Purpose:The clinical course of pediatric hypertrophic cardiomyopathy (HCM) is variable and the need for heart transplantation (HTx) within this patient (pt) group and the outcomes thereafter remain ill-defined. The purpose of this study was to investigate the outcomes of listing for HTx and risk factors for adverse outcomes among these pts. Methods and Materials: A prospective, multi-institutional, eventdriven data registry of pts Ͻ18 yrs of age listed for HTx between 1/93-12/06 was used to assess risk factors and outcomes of HCM pts listed for HTx. Results: There were 3147 children listed for HTx over the study period. Of l320 listed CM pts, 77 (6%) had HCM (male 61%, white 79%); mean age at listing was 7.6 yrs. At listing, 59% were UNOS Status I, 30% on high-dose inotropes, 27% ventilated, and 8% on ECMO. Arrhythmia had occurred in 19% and 14% had failure to thrive. Sixty-five percent underwent HTx within 1 yr (Fig). Overall, 25 pts died post-listing: 11 (14%) died while waiting with 14 (29%) post-HTx deaths. Survival among HCM pts was lower for those listed Ͻ1yr of age vs those Ͼ1yr (pϭ0.0005, Fig). The only risk factor for death while waiting was UNOS Status 1 (pϭ0.01). ECMO was the only risk factor for death early post-HTx (RR 9.78, pϭ0.003). Late (10-yr) post-HTx survival for HCM pts was 60%, similar to the non-CM population (63%). Conclusions: HCM pts comprise the smallest proportion of children with CM listed for HTx. HCM pts listed Ͻ1yr of age or UNOS status 1 had the highest mortality awaiting HTx. In addition, need for ECMO was a significant risk factor for early post-HTx death. With limited infant donors, HTx of infants with HCM requires careful consideration, and more rigorous identification of key pre-HTx risk factors is warranted.
540Purpose: The procurement of donor organs with prolonged ischemic time for pediatric heart transplant recipient has raised some concern over the potential increased risk of using organs with long ischemic time. We hypothesized that there would be no difference in outcome based on prolonged donor ischemic time. Methods and Materials: This retrospective study looks at the long-term outcome children who had a long (IT Ͼ 6 hrs) donor ischemic time comparing them to those who had organs with short (IT Ͻ6 hr) ischemic times. Outcome variables included prolonged graft downtime (Ͼ30min), graft loss (GL), death, post-transplant coronary disease (CAD), GL in the first year, acute rejection and other causes of GL. Groups were compared using Student's t-test, chi-square analysis and Kaplan-Meier. Results are given as median and range. A p-value Ͻ 0.05 was considered significant. Results: From 1985 until May 2007, 459 children have undergone heart transplantation at our institution. Of these, 123 children had donors with a long ischemic time. The table shows transplant age, length of follow-up and variable distribution. Although the percentages were higher in the long IT group, these differences were not statistically significant. There was no relationship between prolonged downtime...
