Early predictors of survival to and after heart transplantation in children with dilated cardiomyopathy.
BACKGROUND
The importance of clinical presentation and pretransplantation course on outcome in children with dilated cardiomyopathy listed for heart transplantation is not well defined.


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.
Division of Cardiology, The Children's Hospital Denver, 13123 E 16th Ave, Aurora, CO 80045, USA. bill.pietra@UCdenver.edu
Biagio A
BA
Pietra
Paul F
PF
Kantor
Heather L
HL
Bartlett
Clifford
C
Chin
Charles E
CE
Canter
Ranae L
RL
Larsen
R Erik
RE
Edens
Steven D
SD
Colan
Jeffrey A
JA
Towbin
Steven E
SE
Lipshultz
James K
JK
Kirklin
David C
DC
Naftel
Daphne T
DT
Hsu
D000367:Age Factors; D002311:Cardiomyopathy, Dilated / Q000150:complications / Q000401:mortality* / Q000601:surgery; D002423:Cause of Death; D002648:Child; D002675:Child, Preschool; D005260:Female; D016027:Heart Transplantation; D006352:Heart Ventricles / Q000000981:diagnostic imaging; D006801:Humans; D007223:Infant; D053208:Kaplan-Meier Estimate; D008297:Male; D009205:Myocarditis / Q000150:complications; D011183:Postoperative Complications / Q000401:mortality; D016016:Proportional Hazards Models; D044469:Racial Groups; D012121:Respiration, Artificial; D012307:Risk Factors; D012720:Severity of Illness Index; D014463:Ultrasonography; D018487:Ventricular Dysfunction, Left / Q000209:etiology; D014850:Waiting Lists