# 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)

**Authors:** Michał Piłka, Szymon Darocha, Marcin Waligóra, Grzegorz Kopeć, Michał Florczyk, Adam Torbicki, Tatiana Mularek-Kubzdela, Anna Smukowska-Gorynia, Ewa Lewicka, Ewa Mroczek, Łukasz Chrzanowski, Piotr Błaszczak, Beata Kuśmierczyk, Katarzyna Ptaszyńska, Katarzyna Mizia-Stec, Ewa Malinowska, Małgorzata Peregud-Pogorzelska, Michał Tomaszewski, Wojciech Jacheć, Ilona Skoczylas, Zbigniew Gąsior, Agnieszka Pawlak, Miłosz Jaguszewski, Grzegorz Grześk, Katarzyna Betkier-Lipińska, Piotr Pruszczyk, Katarzyna Widejko, Judyta Winowska-Józwa, Marcin Kurzyna

PMC · DOI: 10.1186/s13023-026-04250-4 · Orphanet Journal of Rare Diseases · 2026-02-25

## TL;DR

This study finds that patients with pulmonary arterial hypertension who respond well to a vasoreactivity test and calcium channel blockers have better long-term outcomes.

## Contribution

The study provides novel insights into the long-term benefits of calcium channel blockers in a large Polish PAH population.

## Key findings

- Acute responders to vasoreactivity testing had better functional and prognostic outcomes compared to non-responders.
- Long-term responders to calcium channel blockers showed significantly lower mortality rates.
- Early post-CCB assessment is critical for predicting long-term prognosis in PAH patients.

## Abstract

The 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.

The 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.

Novel analysis in a unique large Polish PAH population highlights that APV testing and rapid early post-CCB assessment are key to long-term prognosis.

## Linked entities

- **Diseases:** pulmonary arterial hypertension (MONDO:0015924)

## Full-text entities

- **Genes:** NPPB (natriuretic peptide B) [NCBI Gene 4879] {aka BNP, Iso-ANP}
- **Diseases:** Pulmonary Hypertension (MESH:D006976), PAH (MESH:D000081029)
- **Chemicals:** APV (MESH:C095108)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

4 figures with captions in the complete paper: https://tomesphere.com/paper/PMC13040805/full.md

## References

6 references — full list in the complete paper: https://tomesphere.com/paper/PMC13040805/full.md

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Source: https://tomesphere.com/paper/PMC13040805