Orphanet journal of rare diseases · 2026
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).
СтатьяМногоцентровое исследованиеНазвание и аннотация: Перевод в очереди
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Piłka Michał, Darocha Szymon, Waligóra Marcin, Kopeć Grzegorz, Florczyk Michał, Torbicki Adam, Mularek-Kubzdela Tatiana, Smukowska-Gorynia Anna, Lewicka Ewa, Mroczek Ewa, Chrzanowski Łukasz, Błaszczak Piotr, Kuśmierczyk Beata, Ptaszyńska Katarzyna, Mizia-Stec Katarzyna, Malinowska Ewa, Peregud-Pogorzelska Małgorzata, Tomaszewski Michał, Jacheć Wojciech, Skoczylas Ilona, Gąsior Zbigniew, Pawlak Agnieszka, Jaguszewski Miłosz, Grześk Grzegorz, Betkier-Lipińska Katarzyna, Pruszczyk Piotr, Widejko Katarzyna, Winowska-Józwa Judyta, Kurzyna Marcin
Аннотация
BACKGROUND: 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.
RESULTS: 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.
CONCLUSIONS: 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.
RESULTS: 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.
CONCLUSIONS: 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.