MYOCARDIAL FIBROSIS - A RARE COMPLICATION IN PATIENTS WITH CYSTIC-FIBROSIS

We report a 10-month-old male infant who was admitted to our hospital with a history of failure to thrive and bulky stools. On examination, he was dystrophic and had a protruding abdomen, but he was well oxygen ated and his lungs were clear on auscultation. A tachycardia of 145 be ats per min and radiological evidence of cardiomegaly indicated involv ement of the heart, but an ECG failed to show signs of myocarditis or cardiac hypertrophy. An elevated sweat chloride concentration of 141 m Eq/l confirmed the diagnosis of cystic fibrosis (CF). Molecular analys is revealed heterozygosity for the common mutation delta F508. He died unexpectedly of a sudden cardiac arrest 2 days later. Autopsy reveale d scattered myocardial necrosis and fibrosis. Some 50 documented cases of myocardial fibrosis in infants with CF have been reported. Suggest ed causes such as malnourishment and hypovitaminosis remain speculativ e as systematic studies have yet to be done.