Chest · 2026
Impact of Pulmonary Arterial Hypertension Therapies on Gas Exchange in Portopulmonary Hypertension.
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Lacoste-Palasset Thomas, Baron Audrey, Ebstein Nathan, Beurnier Antoine, Grech Audrey K, Robert Fabien, Tu Ly, Jevnikar Mitja, Jaïs Xavier, Boucly Athénaïs, Valentin Simon, Magro Pascal, Horeau-Langlard Delphine, Aguado Benoit, Guignabert Christophe, Bauer Fabrice, Montani David, Humbert Marc, Sitbon Olivier, Savale Laurent
Аннотация
BACKGROUND: Portopulmonary hypertension (PoPH) may coexist with hepatopulmonary syndrome (HPS), a condition characterized by intrapulmonary vascular dilatations (IPVDs) and hypoxemia. Although pulmonary arterial hypertension (PAH) therapies are commonly used to treat PoPH, their effects on gas exchange remain insufficiently characterized.
RESEARCH QUESTION: What are the effects of PAH-specific therapies on gas exchange and the development of IPVDs in patients with PoPH?
STUDY DESIGN AND METHODS: A retrospective cohort of patients with PoPH was used to assess changes in the alveolar-arterial oxygen gradient (A-aDO2) following initiation of PAH therapy. A prospective cohort underwent systematic screening for IPVDs to assess their prevalence at baseline and incidence during treatment.
RESULTS: The retrospective cohort included 107 patients (70% male; mean age, 55 ± 8 years) with a baseline mean A-aDO2 of 37.1 ± 13.0 mm Hg. Eighty-three patients received initial oral monotherapy and 24 received dual oral therapy. After a median follow-up of 4.5 months, A-aDO2 remained stable overall (mean change, -0.92 mm Hg; 95% CI, -3.2 to 1.3; P = .42), with substantial interindividual variability. Changes in A-aDO2 were not associated with variation in 6-minute walk distance or World Health Organization/New York Heart Association functional class. Baseline A-aDO2 and cardiac output were independently associated with subsequent improvement in A-aDO2. Treatment-induced reductions in mean pulmonary artery pressure and increases in cardiac output correlated with greater A-aDO2 improvement. In the prospective cohort (n = 28), HPS was present in 39% of patients newly diagnosed with PoPH. Baseline IPVDs did not influence A-aDO2 evolution; however, 4 patients developed new IPVDs within the first year following PAH therapy initiation.
INTERPRETATION: Our results show that 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.
RESEARCH QUESTION: What are the effects of PAH-specific therapies on gas exchange and the development of IPVDs in patients with PoPH?
STUDY DESIGN AND METHODS: A retrospective cohort of patients with PoPH was used to assess changes in the alveolar-arterial oxygen gradient (A-aDO2) following initiation of PAH therapy. A prospective cohort underwent systematic screening for IPVDs to assess their prevalence at baseline and incidence during treatment.
RESULTS: The retrospective cohort included 107 patients (70% male; mean age, 55 ± 8 years) with a baseline mean A-aDO2 of 37.1 ± 13.0 mm Hg. Eighty-three patients received initial oral monotherapy and 24 received dual oral therapy. After a median follow-up of 4.5 months, A-aDO2 remained stable overall (mean change, -0.92 mm Hg; 95% CI, -3.2 to 1.3; P = .42), with substantial interindividual variability. Changes in A-aDO2 were not associated with variation in 6-minute walk distance or World Health Organization/New York Heart Association functional class. Baseline A-aDO2 and cardiac output were independently associated with subsequent improvement in A-aDO2. Treatment-induced reductions in mean pulmonary artery pressure and increases in cardiac output correlated with greater A-aDO2 improvement. In the prospective cohort (n = 28), HPS was present in 39% of patients newly diagnosed with PoPH. Baseline IPVDs did not influence A-aDO2 evolution; however, 4 patients developed new IPVDs within the first year following PAH therapy initiation.
INTERPRETATION: Our results show that 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.