TOPIC: Pulmonary Vascular Disease TYPE: Original Investigations PURPOSE: Portopulmonary hypertension (PoPH) is pulmonary arterial hypertension (PAH) associated with portal hypertension. The diagnosis of PoPH can be challenging due to overlapping symptoms of decompensated liver disease, other types of PAH, and the paucity of available epidemiological and real-world evidence. We compared the clinical presentation of PoPH with idiopathic or hereditary PAH (I/HPAH) patients in a real-world US-based clinical practice setting. METHODS: This is a descriptive analysis of the Adelphi PAH Disease Specific Programme, a point-in-time physician survey providing data on patient and disease characteristics among PAH patients visiting pulmonologists, cardiologists and internists for routine care in the USA. Data collection was completed July-October 2019, with a PoPH oversample collected May-June 2020. RESULTS: A total of 90 PoPH patients from 44 physicians and 255 I/HPAH patients (233 IPAH, 22 HPAH) from 68 physicians were included in this study. 51.1% of PoPH and 45.1% of I/HPAH patients were male, with a mean age of 56.8 and 55.3 years, respectively. Prior to PAH treatment, physicians classified 65.6% of PoPH patients as functional class III or IV compared with 50.2% of I/HPAH patients. Most patients (60.0% PoPH, 56.1% I/HPAH) were reported by physicians as having "stable” PAH disease, with 18.9% of PoPH and 17.3% of I/IPAH patients considered to be "deteriorating", at the time of data collection. Physicians reported ascites, symptoms of abdomen distension and pain in 5.6%, 14.4% and 7.8% of PoPH patients and 3.1%, 7.5% and 4.3% of I/HPAH patients, respectively. Dyspnea following exertion (32.2% PoPH, 42.4% I/HPAH) and chest pressure/tightness (13.3% PoPH, 22.4% I/HPAH) were reported in a higher proportion of I/HPAH patients compared with PoPH patients. The mean Charlson Comorbidity Index score was 1.5 in PoPH and 0.4 in I/HPAH patients, indicating a poorer prognosis for patients with PoPH. Comorbid anxiety was reported in 17.8% of PoPH and 11.8% I/HPAH patients, depression in 16.7% and 12.9%, and obesity in 15.6% and 10.6% of patients, respectively. CONCLUSIONS: This study showed differences in disease severity prior to treatment as well as symptoms of PoPH compared with I/HPAH, with a higher proportion of PoPH patients with ascites, abdominal distension and pain, but a lower proportion with chest pain and dyspnea following exertion. The results also suggested a greater comorbidity burden among PoPH vs I/HPAH patients, particularly related to mental health. CLINICAL IMPLICATIONS: Our findings add to the understanding of clinical presentation of PoPH in a real-world setting, and highlight previously unreported differences in disease severity, symptoms, and comorbidity burden between PoPH and I/HPAH. DISCLOSURES: Employee relationship with Janssen Pharmaceuticals Please note: June 16, 2017 to now Added 04/26/2021 by Peter Agron, source=Web Response, value=Salary Consultant relationship with Janssen Please note: 2020-2021 Added 04/26/2021 by Hilary Dubrock, source=Web Response, value=Consulting fee Advisory Committee Member relationship with Janssen Please note: 2020 Added 04/26/2021 by Hilary Dubrock, source=Web Response, value=Honoraria Advisory Committee Member relationship with United Therapeutics Please note: 2021 Added 04/26/2021 by Hilary Dubrock, source=Web Response, value=Honorariaunknown relationship with Anumana Please note: Ongoing Added 04/26/2021 by Hilary Dubrock, source=Web Response, value=Intellectual property rights Removed 04/26/2021 by Hilary Dubrock, source=Web Response No relevant relationships by Julia Harley, source=Web Response Employee relationship with Janssen Please note: May 2020 to present Added 04/21/2021 by Sumeet Panjabi, source=Web Response, value=Salary Employee relationship with Janssen Please note: May 2020 to present Added 04/21/2021 by Sumeet Panjabi, source=Web Response, value=Ownership interest No relevant relationships by Megan Scott, source=Web Response No relevant relationships by Mark Small, source=Web Response Employee relationship with Janssen Scientific Affairs, LLC Please note: 2015 to present Added 04/20/2021 by Yuen Tsang, source=Web Response, value=Salary Employee relationship with Janssen Scientific Affairs, LLC Please note: 2015 to present Added 04/20/2021 by Yuen Tsang, source=Web Response, value=Stock