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Outcomes of pediatric patients with hypertrophic cardiomyopathy listed for transplant.
MedLine Citation:
PMID:  19782603     Owner:  NLM     Status:  In-Process    
Abstract/OtherAbstract:
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.
Authors:
Robert Gajarski; David C Naftel; Elfriede Pahl; Juan Alejos; F Bennett Pearce; James K Kirklin; Mary Zamberlan; Anne I Dipchand;
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2009-09-26
Journal Detail:
Title:  The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation     Volume:  28     ISSN:  1557-3117     ISO Abbreviation:  J. Heart Lung Transplant.     Publication Date:  2009 Dec 
Date Detail:
Created Date:  2009-11-30     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9102703     Medline TA:  J Heart Lung Transplant     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1329-34     Citation Subset:  IM    
Affiliation:
Division of Pediatric Cardiology, C.S. Mott Children's Hospital, Ann Arbor, Michigan 48109, USA. rjgaj@med.umich.edu
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