Two children with cystic fibrosis and myocardial fibrosis.

Usually cardiac affection in cystic fibrosis is secondary to progressive affection of 
the lungs (cor pulmonale). In rare cases, babies and young children 
show a primary and severe cardiac affection caused by myocardial fibrosis. 
We could recently observe two of these cases in our clinic. 
The cases are presented and discussed. In babies and young children 
cardiac insufficiency caused by cardiomyopathy might be the first presenting symptom 
in CF. Therefore, in all children with cardiomyopathy of unknown origin, 
CF has to be suspected and the sweat test has to 
be performed. Rapidly progressive cardiac insufficiency responding poorly to treatment and 
rhythm disturbances are typical. Correct early diagnosis is important for early 
genetic counseling. 