Platypnea orthodeoxia syndrome: a rare phenomenon in hepatopulmonary syndrome

SESSION TITLE: Pulmonary Manifestations of Systemic Disease Case Report Posters 14 SESSION TYPE: Case Report Posters PRESENTED ON: 10/10/2023 09:40 am - 10:25 am INTRODUCTION: Hepato-pulmonary syndrome (HPS) is a known yet relatively rare condition that occurs in patients with severe liver cirrhosis. It's prevalence in literature varies between 4% and 32%, and 25.6% among liver transplant candidates. Its pathophysiology surrounds the systemic vasodilation that occurs most notably due to elevated levels of nitric oxide found in the circulation of patients with chronic liver failure. This condition in itself leads to a two-fold increase in mortality when compared to patients with liver cirrhosis who do not have HPS. Here we discuss a patient with HPS presenting with a rare phenomenon called platypnea orthodeoxia syndrome. CASE PRESENTATION: A 62 yr old female with history of alcoholic liver cirrhosis with ascites (requiring serial paracentesis), grade III esophageal varices (had undergone banding), hepatic encephalopathy presented to the hospital for shortness of breath. She was noted to be hypoxic with oxygen saturation as low as 63% requiring supplemental oxygen. She was afebrile, had no leucocytosis. MELD-Na on admission was 16. ProBNP and Ddimer were within normal limits. Other significant labs included elevated transaminases. Chest x-ray showed a large right pleural effusion and she underwent thoracentesis. Unfortunately, she continued to be hypoxic. Cardiac and pulmonary causes of hypoxia were ruled out with imaging including CT of chest and echocardiogram. While in the hospital, she was noted to have orthodeoxia and platypnea as she would maintain saturation within normal range while supine, however would consistently desaturate down to as low as 78% while in a sitting position. Concern for hepato-pulmonary syndrome along with platypnea orthodeoxia syndrome was raised. Repeat echocardiogram with bubble study was performed, demonstrating bubbles on 4th cycle. These findings were deemed consistent with an intrapulmonary shunt secondary to portopulmonary syndrome. Gastroenterology did not recommend TIPS procedure given portal vein thrombosis and cavernous transformation. Pulmonology recommended optimizing diuresis, given fluid overload contributing to worsening of the shunt. Cardiology was also consulted with assistance in confirming diagnosis of intra pulmonary shunting versus intracardiac shunting. They recommended right heart catheterization if patient continued to be hypoxic. She was deemed a candidate for liver transplantation. DISCUSSION: This patient is a particularly complex patient with many contributors to her overall poor health. Her extensive liver cirrhosis and subsequent periodic requirement for paracentesis and large volume accumulation of pleural fluid as well as her elevated MELD score are poor prognostic indicators. This patient's POS that resulted in the diagnosis of HPS is an additional factor that lends to her concerning prognosis. The only definitive treatment for POS is liver transplantation. CONCLUSIONS: Platypnea orthodeoxia syndrome (POS) is characterized by dyspnea and hypoxemia that occurs in the upright position, with improvement in work of breathing and partial pressure of oxygen while supine. It is more commonly seen in patients with a patent foramen ovale or other intracardiac shunt which account for 87% of reported cases. However, in the correct clinical context such as in severe liver cirrhosis, POS should prompt for evaluation for an intrapulmonary shunt. REFERENCE #1: https://pubmed.ncbi.nlm.nih.gov/34636408/ - Hepatopulmonary Syndrome and Portopulmonary Hypertension: Pulmonary Vascular Complications of Liver Disease, authors: Kathryn Del Valle 1, Hilary M DuBrock 1 REFERENCE #2: https://www.resmedjournal.com/article/S0954-6111(17)30158-0/fulltext - The multiple dimensions of POS – A review authors: Abhinav Agrawal, MD, Atul Palkar, MD, Arunabh Talwar, MD FCCP. DISCLOSURES: No relevant relationships by Lucy Checchio No relevant relationships by Joseph Landau No relevant relationships by Rahat Memon No relevant relationships by Rehan Saeed No relevant relationships by Shreeja Shah