Partial remission induction in pulmonary arterial hypertension and its predictors

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Background: Continuous intravenous prostacyclin (PGI2) therapy improves outcomes in pulmonary arterial hypertension (PAH), yet its management is challenging. There have been a few reports of successful discontinuation of PGI2 therapy without patient deterioration, which is considered a partial remission; however, the predictors for this success remain unclear. Aims: To identify the incidence and determinants of successful partial remission in PAH patients. Methods: In this retrospective, single-center study, we included PAH patients who initiated continuous PGI2 therapy between 1999 and 2023. Withdrawal of PGI2 was attempted when PGI2 dosage was stable for at least 3 months with mean pulmonary arterial pressure (mPAP) below 25 mmHg. Logistic regression was used to assess potential predictors, including age, sex, initial mPAP, early PGI2 initiation (within 3 months of the initial visit), and pregnancy-associated PAH. Results: Out of 108 patients, 21 (19%) discontinued PGI2. During a mean follow-up of 9.3 years, 45 patients died. In the discontinuation group, no deaths occurred, and mPAP remained unchanged (18 vs. 18 mmHg, p=0.66) over a mean follow-up of 4 years after discontinuation of PGI2. Multivariable analysis revealed early PGI2 initiation (odds ratio=3.71 [1.29–10.7], p=0.015) and pregnancy-associated PAH (odds ratio=9.06 [1.90–43.1], p=0.006) as independent predictors of successful PGI2 withdrawal. Conclusion: Approximately 20% of PAH patients maintained normal hemodynamics after PGI2 discontinuation, indicating partial remission could be achieved. The early and intensive treatment approach, particularly in pregnancy-associated cases, would be key to achieve partial remission.

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