[Myocardial fibrosis in 2 children with cystic fibrosis]

Area of Science:

• Pediatric Cardiology
• Gastroenterology
• Pathology

Context:

• Cystic fibrosis is a genetic disorder primarily affecting the lungs and digestive system.
• Congestive heart failure in infants is a serious condition requiring prompt diagnosis and management.
• Cardiac manifestations of cystic fibrosis are less common but can be severe.

Purpose:

• To report two cases of infants diagnosed with cystic fibrosis who presented with congestive heart failure.
• To investigate the pathological findings in patients with cystic fibrosis and heart failure.
• To explore potential mechanisms linking pancreatic involvement to myocardial damage in cystic fibrosis.

Summary:

• Two infants, aged 9 and 15 months, developed congestive heart failure at 9 and 10 months, respectively, and were diagnosed with cystic fibrosis.
• Both patients died within the first year post-diagnosis due to heart failure.
• Necropsy revealed pancreatic abnormalities and significant heart lesions, including myocardial fibrosis and necrosis.

Impact:

• Highlights the potential for severe cardiac complications in cystic fibrosis patients.
• Underscores the importance of considering cardiac evaluation in infants with cystic fibrosis presenting with heart failure.
• Suggests a potential pathophysiological link between pancreatic dysfunction and cardiac fibrosis in cystic fibrosis.