Cor pulmonale in cystic fibrosis.

Abstract

Introduction In cystic fibrosis (CF) precapillary injury is the main pathology leading to pulmonary heart disease according to the WHO definition of cor pulmonalel. Interference with efficient intrapulmonary gas exchange due to bronchial wall and parenchymal damage will lead to an increase ofpulmonary artery pressure or pulmonary vascular resistance early in the course of the disease both at rest and during exercise, without clinical signs ofright sided cardiac failure. The ensuing right ventricular hypertrophy has no clinical correlates and overt clinical signs are rare and appear late in the course of CF. Cor pulmonale as a complication of CF was reported as early as 19512. Whereas autopsy material showed cardiac involvement in about 70% of children dying from CF, only 15% of patients with CF older than 25 years showed clinical right heart failure3. Other centres have reported right ventricular failure in 46% of CF patients from the age of 15 years onwards for at least two weeks before death4. With the improved life expectancy ofCF, pulmonary heart disease will gain greater importance. It should be stressed that the synonymous term cor pulmonale does not necessarily stand for right heart failure. Advances in the management of heart disease in chronic obstructive pulmonary disease (COPD) have been made56 which may bear direct relevance to future treatment of CF. In addition, drug-mediated pulmonary artery dilatation by vasoactive substances has gained acceptance and should be explored for use in CF7.