Abstract / Summary
Background Constrictive pericarditis is rarely reported in people with cystic fibrosis and may be overlooked because dyspnoea, fatigue, oedema and reduced exercise tolerance overlap with pulmonary exacerbation. As survival improves with highly effective cystic fibrosis transmembrane conductance regulator modulator therapy, cardiovascular causes of cardiorespiratory symptoms may become increasingly relevant in adults with cystic fibrosis. This case highlights the importance of recognising constrictive pericarditis in this population and individualising surgical decision-making. Case Summary A 33-year-old woman with homozygous F508del cystic fibrosis, stable on elexacaftor/tezacaftor/ivacaftor, presented with chest pain, left scapular discomfort, breathlessness, night sweats and signs of systemic venous congestion. Computed tomography pulmonary angiography excluded pulmonary embolism but demonstrated pericardial calcification, thickening, bilateral pleural effusions and a small pericardial effusion. Transthoracic echocardiography, cardiac magnetic resonance imaging and invasive haemodynamic assessment confirmed chronic constrictive pericarditis. Following treatment of an acute infective/inflammatory episode and recovery from congestive hepatopathy, she underwent off-pump complete pericardiectomy via median sternotomy with phrenic-to-phrenic resection. She was extubated within one hour, received immediate chest physiotherapy and was discharged on postoperative day four. A reactive pleural effusion on day 28 resolved with drainage and antibiotics. At four months, oedema had resolved, exercise tolerance had improved, quality-of-life scores improved, and echocardiography showed no recurrent constriction. Discussion Constrictive pericarditis should be considered in people with cystic fibrosis when symptoms are disproportionate to pulmonary disease severity. Cystic fibrosis should not automatically preclude pericardiectomy; in selected patients, cystic fibrosis-specific perioperative planning may enable definitive surgery with clinical and quality-of-life benefit.