Idiopathic Constrictive Pericardial Disease in a Patient with Cystic Fibrosis

Introduction We report on a case of a patient with cystic fibrosis and co-existent constrictive pericardial disease. This unique presentation has been presented only once before in the literature. Our case is the first documented case where a patient with cystic fibrosis has undergone curative pericardial stripping. Case The case is about a patient who was diagnosed with cystic fibrosis at the age of 45. His sweat chloride level was 77.0mmol/L and genotype heterozygous for RII7H and delta F508. His FEV1 was 75% predicted at the time of admission. The patient was hospitalized for a presumed pulmonary exacerbation of his cystic fibrosis. He presented with cough with scant mucous production and no hemoptysis. He also noted dyspnea on exertion and new onset peripheral edema. He was treated for a pulmonary exacerbation with intravenous antibiotics and airway clearance measures and discharged home after two days. He worsened despite optimal therapy for a pulmonary exacerbation. A simultaneous right and left heart catheterization was performed that was suggestive of constrictive pericardial disease. The patient underwent a pericardial stripping that resolved his symptoms. He was discharged home after surgery and has been doing well. Discussion Constrictive pericardial disease is an extremely rare complication that is not frequently seen in cystic fibrosis. Simultaneous left and right heart catheterization can be used to make the diagnosis. Surgery is curative. Although pulmonary exacerbation is the most common cause of shortness of breath in patients with cystic fibrosis other diagnosis should be considered.