Key result
Portopulmonary hypertension carries high disease burden with ~80% of patients receiving PAH-specific treatment.
Why the study?
Demographic and clinical characteristics, treatment patterns, and healthcare resource utilisation among patients with portopulmonary hypertension were not well defined in real-world settings.
Cross-Sectional (n=90)
Yes
Despite 80% of portopulmonary hypertension patients receiving PAH-specific therapy, there remains a high symptom burden and hospitalization rate, highlighting the need for new treatment options.
Answers are grounded in this paper and cite their sources.
High burden persists despite PAH therapy in portopulmonary hypertension; hypothesis-generating for novel options and prospective trials.
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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DuBrock et al. (2025) conducted a cross-sectional in Portopulmonary hypertension (PoPH) (n=90). Portopulmonary hypertension was evaluated on Demographic characteristics, initial presentation/diagnosis, disease trajectory, symptoms, comorbidities, treatment patterns and healthcare resource utilisation. Among 90 patients with portopulmonary hypertension, 80.0% received PAH-specific treatment and 31.1% were hospitalized for PAH in the past 12 months, indicating a high disease burden.
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