부산대학교 도서관

A 56–year–old woman is admitted to our centre for biventricular heart failure. In anamnesis she reports a submandibular phlegmon complicated by pleuropericarditis at the age of 13 years requiring repeated pericardiocentesis. In the last three years she has been hospitalised twice for ascites and discharged with a diagnosis of idiopathic liver cirrhosis. On admission she presented with peripheral oedema, ascites and hippocratic fingers; oxygen saturation is 80% in room air. On blood tests creatinine, transaminases, albumin, coagulation and NT–proBNP are normal, bilirubin is elevated. An echocardiography is performed showing a calcific spicola impinging the free wall of the right ventricle producing endocavitary obstruction; diffusely thickened pericardium, septal bounce, annulus reversus and expiratory reflux in the suprahepatic veins. Biventricular contractile function is normal and no significant valvulopathy is present. CT scan confirms the presence of diffuse pericardial calcifications, small liver and irregular profile with large ascitic effusion. Decongestive therapy is started with weight loss of 22 kg and regression of oedema but severe hypoxia persists with evidence of platypnea–orthodeoxia syndrome. An echocardiography is repeated showing passage of microbubbles in the left atrium from the sixth beat onwards, compatible with intrapulmonary shunt. A large arteriovenous malformation (AVM) of the right lower pulmonary branch is found at CT angiography, which is also revealed by targeted invasive angiography. Cardiac catheterisation and cardiac MRI confirmed the diagnosis of constrictive pericarditis. Percutaneous closure of the AVM with complete resolution of the hypoxia and subsequent pericardiectomy is performed. The patient is discharged on day XXVII with normal saturation on room air, in good haemodynamic compensation and with normal liver stasis parameters. In conclusion, constrictive pericarditis is a rare cause of heart failure and to our knowledge this is the second case described reporting a pulmonary AVM secondary to constrictive pericarditis.