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.