A United Network for Organ Sharing analysis of heart transplantation in adults with congenital heart disease: outcomes and factors associated with mortality and retransplantation.

OBJECTIVES: Heart transplantation in patients with adult congenital heart disease is increasing, yet no large studies have defined how this subgroup differs from other adult recipients. We investigated outcomes and risk factors for mortality and retransplantation among patients with adult congenital heart disease compared with adult recipients.

METHODS: A review was performed of 18- to 45-year-old patients undergoing heart transplantation from 1990-2008 reported to the United Network for Organ Sharing database. Trends were compared between 2 eras: era 1 (1990-1998) and era 2 (1999-2008). Multivariable semiparametric hazard models identified factors associated with time-related death and retransplantation.

RESULTS: Of 8496 patients identified, 575 had adult congenital heart disease. The prevalence of heart transplantation among adult recipients decreased by 28% over time (P < .001) and increased among patients with adult congenital heart disease by 41% (P < .001). Induction therapy use was less in patients with adult congenital heart disease (66%) compared with that seen in adult recipients (71%, P = .02). Steroid maintenance was less in patients with adult congenital heart disease (92%) compared with that seen in adult recipients (97%, P < .001). Post-heart transplantation survival among adult recipients improved over time (P = .02) but not among patients with adult congenital heart disease (P = .81). Overall post-heart transplantation mortality (P = .006) and retransplantation (P = .03) were significantly higher for patients with adult congenital heart disease than for adult recipients, mainly because of an early hazard phase. Adult congenital heart disease was a risk factor for both death (P < .001) and retransplantation (P = .04). Any induction therapy and steroid maintenance was associated with improved survival for all recipients (P = .001).

CONCLUSIONS: Adult congenital heart disease represent an increasing proportion of heart transplant recipients. Compared with adult recipients, patients with adult congenital heart disease experience higher post-heart transplantation mortality and retransplantation. Immunosuppression differs among patients with adult congenital heart disease and adult recipients. Further studies should investigate whether post-heart transplantation outcomes would be improved by more aggressive induction therapy or judicious steroid tapers.