Intermediate-term results of heart-lung transplantation for cystic fibrosis

B P Madden1, M E Hodson, V Tsang

  • 1Harefield Hospital, Middlesex, UK.

PubMed

Insights

Heart-lung transplantation for cystic fibrosis shows encouraging intermediate-term survival. Key challenges include donor organ shortage and obliterative bronchiolitis, impacting long-term outcomes.

Area of Science:

  • Cardiology
  • Pulmonology
  • Transplantation Medicine

Background:

  • Cystic fibrosis (CF) is a progressive genetic disorder leading to end-stage lung disease.
  • Heart-lung transplantation (HLT) offers a potential solution for patients with end-stage CF.
  • Intermediate-term outcomes of HLT for CF require further evaluation.

Purpose of the Study:

  • To report the intermediate-term outcomes of heart-lung transplantation in patients with end-stage cystic fibrosis.
  • To assess patient survival, complication rates, and functional improvements following HLT.
  • To identify factors influencing outcomes and long-term challenges in CF patients undergoing HLT.

Main Methods:

  • Retrospective analysis of 79 patients undergoing HLT for end-stage CF between 1984 and 1991.
  • Evaluation of actuarial patient survival, complication incidence (rejection, infection, lymphoproliferative disorders, obliterative bronchiolitis), and lung function.
  • Assessment of risk factors, including diabetes mellitus and other comorbidities, on survival.

Main Results:

  • Overall actuarial survival was 69% at 1 year, 52% at 2 years, and 49% at 3 years.
  • Significant improvements in lung function (FEV1, FVC) were observed post-transplantation, maintained long-term.
  • Common complications included acute rejection (92%) and obliterative bronchiolitis (17% at 1 year, 48% at 3 years).
  • Diabetes mellitus and other high-risk factors showed slightly reduced survival rates.
  • No coronary artery disease was detected in patients undergoing angiography.

Conclusions:

  • Heart-lung transplantation provides significant functional benefit and encouraging intermediate-term survival for end-stage CF patients.
  • Obliterative bronchiolitis and donor organ shortage remain critical obstacles.
  • Successful management of lymphoproliferative disorders and the potential for domino heart transplantation are noted.

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