An Unexpected Cause for Hypoxia in a 40-Year-Old Male

Abstract

SESSION TITLE: Fellow Case Report Poster - Pulmonary Manifestations of Lung Disease SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM INTRODUCTION: Hepatopulmonary syndrome (HPS) is an uncommon manifestation of hepatic disease that can lead to significant morbidity and mortality. We describe a case where dyspnea and hypoxemia were the initial presenting symptoms/signs of liver dysfunction. CASE PRESENTATION: A 40 year old male with a history of common variable immune deficiency (CVID) presented for evaluation of progressive dyspnea. Resting oxygen saturation on room air was 93% and decreased to 88% with walking 300 feet. Pulmonary function testing was normal except for a diffusion capacity reduced to 56% of predicted. CT angiography was normal. Transthoracic contrast echocardiogram with agitated saline injected into a peripheral vein showed bubbles in the left atrium 4 beats after opacification of the right atrium, consistent with extra cardiac shunting. The patient had no signs or symptoms of chronic liver disease although abdominal ultrasound showed a nodular liver. A transcutaneous liver biopsy revealed granulomatous hepatitis and bridging fibrosis, a result of CVID. The patient underwent orthotopic liver transplantation and has successfully been weaned off oxygen following transplantation. DISCUSSION: This is a patient found to have liver cirrhosis with HPS whose presenting complaint was isolated dyspnea with hypoxemia and otherwise no classic stigmata of cirrhosis. This is an extremely uncommon presentation, and it highlights the importance of a multisystem evaluation of hypoxia. Diagnosis of HPS requires evidence of liver disease, hypoxemia without an alternate cause, and evidence of intrapulmonary vascular dilatations usually diagnosed by contrast echocardiogram. HPS is present in 8-33% of patients undergoing workup for liver transplantation. No clear correlation exits between severity of liver disease and presence of HPS. Liver transplantation is the only known treatment for HPS, typically with a return to normal oxygenation within 12 months. CONCLUSIONS: We present a case of a patient with cirrhosis complicated by HPS who initially presented with dyspnea and hypoxemia who was successfully treated with liver transplantation. Reference #1: Arguedas MR, Abrams GA, Krowka MJ, Fallon MB. Prospective evaluation of outcomes and predictors of mortality in patients with hepatopulmonary syndrome undergoing liver transplantation. Hepatology 2003;37:192-197. Reference #2: Fritz JS, Fallon MB, Kawut SM. Pulmonary vascular complications of liver disease. Am J Respir Crit Care Med 2013;187:133-143. DISCLOSURE: The following authors have nothing to disclose: Jonathan Holdorf, Anna Rudnicki No Product/Research Disclosure Information