Impact of congenital heart disease on outcomes of pediatric heart‐lung transplantation

Abstract

HLT is reserved for children with cardiopulmonary disease not amendable to alternative therapies. Children with CHD with or without ES may be considered for HLT. Outcomes of HLT in this population are not well described. To test the hypothesis that CHD without ES is associated with worse graft survival and identify factors associated with poor outcome, a retrospective analysis of the UNOS database was performed. One hundred and seventy-eight pediatric HLTs were performed between 1987 and 2011. CHD was the diagnosis in 65 patients, of which 34 had CHD without ES. Patients with CHD without ES had decreased patient survival (median 1.31 yr) compared with CHD with ES (4.80 yr, p = 0.05). On multivariable analysis, the following were associated with graft failure: CHD without ES (adjusted HR 1.69, 95% CI 1.09-2.62), younger age (1.04, 1.01-1.08), pretransplant mechanical ventilation (1.75, 1.01-3.06), pretransplant ECMO (3.07, 1.32-7.12), pretransplant PRAs (1.53, 1.06-2.20), and transplant era (1.85, 1.16-2.94). In children with CHD who require HLT, underlying physiology influences outcomes. Those without ES have a worse prognosis. The diagnosis of CHD without ES and preoperative factors may inform decisions in a complex patient population.

Pediatric heart‐lung transplantation: A contemporary analysis of outcomes

“…Due to the low number of transplants taking place, reports of outcomes are sparse, with some expectations extrapolated from young adult HLT outcomes, and risk factors for mortality are not well defined . Furthermore, there is no description of the waitlist outcomes of patients listed for pediatric HLT.…”

“…However, patients with a diagnosis of “Other” constitute 1/4 of HLTs, and it is encouraging to know that once transplanted, their survival is on par with the best outcomes for this operation. Unlike prior studies, we did not find panel‐reactive antibody to be an important risk factor for post‐transplant survival, yet ECMO remained the greatest risk factor and is unsurprisingly associated with more inotropic and ventilator support . Increasing age was shown to be protective for post‐transplant survival but could be related to different diagnoses presenting at different ages in addition to selection bias .…”

“…Though temporally this is not even to the year, it allowed for a similar number of patients in each era for comparison in addition to coinciding with a change in listing patterns and an overall decrease in pediatric HLT. Patient diagnosis was classified as CHD with ES, CHD without ES, PPH and Other as previously described by Keeshan et al Age categories were additionally created consistent with prior methods: infants (<1 year old), 1‐ to 11‐year‐olds, and adolescents (>11 years old).…”

“…End‐stage primary pulmonary hypertension (PPH) and congenital heart disease (CHD) with or without Eisenmenger's syndrome (ES) are the leading conditions necessitating heart‐lung transplantation (HLT) for pediatric patients . The first successful HLT, performed on a 45‐year‐old patient in 1981, was rapidly followed by the first successful pediatric HLT in 1985 .…”

“…The majority of HLTs are performed in adolescents, and there has been only one infant HLT performed since 2007. Worldwide pediatric HLT survival has improved over the past three decades with a reported 5‐year survival rate of 49% in the 2000s …”