Outcomes for children with cardiomyopathy awaiting transplantation

Objective: To determine factors associated with outcomes after listing for transplantation in children with cardiomyopathies. Background: Childhood ca rdiomyopathies form a heterogeneous group of diseases, and in many, the pro gnosis is poor, irrespective of the etiology. When profound heart failure d evelops, cardiac transplantation can be the only viable option for survival . Methods: We included all children with cardiomyopathy listed for transpla ntation between 12/89 and 4/98 in this historical cohort study. Results: We listed 31 patients, 15 male and 16 female, 27 with dilated and 4 with rest rictive cardiomyopathy, for transplantation. The median age at listing was 5.7 years, with a range from fetal life to 17.8 years. Transplantation was achieved in 23 (74%), with a median interval from listing of 54 days, and a range from zero to 11.4 years. Of the patients, 14 were transplanted withi n 30 days of listing. Five patients (16%) died before transplantation. With in the Canadian algorithm, one of these was in the third state, and four in the fourth state. One patient was removed from the list after 12 days, hav ing recovered from myocarditis, and two remain waiting transplantation afte r intervals of 121 and 476 days, respectively. Patients who died were more likely to be female (5/5 vs. 11/26; p=0.04) and to have been in the third o r fourth states at listing (5/5 vs. 15/26; p=0.04). The use of mechanical v entricular assistance, in 10 patients, was not a predictor of an adverse ou tcome. While not statistically significant, survival to transplantation was associated with treatment using inhibitors of angiotensin converting enzym e, less mitral regurgitation, a higher mean ejection fraction and cardiac i ndex, and lower right ventricular systolic pressure. Conclusions: Children with. cardiomyopathy awaiting transplantation have a mortality of 16% relat ed to their clinical state at the time of listing.