B47-25 A 45-Year-Old Woman With Progressive Dyspnoea

Case Description A 45-year-old female with cystic fibrosis (CF) F508del homozygous managed with Ivacaftor/Tezacaftor/Elexacftor (Kaftrio) and Ivacaftor (Kalydeco) presented with 4 weeks of progressive dyspnea, chest “fullness” worse when recumbent, fevers, and increased sputum production. She had a background of CF-related diabetes, and chronic kidney disease. Initial workup revealed elevated C reactive protein (CRP) 72mg/L and white cell count (WCC) 12.2 10^9 /L. Chest radiograph demonstrated bilateral lower lobe consolidation and she was initiated on Tobramycin and Doxycycline for presumed infectious exacerbation of CF. Due to persistent chest discomfort a computed tomography pulmonary angiogram (CTPA) was performed revealing a moderate pericardial effusion with no pulmonary embolism. Echocardiography showed a moderate effusion with features suggestive of constrictive physiology including a septal bounce and respiratory variation in mitral valve blood flow. Infectious and rheumatologic workups were negative. Simultaneous right and left heart catheterization demonstrated diastolic equalization of intracardiac pressures and most importantly ventricular discordance (simultaneous decrease in left ventricular pressure and increase in right ventricular pressure during inspiration), confirming a diagnosis of effusive-constrictive pericarditis. Pericardiocentesis was not required as the patient remained hemodynamically stable and she was commenced on colchicine and broad-spectrum antibiotics. Despite this her condition deteriorated with persistent fevers and rising inflammatory markers. A trial of steroids and antifungals to manage this was limited by hyperglycemia and hepatotoxicity, respectively. High-dose aspirin 600mg three times daily with a slow taper and colchicine for targeted anti-inflammatory effect, alongside diuresis with bumetanide led to clinical and biochemical improvement with NT-proBNP decreasing from 3000 ng/L to 200ng/L, CRP normalizing to 14mg/L, and a follow-up echocardiogram showing resolution of the pericardial effusion. She was discharged home clinically improved and has remained well to date with her 1 month follow up echocardiogram showing her constrictive physiology and effusion has completely resolved. Discussion Constrictive pericarditis is rare in patients with CF with 5 previous cases reported in the literature. Traditional management focuses on managing the underlying cause, pericardiocentesis where required and occasionally surgery (pericardiectomy). This case highlights some of the challenges that could be faced in an older CF patient population in the era of modulators. Surgical pericardiectomy carries substantial risk and sternotomy may complicate eligibility for lung transplantation if required in the future. Our successful use of high dose aspirin and colchicine avoided the need for high-risk surgery thus preserving future eligibility for lung transplantation supporting future consideration of this treatment regime. This abstract is funded by: None