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• W4387248812 abstract "SESSION TITLE: Pulmonary Manifestations of Systemic Disease Case Report Posters 16 SESSION TYPE: Case Report Posters PRESENTED ON: 10/10/2023 09:40 am - 10:25 am INTRODUCTION: Platypnea-orthodeoxia syndrome (POS) is a rare clinical syndrome characterized by the increase in oxygenation (orthodeoxia) in supine position (platypnea). In most cases, the hypoxia is the result of a right to left shunt, either intracardiac or extracardiac. In POS, right to left blood flow through the shunt increases in the supine position; the mechanism depends on the etiology. Here we describe a case of hepatopulmonary syndrome causing POS. CASE PRESENTATION: Patient is a 51 year old female with a history of COPD and alcoholic cirrhosis complicated by portal hypertension who presented with altered mental status, found to have an intracranial hemorrhage. She was intubated and admitted to the neurological ICU. She was noted to have acute hypoxic respiratory failure initially thought to be due to COPD, then pneumonia. Her CT angio chest was negative for pulmonary embolism and was noted to have bronchial wall thickening that was consistent with bronchitis or bronchiolitis, as well as scattered centrilobular ground glass nodules, concerning for an endobronchial infectious/inflammatory process, including new nodules within the right lower lobe (Figure 1). On further examination, the patient had SatO2 89% on HFNC 50/40 while seated. She was then laid flat for 2 minutes and her O2 sat slowly increased to 94%. Given the patient's platypnea-orthodeoxia syndrome, an echocardiogram was ordered to assess for intra and extracardiac shunt. The TEE saline bubble test was positive for intrapulmonary shunting and negative for atrial shunting. A diagnosis of hepatopulmonary syndrome was made due to the patient's POS and extracardiac shunt in the setting of severe cirrhosis. The only definitive treatment for hepatopulmonary syndrome is liver transplant. Unfortunately, the patient was not a candidate for transplant and transitioned to comfort measures only. DISCUSSION: This is a case of persistent acute hypoxic respiratory failure in a patient with advanced cirrhosis found to have POS due to hepatopulmonary syndrome. The positional change in POS is the opposite effect of common causes of hypoxic respiratory failure (ie pulmonary edema), making it challenging to diagnose.The pathophysiology of POS is poorly understood. Pathophysiology for most etiologies of POS are due to ventilation-perfusion mismatch secondary to a shunt, with shunt flow that increases in the upright position. The most common site of an extracardiac shunt is the lung, which can be worked up using an echocardiogram with a bubble study. Hepatopulmonary syndrome is a rare extrahepatic manifestation of liver disease characterized by liver dysfunction, hypoxemia, and dilated pulmonary vessels. The patient's advanced cirrhosis resulted in a high output state and caused intrapulmonary vascular dilation in the pre- and post capillary vessels at the lung bases. This causes V/Q mismatch secondary to arteriovenous shunting. In the upright position, blood flow increases in gravity dependent areas of the lungs, presenting as POS. CONCLUSIONS: Platypnea-orthodeoxia syndrome is caused by rare etiologies. In patients with advanced cirrhosis and unexplained hypoxemia, hepatopulmonary syndrome should be considered. Echocardiogram with bubble study is helpful in identifying an extracardiac shunt in the form of dilated pre and post capillary lung vessels. REFERENCE #1: Agrawal A. Et al. The Mutiple dimensions of platypnea-orthodoexia syndrome: a review. Resp Med 2017; 129:31-38. REFERENCE #2: Köksal D, Et al. Evaluation of intrapulmonary vascular dilatations with high-resolution computed thorax tomography in patients with hepatopulmonary syndrome. J Clin Gastroenterol. 2006;40(1):77. REFERENCE #3: Grilo-Bensusan I, Pascasio-Acevedo JM. Hepatopulmonary syndrome: What we know and what we would like to know. World J Gastroenterol. 2016 Jul 7;22(25):5728-41. doi: 10.3748/wjg.v22.i25.5728. PMID: 27433086; PMCID: PMC4932208. DISCLOSURES: No relevant relationships by Carlo Arevalo No relevant relationships by Manoj Mammen No relevant relationships by Anne Zhang" @default.
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• W4387248812 date "2023-10-01" @default.
• W4387248812 modified "2023-10-03" @default.
• W4387248812 title "HEPATOPULMONARY SYNDROME MANIFESTING AS PLATYPNEA-ORTHODEOXIA SYNDROME" @default.
• W4387248812 doi "https://doi.org/10.1016/j.chest.2023.07.3675" @default.
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