Pulmonary hypertension is associated with higher mortality in cystic fibrosis patients awaiting lung transplantation

F. Venuta, A. R. Tonelli, M. Anile, D. Diso, T. De Giacomo, F. Ruberto, E. Russo, M. Rolla, S. Quattrucci, E. A. Rendina, N. Li Phd, G. F. Coloni

Research output: Contribution to journalArticlepeer-review

Abstract

Aim. Pulmonary hypertension (PH) is frequently found in patients with advanced parenchymal lung diseases. In advanced stages, cystic fibrosis (CF) patients can develop PH and eventually cor pulmonale. Little is known about the prevalence of PH in CF patients and its impact on outcome. Methods. We retrospectively studied a large cohort of CF patients evaluated for lung transplantation between 1995 and 2010. All the patients underwent right heart catheterization as part of the evaluation. We included 179 unique consecutive adult CF patients. Age was 24±9 years and 45.8% were women. Results. Eighty-seven patients were transplanted (48.6%) and 65 died (36.3%) while waiting for LT. By right heart catheterization, 38.5% of the patients had PH (mean ≥25 mm Hg). PaCO2 (P=0.045) and forced vital capacity (P=0.023) were independent predictors of PH in CF patients. The median survival (free of lung transplantation) was 13.4 months. After adjusting for several covariates, the presence of PH significantly increased mortality (hazard ratio, HR) (P

Original languageEnglish
Pages (from-to)817-820
Number of pages4
JournalJournal of Cardiovascular Surgery
Volume53
Issue number6
Publication statusPublished - Dec 2012

Keywords

  • Cystic fibrosis
  • Hypertension, pulmonary
  • Lung transplantation

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Surgery

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