Ventricular interdependence in severe cystic fibrosis. A two-dimensional echocardiographic study

A variety of mechanisms have been implicated in the development of left ventricular dysfunction in patients with chronic cor pulmonale. A two-dimensional echocardiographic study of cystic fibrosis (CF) patients with severe cor pulmonale was undertaken to evaluate the effects of long-term pulmonary abnormalities on right and left ventricular geometry. Ten patients with severe obstructive pulmonary disease secondary to CF underwent evaluation by a mechanical sector scanner from the long axis, short axis, and four chambered views. All patients manifested right heart failure. Eight had clinical scores less than 40 and died within six months of the initial examination. All patients were receiving diuretics, and six were taking digoxin at the time of the study. The most striking echographic feature was flattening or compression of the left ventricle along its minor dimension by a massively dilated right ventricle. Compression of the left ventricle and additional abnormalities of interventricular septal motion resulted in dyskinetic contraction and relaxation that could contribute to a diminished stroke volume. Massive right ventricular enlargement appears to be a major factor producing left ventricular dysfunction in chronic cor pulmonale.