Abstract 2558: Outcomes of Children with Cardiomyopathy Listed for Heart Transplant: A Multi-Institutional Study
BACKGROUND: Dilated (DCM), restrictive (RCM), and hypertrophic (HCM) cardiomyopathies (CM) in children have widely varying clinical courses and therapeutic options. For all types, heart transplant (HTx) offers a chance for long-term survival, but clinical outcomes following listing and predictors to guide in the optimal timing of listing have not been well defined.
METHODS AND RESULTS: A multi-institutional, prospective, event-driven registry of pts<18 years of age listed for HTx from 01/93 to 12/05 (n=2,792) was used to compare outcomes of DCM (n=965; 84%); RCM (n=126; 11%); and HCM (n=63; 5%). Waitlist mortality for all pts was 12% with no difference by diagnosis (p=0.69). At listing, more DCM were Status 1 (p<0.0001), on high dose inotropes (p<0.0001), ventilated (p<0.0004), or on an assist device (VAD, p=0.0081). At the time of HTx, Status 1 (p<0.0001) or VAD (p=0.0003) were more common in DCM pts. Despite being more acutely unwell, overall survival from listing was higher in DCM pts (p=0.05), which was most dramatic for those listed in infancy (p<0.0001).[Fig⇓] Survival was particularly poor in RCM and HCM pts who were <1 yr of age at listing.[Fig⇓] Late (10 yr) survival post-HTx was also higher in DCM pts (82%) compared with RCM (76%) and HCM pts (65%, p=0.02).
CONCLUSION: Children with CM awaiting HTx have a low waitlist mortality and good survival post-HTx with DCM pts demonstrating a consistent, modest survival advantage from listing. The relatively poor survival in select groups of RCM and HCM pts suggests the need for further study to better understand the causes and timing of death, timing of listing, and influence of patient characteristics on survival.s