ArticleOrphanet journal of rare diseases2026
The prevalence and long-term response to calcium channel blockers in patients with pulmonary arterial hypertension and positive vasoreactivity test - results of multicenter national registry (BNP-PL).
Article in Orphanet journal of rare diseases, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
backgroundThe profile of patients with pulmonary arterial hypertension (PAH) and positive acute haemodynamic response (acute responders) who may benefit on long-term calcium channel blocker (CCB) therapy is not yet well documented.
resultsThe aim of this study was to identify the subgroup of patients with PAH from the Polish Multicenter Registry of Pulmonary Hypertension (BNP-PL) who were found to be responders at the initial acute pulmonary vasoreactivity test (APV-test), and analyse the frequency, characteristics and prognosis of patients with different long-term response to treatment with CCB. Out of 534 patients 49 (9.2%) fulfilled the responder criteria (acute responders). Patients with positive APV test had a less advanced WHO class (p < 0.001), longer distance in a 6-minute walking test (6MWT) and had a significantly lower NTproBNP compared to non-acute responders (540 m/IQR:480-560/ vs. 280 m/IQR:160-390/, p < 0.001; 503.2 pg/ml/IQR:214.6-1533/ vs. 2019 pg/ml/IQR:806-4286/, p < 0.001; respectively). The hemodynamic profile of the above two groups (acute responders and non-acute responders) was similar. CCB long-term response was found in 55.1% (n = 27) of acute responders. All-cause mortality was lower among acute responders (11.54%) compared to non-acute responders (36.07%); p = 0.039. Among acute responders, long-term responders showed markedly lower mortality (0%) compared to non-long-term responders (21.05%); p = 0.05.
conclusionsNovel analysis in a unique large Polish PAH population highlights that APV testing and rapid early post-CCB assessment are key to long-term prognosis.
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