Outcomes of children with cardiomyopathy listed for transplant: a multi-institutional study.
BACKGROUND
Dilated (DCM), restrictive (RCM), and hypertrophic (HCM) cardiomyopathies (CM) in children have varying clinical courses and therapeutic options. Heart transplantation (HTx) offers a chance for long-term survival; but outcomes after listing have not been well defined.


METHODS
A multi-institutional registry of 3,147 patients listed for HTx (January 1993-December 2006) was used to compare outcomes of 1,320 children with CM (42%) and 1,827 with non-CM (58%) etiologies. Comparisons were made between sub-groups: 1,098 DCM (83%), 145 RCM (11%), and 77 HCM (6%).


RESULTS
CM patients had a waitlist mortality of 17% vs 32% for non-CM patients (p < 0.0001), with no difference between the CM sub-groups. Risk factors were younger age, black race (relative risk [RR], 1.65; p = 0.009), mechanical ventilation (RR, 3.17; p < 0.001), and extracorporeal membrane oxygenation (RR, 2.16; p < 0.001). Ten-year survival after listing was 66% for CM vs 53% for non-CM (p < 0.0001). HCM and RCM patients aged < 1 year at the time of listing had the highest waitlist mortality and the lowest overall survival. CM patients had a better 10-year survival after HTx (68% vs 61%, p < 0.0001). Risk factors for death early after HTx included mechanical ventilation at HTx (RR, 3.07; p < 0.001), longer ischemic time (RR, 1.27; p = 0.01), and earlier era (RR, 1.77; p = 0.002). Late risk factors included black race (RR, 3.01; p < 0.001), HCM or RCM (RR, 1.93; p = 0.007), and older age (RR, 1.9; p < 0.001).


CONCLUSIONS
Children with CM have a lower waitlist mortality and better survival post-HTx than children with a non-CM diagnosis. DCM patients have the best and HCM or RCM patients aged younger than 1 year have the worst overall outcomes.
Dipchand
Anne I
AI
Hospital for Sick Children, Ontario, Canada. anne.dipchand@sickkids.ca
Naftel
David C
DC
Feingold
Brian
B
Spicer
Robert
R
Yung
Delphine
D
Kaufman
Beth
B
Kirklin
James K
JK
Allain-Rooney
Tina
T
Hsu
Daphne
D
D002170:Canada / Q000453:epidemiology; D009202:Cardiomyopathies / Q000401:mortality / Q000601:surgery*; D002423:Cause of Death / Q000639:trends; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D016027:Heart Transplantation; D006801:Humans; D008297:Male; D011379:Prognosis; D012189:Retrospective Studies; D012307:Risk Factors; D015996:Survival Rate / Q000639:trends; D013997:Time Factors; D014481:United States / Q000453:epidemiology; D014850:Waiting Lists