Abstract | BACKGROUND: 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.
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Authors | Robert Gajarski, David C Naftel, Elfriede Pahl, Juan Alejos, F Bennett Pearce, James K Kirklin, Mary Zamberlan, Anne I Dipchand, Pediatric Heart Transplant Study Investigators |
Journal | The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation
(J Heart Lung Transplant)
Vol. 28
Issue 12
Pg. 1329-34
(Dec 2009)
ISSN: 1557-3117 [Electronic] United States |
PMID | 19782603
(Publication Type: Comparative Study, Journal Article, Multicenter Study, Research Support, Non-U.S. Gov't)
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Topics |
- Cardiomyopathy, Hypertrophic
(mortality, surgery)
- Cause of Death
(trends)
- Child
- Disease Progression
- Female
- Follow-Up Studies
- Heart Transplantation
- Humans
- Male
- Ontario
(epidemiology)
- Preoperative Period
- Prognosis
- Retrospective Studies
- Survival Rate
(trends)
- United States
(epidemiology)
- Waiting Lists
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