P2870 - Pulmonary Hypertension as a Rare Complication After Orthotopic Liver Transplant in a Patient With NASH Cirrhosis Complicated by Hepatopulmonary Syndrome

Armando Bosch, MD¹, Apaar Dadlani, MBBS¹, Michael Eiswerth, DO², Tyler D. Sharpe, MD^2
1University of Louisville School of Medicine, Louisville, KY; ²University of Louisville, Louisville, KY

Introduction: Hepatopulmonary syndrome (HPS) is a phenomenon that results in an intrapulmonary shunt leading to dyspnea and hypoxemia with poor response to oxygen supplementation. In this case, we describe a rare occurrence of pulmonary hypertension developing after orthotopic liver transplant (OLT) in a cirrhotic patient with HPS.

Case Description/Methods: 61-year-old female with a history of NASH cirrhosis complicated by esophageal varices and hepatic encephalopathy underwent liver transplant for worsening shortness of breath secondary to HPS. Two years after transplant, she presented with worsening dyspnea, saturating 86% on room air. Chest X ray and CT scan showed enlarged pulmonary trunk without consolidation or pulmonary edema, and 2D-echo showed normal LV ejection fraction with no valvular abnormality. Right and left heart catheterization showed high mPAP of 66 mm Hg consistent with severe pulmonary hypertension, with normal wedge pressure. She was started on intravenous epoprostenol and riociguat for pulmonary arterial hypertension, as well as diuretics. Repeat right heart catheterization during hospitalization showed mild improvement of mPAP to 57 mm Hg. However, patient’s hospital course was complicated by worsening shortness of breath requiring intubation, acute kidney injury and blood loss anemia from variceal bleed. Unfortunately, the patient’s condition continued to deteriorate, and comfort measures were initiated.

Discussion: Hepatopulmonary syndrome, prevalent in 5-23% of cirrhotic patients, classically presents with progressive dyspnea and exercise induced hypoxemia. Mechanism consists of pulmonary vascular dilation secondary to overproduction of nitric oxide affectively creates an intrapulmonary shunt causing hypoxemia. HPS is reversible with liver transplantation, however, some transplants result in subsequent pulmonary hypertension. Postulated mechanisms include unmasking of underlying pulmonary hypertension with OLT, and increased PVR due to increased blood flow in HPS. In a case series by Zopey et al., three patients achieved spontaneous resolution of HPS and subsequently developed portopulmonary hypertension before liver transplantation, highlighting the intricate balance between pulmonary vasodilators and vasoconstrictors. Our case adds to the growing recognition of the development of pulmonary hypertension after liver transplant in patients with HPS. It is important for providers to be aware of this phenomenon as mortality is estimated to be 69%.


Disclosures:


Armando Bosch, MD¹, Apaar Dadlani, MBBS¹, Michael Eiswerth, DO², Tyler D. Sharpe, MD². P2870 - Pulmonary Hypertension as a Rare Complication After Orthotopic Liver Transplant in a Patient With NASH Cirrhosis Complicated by Hepatopulmonary Syndrome, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.