A RARE CASE OF PORTOPULMONARY HYPERTENSION AS A COMPLICATION OF CRYPTOGENIC CIRRHOSIS

Abstract

SESSION TITLE: Medical Student/Resident Pulmonary Vascular Disease Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Portopulmonary hypertension (PoPH) is a rare and serious complication of liver cirrhosis. The prevalence of PoPH is about 0.25–4% among patients with cirrhosis. Untreated PoPH can have a poor prognosis. We report a rare case of PoPH. CASE PRESENTATION: A 35 year old female with a history of cryptogenic liver cirrhosis with portal hypertension and transjugular intrahepatic portosystemic shunt (TIPS) presented to the emergency room with complaints of dyspnea, orthopnea, and dizziness which started 2 days after her 5th caesarean section. Her exercise tolerance was reduced to one block. The patient had an elevated ProBNP of 1100. Computed tomographic pulmonary angiography was negative for pulmonary embolism. Transthoracic echocardiogram showed right ventricular systolic pressure elevated at >60mm Hg, consistent with severe pulmonary hypertension (PAH) with severely dilated right atrium. The patient was transferred to a tertiary care hospital for confirmation of the diagnosis of PoPH. The patient underwent Right Heart Catheterization which confirmed severe PAH with elevated mean pulmonary artery pressure (mPAP) of 60mmhg, normal pulmonary capillary wedge pressure of 12mmhg, elevated pulmonary vascular resistance of 17 wood units. The hepatic vein wedge pressure was 23mmHg and hepatic vein pressure was 20mmHg.The hepatic venous pressure gradient (HVPG) was 3mmhg which was normal and was consistent with the fact that the patient already underwent TIPS due to underlying portal hypertension. The patient met the diagnostic criteria for PoPH. The patient was started on treprostinil infusion for pulmonary vasodilatation. The patient improved symptomatically and was eventually discharged. In her follow up pulmonary clinic appointment her exercise tolerance improved to 10 blocks with complete resolution of orthopnea. DISCUSSION: The development of PAH in patients with cirrhosis can be explained by shunting of vasoactive mediators from splanchnic to pulmonary circulation through portosystemic shunts causing vasoconstriction of the pulmonary vasculature. The acute volume loading with mobilization of plasma volume in the immediate postpartum period may have tipped the patient into acute on chronic right ventricular failure and thereby revealed her symptomatic PAH. The prompt recognition of PoPH and initiation of appropriate medical treatment ensured the recovery of the patient. CONCLUSIONS: The diagnosis of PoPH is crucial before liver transplantation as there is a high risk of death following liver transplantation if the mPAP is more than 35 mmHg. Medical treatment is essential to lower the mPAP to less than 35mmhg before proceeding for liver transplantation. Liver transplantation can be planned after successful medical treatment of PoPH with pulmonary arterial vasomodulators like prostanoids, phosphodiesterase inhibitors, and endothelin antagonists. Reference #1: Benjaminov FS, Prentice M, Sniderman KW, et al. Portopulmonary hypertension in decompensated cirrhosis with refractory ascites. Gut 2003; 52: 1355-1362. Reference #2: Saleemi S. Portopulmonary hypertension.Ann Thorac Med. 2010 Jan-Mar; 5(1): 5–9. Reference #3: Porres-Aguilar M, Zuckerman MJ, Figueroa-Casas JB, et al.Portopulmonaryhypertension: state of the art. Ann Hepatol 2008; 7:321-330 DISCLOSURES: No relevant relationships by Antony Arumairaj, source=Web Response No relevant relationships by Diana Cativo, source=Web Response No relevant relationships by Imnett Habtes, source=Web Response