Constrictive pericarditis in cystic fibrosis: a case report of an emerging cardiovascular complication successfully managed with off-pump pericardiectomy.

Abstract

Background: Constrictive pericarditis (CP) is rarely reported in people with cystic fibrosis (CF) and may be overlooked because dyspnoea, fatigue, oedema, and reduced exercise tolerance overlap with pulmonary exacerbation. As survival improves with highly effective CF transmembrane conductance regulator modulator therapy, cardiovascular causes of cardiorespiratory symptoms may become increasingly relevant in adults with CF. This case highlights the importance of recognizing CP in this population and individualizing surgical decision-making.

Case summary: A 33-year-old woman with homozygous F508del CF, 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 CP. 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 1 h, received immediate chest physiotherapy, and was discharged on postoperative day 4. A reactive pleural effusion on day 28 resolved with drainage and antibiotics. At 4 months, oedema had resolved, exercise tolerance had improved, quality-of-life scores improved, and echocardiography showed no recurrent constriction.

Discussion: CP should be considered in people with CF when symptoms are disproportionate to pulmonary disease severity. CF should not automatically preclude pericardiectomy; in selected patients, CF-specific perioperative planning may enable definitive surgery with clinical and quality-of-life benefit.