Hepatopulmonary Syndrome: A Commonly Missed Complication of Chronic Liver Disease

Abstract

Case of a 65-year-old man with medical history of hypertension and cirrhosis secondary to Hepatitis C, who was initially consulted to gastroenterology service due to intermittent loose and blood tinged stools for which he was scheduled for a colonoscopy. On the procedure day he was found with a peripheral O2 saturation of 88% at room air, improving to 99% with nasal cannula. Hepatopulmonary syndrome was suspected, but prior to starting work up, patient was admitted under Internal Medicine service due to progressive dyspnea and multiple syncopal episodes. During admission a pulmonary scintigraphy was performed reporting abnormal tracer concentration in several extrapulmonary organs, highly suggestive of a right-to-left shunt. Pulmonary service evaluated patient and noted that after exercise testing disease severity interpretation had changed from moderate to very severe. Upon discharge patient was requiring continuous oxygen supplementation. Subsequently, cardiology service performed contrast echocardiogram with agitated saline in which contrast was seen to enter the left atrium 6 beats after first entering the right atrium, confirming hepatopulmonary syndrome. Patient was referred to the liver transplant center, where he was placed on transplant list with MELD exception. Hepatopulmonary syndrome is caused by a defect in arterial oxygenation due to pulmonary vascular dilatation in the setting of liver disease. Contrast-enhanced transthoracic echocardiography with saline is the most practical method to detect pulmonary vascular dilatation. Dyspnea at exertion or at rest, although non-specific, is the most common presenting finding. Prevalence of this syndrome ranges from 5%-32% although no prospective studies are available. Liver transplantation is the only treatment with documented success. This case serves to illustrate the importance of maintaining a keen eye on subtle symptoms that a patient with chronic liver disease may develop and which may usually be put off as part of the disease per se. Patients with chronic liver disease should have at least peripheral oximetry obtained in each routine visit and arterial blood gases should be obtained if there is dyspnea. Early suspicion of hepatopulmonary syndrome in cirrhotic patients is important in order to provide them a prompt work up which will definitely expedite the liver transplant process before the syndrome progresses and excludes them as candidates.