Impact of pulmonary arterial hypertension therapies on gas exchange in portopulmonary hypertension.
retrospective_cohort · Level III
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- Record sourced from PubMed, PMID 42066896.
- Also identified by DOI 10.1016/j.chest.2026.04.027.
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Abstract
Portopulmonary hypertension (PoPH) may coexist with hepatopulmonary syndrome (HPS), a condition characterized by intrapulmonary vascular dilatations (IPVDs) and hypoxemia. While pulmonary arterial hypertension (PAH) therapies are commonly used to treat PoPH, their effects on gas exchange remain insufficiently characterized. What are the effects of pulmonary arterial hypertension (PAH)-specific therapies on gas exchange and the development of IPVDs in patients with PoPH? A retrospective cohort of PoPH patients was used to assess changes in the alveolar-arterial oxygen gradient (A-aDO<sub>2</sub>) following initiation of PAH therapy. A prospective cohort underwent systematic screening for IPVDs to assess their prevalence at baseline and incidence during treatment. The retrospective cohort included 107 patients (70% male, mean age 55 ± 8 years) with a baseline mean A-aDO<sub>2</sub> of 37.1 ± 13.0 mmHg. Eighty-three patients received initial oral monotherapy and 24 dual oral therapy. After a median follow-up of 4 months, A-aDO<sub>2</sub> remained stable overall (mean change -0.92 mmHg; 95% CI -3.2 to +1.3; P=0.42), with substantial interindividual variability. Changes in A-aDO<sub>2</sub> were not associated with variation in 6-minute walk distance or WHO/NYHA functional class. Baseline A-aDO<sub>2</sub> and cardiac output (CO) were independently associated with subsequent improvement in A-aDO<sub>2</sub>. Treatment-induced reductions in mPAP and increases in CO correlated with greater A-aDO<sub>2</sub> improvement. In the prospective cohort (n=28), HPS was present in 39% of newly diagnosed PoPH patients. Baseline IPVDs did not influence A-aDO<sub>2</sub> evolution; however, 4 patients developed new IPVDs within the first year after PAH therapy initiation. PAH therapy was not associated with overall deterioration in gas exchange in PoPH, despite substantial individual variability. Changes in oxygenation were linked to baseline and treatment-induced hemodynamic parameters. The development of new IPVDs in some patients warrants careful longitudinal monitoring.