An Unusual Case of Cirrhosis

Abstract

Purpose: A 49-year-old white female with history of sarcoidosis was referred to GI when her liver was noted to be nodular during laparoscopy for an ovarian cyst. She denied fatigue, hematemesis, abdominal distension and pain, ankle swelling, itching, yellow discoloration of skin and eyes and episodes of confusion or sleepiness. She denied alcohol abuse. Physical examination revealed normal VS and no icterus, spider nevi, clubbing, ascites, hepatosplenomegaly or edema. LFTs revealed mild elevation in alkaline phosphatase and PT was slightly prolonged. CBC showed mild thrombocytopenia. Hepatitis serologies, ANA, AMA, ASMA, Ferritin, Ceruloplasmin, and α1-AT level were unremarkable. A liver biopsy confi rmed cirrhosis. Biopsy did not show any granulomas, but showed sinusoidal dilatation, which prompted a referral to cardiology. ECHO showed enlarged IVC and was otherwise unremarkable. A left and right heart catheterization was done. LHC showed normal coronaries, and RHC showed RAP 12 mm Hg, PAP 32/15 (mean 21) mm Hg, PAWP 18 mm Hg, LVEDP 18 mm Hg and CO 5.2 l/min. She developed worsening shortness of breath and was referred to pulmonology here. PFTs showed mild restriction, but CXR was unrevealing. CTPA ruled out PE and showed scattered pericardial calcification. Bubble ECHO did not show a right to left shunt. She developed SVT and was hospitalized here. She underwent a repeat left and right heart catheterization: RAP was 25 mm Hg, PAP 52/25 (mean 37) mm Hg, PAWP 32 mm Hg, LVEDP 36 mm Hg, and CO 4.12 l/min. Simultaneous measurement of left and right sided pressures confirmed constrictive pericarditis. In retrospect, the cirrhosis is “cardiac” cirrhosis and the result of long-standing elevated right sided heart pressures. Similarly, her dyspnea on exertion can be explained by constrictive pericarditis. We believe that constrictive pericarditis resulted from Sarcoidosis. She is now being evaluated for pericardiectomy. Clinical pearls: 1.) A cardiac etiology should be considered in the work-up of cirrhosis especially when the most common causes are not found. 2.) Pericardial calcification should suggest the possibility of constrictive pericarditis. 3.) Simultaneous measurement of left and right sided pressures should be performed when there is suspicion of constrictive pericarditis. 4.) Sarcoidosis is a rare but well known cause of pericardial disease.Figure