(183) Idiopathic Constrictive Pericarditis With Pericardial Effusion in a Cystic Fibrosis Patient

Introduction: Cystic fibrosis (CF) is a genetic disease affecting airway liquid balance, leading to chronic respiratory issues. Survival rates have improved, with many patients now reaching adulthood. CF presents with complications including diabetes and bronchiectasis-related problems. Recurrent pulmonary infections are the primary cause of mortality. Treatment involves both chronic therapies and managing acute infections. Acute pericarditis with pericardial effusion is a rare complication in CF, possibly due to anatomical connections. Constrictive pericarditis (CP) can exacerbate CF symptoms, commonly managed with NSAIDs and colchicine. Despite its rarity, CP complicates CF management, requiring additional attention.

Description: A 24-year-old female with Cystic Fibrosis, diabetes mellitus, and pancreatic insufficiency presented with chest pain and shortness of breath. Her symptoms included chest pain exacerbated by lying flat and relieved by leaning forward, along with tachycardia, tachypnea, and low oxygen saturation. Her vital signs were heart rate of 112 bpm, RR of 23, and oxygen saturation at 88% on room air and blood pressure of 113/74. Physical examination revealed pericardial friction rub, bilateral lung rales, pitting edema, and facial swelling. Laboratory findings showed elevated white blood cell count of 13.1 glucose of 402, and Pro-BNP of 202, with normal hemoglobin and renal function. Tests for autoimmune diseases and infections were mostly negative, except for MRSA and MSSA detected in nasal swabs and blood cultures. Imaging revealed bilateral lung opacities, pleural and pericardial effusions, and signs of pneumonia, cirrhosis, and splenomegaly. Echocardiogram and cardiac catheterization confirmed constrictive pericarditis. Treatment involved medications targeting infection, fluid retention, and inflammation, alongside pericardiocentesis. Follow-up showed improvement with no recurrence of symptoms.

Discussion: Constrictive pericarditis is a rare complication in cystic fibrosis (CF) patients, often challenging to diagnose. Close monitoring and comprehensive management are essential, including NSAIDs, colchicine, and pericardiocentesis for tamponade physiology. Clinicians should remain vigilant for symptoms, ensuring timely intervention to optimize patient outcomes.