Assessment of Cor Pulmonale in Cystic Fibrosis by Echocardiography

Abstract

Thirty-one patients with cystic fibrosis of varying severity were examined by echocardiography. Right ventricular dimension (RVD) was above upper normal limit in 14 patients and right ventricular dimension index (RVD index) was higher than the upper normal limit in 11 patients. Furthermore, there was a significant relationship between increasing RVD index and 1) decreasing forced vital capacity (FVC) both actual test results and average 6 months values; and 2) decreasing peak-expiratory flow rate (PEFR) both actual test results and average 6 months values. This observation suggests a persistent heart involvement. Five patients had either heart failure and/or electrocardiographic evidence of right ventricular abnormality. These patients had increased RVD index and one patient with the highest RVD index died 8 weeks after the examination. The present study has shown the usefulness of echocardiographic measurement of right ventricular dimension and of septal motion in assessing cor pulmonale, before development of electrocardiographic abnormalities and right heart failure.

Technological advances shed light on left ventricular cardiac disturbances in cystic fibrosis

“…Despite it being less reliable than radionucleotide angiography, transthoracic echocardiography has become the most popular method of examining heart function due to its noninvasive nature, ease of accessibility, ability to serially examine both the structure and function the heart, and good correlation with pulmonary function tests and the clinical status of patients [16][17][18][30][31][32][33][34][35]. Since the Bright-Thomas et al review in 2001, an additional eight studies utilizing echocardiography have been performed.…”

Cor Pulmonale in Cystic Fibrosis

“…Older studies using M-mode echocardiography found the dimension and function of the right heart to be related to clinical disease scores and pulmonary function tests. 30,31 A later two-dimensional and Doppler echocardiographic study found a poor correlation between right ventricular ejection fraction and clinical score in a group of patients with mild to moderate disease. 32 This result is corroborated by a radionuclide study of right ventricular ejection fraction in children with CF that showed only a moderate decrease in ejection fraction in patients with severe lung disease and found that some patients with terminal lung disease had normal contractility.…”

Suppression of Early Mitral Valve Opening in Cor pulmonale

“…Many M-mode and 2-D échocardiographie studies of cor pulmonale have been published over the last decade [8][9][10][11][12][13][14][15][16][17][18][19][20][21], All these reports have featured increased RV dimension or size, RV anterior wall thickness, pulmo nary valve motion abnormalities suggestive of pulmo-…”