HTx. Pre-HTx survival was lower for those listed at age < 1 year (p = 0.0005). Risk factors for death after listing included UNOS status 1 (p = 0.01) and younger age (relative risk, 2.3; p = 0.001). Late (10-year) survival after HTx for HCM patients was 47% vs 63% for non-CM patients within the database.

Conclusions

Children with HCM listed for HTx age < 1 year and UNOS status 1 have the highest mortality awaiting HTx. A more rigorous identification of additional risk factors should be performed to better define timing of listing and which patient sub-group may derive optimal benefit from HTx.