Echocardiographic assessment of cor pulmonale in patients with cystic fibrosis

Abstract

Cystic fibrosis (CF) is an inherited multisystem disorder, characterized mainly by obstruction and infection of airways and by maldigestion and its consequences. An increasing interest concerning patients suffering from CF has been observed. Aim of this study was echocardiographic study of morphological structure, functional index and the grade of pulmonary hypertension in patients with CF. In 45 patients aged 3 to 24 years (mean 15.1 years) echocardiography was performed. Right ventricular end-diastolic dimension (RVDd) was increased in 67% of patients, right ventricular anterior wall thickness (RVAWd) was increased in 71% of patients. Pulmonary artery pressure was abnormally high in 84% of CF patients. Significant correlations between RVDd, RVAWd, PAP and Shwachman-Kulczycki clinical score was found. The dimensions of the left ventricular cavity were abnormal in 22% of patients. Insufficiency of pulmonary valve was observed in 47% of CF patients and tricuspid insufficiency in 53%. This study demonstrates that: 1) changes occurs mainly in right ventricle and appear to worsen as the disease progresses, 2) all patients with CF should remain under cardiological control, 3) echocardiography could be a useful method of long-term monitoring patients with CF.