Cor pulmonale and related cardiovascular effects of cystic fibrosis. pp. 41-80.

Cor pulmonale was defined by a committee of the World Health Organization 
as "right ventricular hypertrophy, secondary to disease of the lung parenchyma 
or pulmonary vasculature, or due to abnormalities of pulmonary function." It 
has been estimated that 70% of children dying with cystic fibrosis 
have cor pulmonale, though as children live longer, the number may 
be higher. Clinical diagnosis, management, prognosis, echocardiography, echocardiography as a measure 
of left ventricular dysfunction, and a study of 94 ambulatory and 
hospitalized cystic fibrosis patients are discussed. The study establishes that in 
patients with cystic fibrosis, left ventricular dysfunction and hypertrophy occur and 
are related to increasing severity of pulmonary involvement. 