TY - JOUR
T1 - Early predictors of survival to and after heart transplantation in children with dilated cardiomyopathy
AU - Pietra, Biagio A.
AU - Kantor, Paul F.
AU - Bartlett, Heather L.
AU - Chin, Clifford
AU - Canter, Charles E.
AU - Larsen, Ranae L.
AU - Edens, R. Erik
AU - Colan, Steven D.
AU - Towbin, Jeffrey A.
AU - Lipshultz, Steven E.
AU - Kirklin, James K.
AU - Naftel, David C.
AU - Hsu, Daphne T.
PY - 2012/8/21
Y1 - 2012/8/21
N2 - Background-The importance of clinical presentation and pretransplantation course on outcome in children with dilated cardiomyopathy listed for heart transplantation is not well defined. Methods and Results-The impact of age, duration of illness, sex, race, ventricular geometry, and diagnosis of myocarditis on outcome in 261 children with dilated cardiomyopathy enrolled in the Pediatric Cardiomyopathy Registry and Pediatric Heart Transplant Study was studied. End points included listing as United Network for Organ Sharing status 1, death while waiting, and death after transplantation. The median age at the time of diagnosis was 3.4 years, and the mean time from diagnosis to listing was 0.62±1.3 years. Risk factors associated with death while waiting were ventilator use and older age at listing in patients not mechanically ventilated (P=0.0006 and P=0.03, respectively). Shorter duration of illness (P=0.04) was associated with listing as United Network for Organ Sharing status 1. Death after transplantation was associated with myocarditis at presentation (P=0.009), nonwhite race (P<0.0001), and a lower left ventricular end-diastolic dimension z score at presentation (P=0.04). In the myocarditis group, 17% (4 of 23) died of acute rejection after transplantation. Conclusions-Mechanical ventilator use and older age at listing predicted death while waiting, whereas nonwhite race, smaller left ventricular dimension, and myocarditis were associated with death after transplantation. Although 97% of children with clinically or biopsy-diagnosed myocarditis at presentation survived to transplantation, they had significantly higher posttransplantation mortality compared with children without myocarditis, raising the possibility that preexisting viral infection or inflammation adversely affects graft survival.
AB - Background-The importance of clinical presentation and pretransplantation course on outcome in children with dilated cardiomyopathy listed for heart transplantation is not well defined. Methods and Results-The impact of age, duration of illness, sex, race, ventricular geometry, and diagnosis of myocarditis on outcome in 261 children with dilated cardiomyopathy enrolled in the Pediatric Cardiomyopathy Registry and Pediatric Heart Transplant Study was studied. End points included listing as United Network for Organ Sharing status 1, death while waiting, and death after transplantation. The median age at the time of diagnosis was 3.4 years, and the mean time from diagnosis to listing was 0.62±1.3 years. Risk factors associated with death while waiting were ventilator use and older age at listing in patients not mechanically ventilated (P=0.0006 and P=0.03, respectively). Shorter duration of illness (P=0.04) was associated with listing as United Network for Organ Sharing status 1. Death after transplantation was associated with myocarditis at presentation (P=0.009), nonwhite race (P<0.0001), and a lower left ventricular end-diastolic dimension z score at presentation (P=0.04). In the myocarditis group, 17% (4 of 23) died of acute rejection after transplantation. Conclusions-Mechanical ventilator use and older age at listing predicted death while waiting, whereas nonwhite race, smaller left ventricular dimension, and myocarditis were associated with death after transplantation. Although 97% of children with clinically or biopsy-diagnosed myocarditis at presentation survived to transplantation, they had significantly higher posttransplantation mortality compared with children without myocarditis, raising the possibility that preexisting viral infection or inflammation adversely affects graft survival.
KW - Cardiomyopathy
KW - Dilated
KW - Heart transplantation
KW - Myocarditis
KW - Pediatrics
UR - http://www.scopus.com/inward/record.url?scp=84865482396&partnerID=8YFLogxK
U2 - 10.1161/CIRCULATIONAHA.110.011999
DO - 10.1161/CIRCULATIONAHA.110.011999
M3 - Article
C2 - 22800850
AN - SCOPUS:84865482396
SN - 0009-7322
VL - 126
SP - 1079
EP - 1086
JO - Circulation
JF - Circulation
IS - 9
ER -