Clinical characteristics, treatment patterns and healthcare resource utilisation for portopulmonary hypertension patients in the USA: a real-world study

Objectives To investigate demographic, clinical characteristics, treatment patterns and healthcare resource utilisation (HCRU) among patients with portopulmonary hypertension (PoPH).Design Retrospective analysis of a real-world cross-sectional survey, the pulmonary arterial hypertension (PAH) Diseas...

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Main Authors: Hilary Dubrock, Julia Harley, Mark Small, Megan Scott, Peter Agron, Yuen Tsang, Sumeet Panjabi
Format: Article
Language:English
Published: BMJ Publishing Group 2025-06-01
Series:BMJ Open
Online Access:https://bmjopen.bmj.com/content/15/6/e089223.full
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author Hilary Dubrock
Julia Harley
Mark Small
Megan Scott
Peter Agron
Yuen Tsang
Sumeet Panjabi
author_facet Hilary Dubrock
Julia Harley
Mark Small
Megan Scott
Peter Agron
Yuen Tsang
Sumeet Panjabi
author_sort Hilary Dubrock
collection DOAJ
description Objectives To investigate demographic, clinical characteristics, treatment patterns and healthcare resource utilisation (HCRU) among patients with portopulmonary hypertension (PoPH).Design Retrospective analysis of a real-world cross-sectional survey, the pulmonary arterial hypertension (PAH) Disease Specific Programme.Setting Secondary care settings in the USA.Participants Pulmonologists (n=22), cardiologists (n=17) and internists (n=7) who saw ≥2 patients with PAH/month provided data on 90 PoPH patients. Mean age of patients was 56.8 years, 51.1% were male, 76.7% were white/Caucasian.Primary and secondary outcome measures Demographic characteristics, initial presentation/diagnosis, disease trajectory, symptoms, comorbidities, treatment patterns and HCRU.Results The most common diagnostic tests were echocardiogram (62.2%), right heart catheterisation (56.7%) and electrocardiogram (48.9%). Dyspnea on exertion (64.4%), dyspnea following exertion (51.1%) and fatigue (48.9%) were the most common symptoms prior to PAH diagnosis as well as the most bothersome. Overall, 13.3% patients had mild disease before treatment vs 42.2% at time of survey. Most (60.0%) had stable disease (21.1% were improving and 18.8% were deteriorating at the time of the survey). Most common recent symptoms were dyspnea on exertion (51.1%), fatigue (37.8%) and dyspnea following exertion (32.2%). Patients had a mean of 1.8 comorbidities. In total, 80.0% of PoPH patients were receiving PAH-specific treatment; 41.7%, 22.2% and 12.5% received monotherapy, dual therapy and triple therapy, respectively. Patients had seen their physician 4.1 times in the past 12 months (3.5 visits specifically for PAH), while 31.1% had been hospitalised for PAH. Most (70.0%) patients were managed in a multidisciplinary team, and 8.9% of patients relied on a caregiver.Conclusions This study highlights the high symptomatic burden coupled with mostly moderate-to-severe disease status and a high hospitalisation rate despite the majority of PoPH patients being on therapy. Therefore, new treatment options in PoPH are in great need.
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spelling doaj-art-309bc73a4cd64505974a57d2b07efb942025-08-20T02:24:13ZengBMJ Publishing GroupBMJ Open2044-60552025-06-0115610.1136/bmjopen-2024-089223Clinical characteristics, treatment patterns and healthcare resource utilisation for portopulmonary hypertension patients in the USA: a real-world studyHilary Dubrock0Julia Harley1Mark Small2Megan Scott3Peter Agron4Yuen Tsang5Sumeet Panjabi6Mayo Clinic, Rochester, Minnesota, USAAdelphi Real World, Bollington, UKAdelphi Real World, Bollington, UKAdelphi Real World, Bollington, UKJanssen Pharmaceutical Companies of Johnson and Johnson, Titusville, New Jersey, USAJanssen Pharmaceutical Companies of Johnson and Johnson, Titusville, New Jersey, USAJanssen Pharmaceutical Companies of Johnson and Johnson, Titusville, New Jersey, USAObjectives To investigate demographic, clinical characteristics, treatment patterns and healthcare resource utilisation (HCRU) among patients with portopulmonary hypertension (PoPH).Design Retrospective analysis of a real-world cross-sectional survey, the pulmonary arterial hypertension (PAH) Disease Specific Programme.Setting Secondary care settings in the USA.Participants Pulmonologists (n=22), cardiologists (n=17) and internists (n=7) who saw ≥2 patients with PAH/month provided data on 90 PoPH patients. Mean age of patients was 56.8 years, 51.1% were male, 76.7% were white/Caucasian.Primary and secondary outcome measures Demographic characteristics, initial presentation/diagnosis, disease trajectory, symptoms, comorbidities, treatment patterns and HCRU.Results The most common diagnostic tests were echocardiogram (62.2%), right heart catheterisation (56.7%) and electrocardiogram (48.9%). Dyspnea on exertion (64.4%), dyspnea following exertion (51.1%) and fatigue (48.9%) were the most common symptoms prior to PAH diagnosis as well as the most bothersome. Overall, 13.3% patients had mild disease before treatment vs 42.2% at time of survey. Most (60.0%) had stable disease (21.1% were improving and 18.8% were deteriorating at the time of the survey). Most common recent symptoms were dyspnea on exertion (51.1%), fatigue (37.8%) and dyspnea following exertion (32.2%). Patients had a mean of 1.8 comorbidities. In total, 80.0% of PoPH patients were receiving PAH-specific treatment; 41.7%, 22.2% and 12.5% received monotherapy, dual therapy and triple therapy, respectively. Patients had seen their physician 4.1 times in the past 12 months (3.5 visits specifically for PAH), while 31.1% had been hospitalised for PAH. Most (70.0%) patients were managed in a multidisciplinary team, and 8.9% of patients relied on a caregiver.Conclusions This study highlights the high symptomatic burden coupled with mostly moderate-to-severe disease status and a high hospitalisation rate despite the majority of PoPH patients being on therapy. Therefore, new treatment options in PoPH are in great need.https://bmjopen.bmj.com/content/15/6/e089223.full
spellingShingle Hilary Dubrock
Julia Harley
Mark Small
Megan Scott
Peter Agron
Yuen Tsang
Sumeet Panjabi
Clinical characteristics, treatment patterns and healthcare resource utilisation for portopulmonary hypertension patients in the USA: a real-world study
BMJ Open
title Clinical characteristics, treatment patterns and healthcare resource utilisation for portopulmonary hypertension patients in the USA: a real-world study
title_full Clinical characteristics, treatment patterns and healthcare resource utilisation for portopulmonary hypertension patients in the USA: a real-world study
title_fullStr Clinical characteristics, treatment patterns and healthcare resource utilisation for portopulmonary hypertension patients in the USA: a real-world study
title_full_unstemmed Clinical characteristics, treatment patterns and healthcare resource utilisation for portopulmonary hypertension patients in the USA: a real-world study
title_short Clinical characteristics, treatment patterns and healthcare resource utilisation for portopulmonary hypertension patients in the USA: a real-world study
title_sort clinical characteristics treatment patterns and healthcare resource utilisation for portopulmonary hypertension patients in the usa a real world study
url https://bmjopen.bmj.com/content/15/6/e089223.full
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