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. 2022 May 9;118(6):1059-1066.
doi: 10.36660/abc.20210492. Online ahead of print.

Soluble Guanylate Cyclase Stimulators (Riociguat) in Pulmonary Hypertension: Data from Real-Life Clinical Practice in a 3-Year Follow-Up

[Article in Portuguese, English]
Fernanda Brum Spilimbergo  1 , Taís Silveira Assmann  1 , Marcelo Bellon  1 , Laís Machado Hoscheidt  1 , Cássia Ferreira Braz Caurio  1 , Márcia Puchalski  1 , Bruno Hochhegger  1 , Gabriela Roncato  1   2 , Gisela Martina Bohns Meyer  1
Affiliations

Soluble Guanylate Cyclase Stimulators (Riociguat) in Pulmonary Hypertension: Data from Real-Life Clinical Practice in a 3-Year Follow-Up

[Article in Portuguese, English]
Fernanda Brum Spilimbergo et al. Arq Bras Cardiol. .

Abstract
in English, Portuguese

Background: Pulmonary hypertension (PH) is a rare and complex disease with poor prognosis, which requires lifelong treatment.

Objective: To describe 3-year follow-up real-life data on treatment with soluble guanylate cyclase stimulators (Riociguat) of patients with PH, measuring current risk assessment parameters.

Methods: This study retrospectively collected clinical and epidemiological data of patients with PH of group 1 (pulmonary arterial hypertension) and group 4 (chronic thromboembolic PH). Non-invasive and invasive parameters corresponding to the risk assessment were analyzed at baseline and follow-up. Statistical analyses were performed using the SPSS 18.0 software, and p-values < 0.050 were considered statistically significant.

Results: In total, 41 patients receiving riociguat were included in the study. Of them, 31 had already completed 3 years of treatment and were selected for the following analysis. At baseline, 70.7% of patients were in WHO functional class III or IV. After 3 years of treatment, the WHO functional class significantly improved in all patients. In addition, the median of the 6-minute walk test (6MWT) significantly increased from 394 ± 91 m at baseline to 458 ± 100 m after 3 years of follow-up (p= 0.014). The three-year survival rate was 96.7%.

Conclusion: In our real-life cohort, most patients with PH treated with riociguat showed stable or improved risk parameters, especially in the 6MWT, at 3 years of follow-up.

Fundamento: A hipertensão pulmonar (HP) é uma doença rara e complexa com prognóstico ruim, que exige tratamento pela vida toda.

Objetivo: Descrever dados de 3 anos de acompanhamento da vida real sobre o tratamento com estimuladores de guanilato ciclase solúvel (Riociguate) de pacientes com HP, medindo parâmetros atuais de avaliação de risco.

Métodos: Coletamos dados clínicos e epidemiológicos retrospectivamente de pacientes com HP do grupo 1 (hipertensão arterial pulmonar) e do grupo 4 (HP tromboembólica crônica). Parâmetros não invasivos e invasivos correspondentes à avaliação de risco foram analisados na linha de base e no acompanhamento. Foram realizadas análises estatísticas usando o software SPSS 18.0, e os p-valores <0,050 foram considerados estatisticamente significativos.

Resultados: No total, 41 pacientes tratados com riociguate foram incluídos no estudo. Entre eles, 31 já concluíram 3 anos de tratamento e foram selecionados para a seguinte análise. Na linha de base, 70,7% dos pacientes estavam nas classes funcionais III ou IV da OMS. Depois de 3 anos de tratamento, a classe funcional da OMS melhorou significativamente em todos os pacientes. Além disso, a mediana do teste de caminhada de 6 minutos (TC6M) aumentou significativamente de 394 ± 91 m na linha de base para 458 ± 100 m após 3 anos de acompanhamento (p= 0,014). O índice de sobrevida após três anos foi de 96,7%.

Conclusão: Em nossa coorte de vida real, a maioria dos pacientes com HP tratados com riociguate demonstraram parâmetros de risco estáveis ou melhores, especialmente no TC6M, aos 3 anos de acompanhamento.

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Conflict of interest statement

Potencial conflito de interesse

Fernanda Brum Spilimbergo – Honorários de palestra e consultoria: Bayer, Eli Lilly e GSK.

Marcelo Bellon – Honorários de palestra e consultoria: Bayer, Eli Lilly e GSK.

Gabriela Roncato – Funcionária da Bayer

Gisela Martina Bohns Meyer – Honorários de palestra e consultoria: Bayer, Eli Lilly e GSK.

Figures

Figura 1
Figura 1. – Fluxograma dos pacientes durante o estudo. HAP: hipertensão arterial pulmonar; HPTEC: hipertensão pulmonar tromboembólica crônica.
Figura 2
Figura 2. – Alteração ao longo do tempo na classe funcional da OMS para pacientes com hipertensão pulmonar. A) Dados de todos os 41 pacientes na linha de base e nos períodos de acompanhamento. B) Dados de todos os 31 pacientes que concluíram 3 anos de acompanhamento.
Figura 3
Figura 3. – Alteração ao longo do tempo no teste da caminhada de seis minutos (TC6M) em pacientes com hipertensão pulmonar. *p-valor< 0,05; +p-valor< 0,10; Teste t de Student pareado comparado com a linha de base.
Figura 4
Figura 4. – Alteração ao longo do tempo no fragmento do Peptídeo natriurético pró-cerebral N-terminal (NT-proBNP) para pacientes com hipertensão pulmonar.
Figure 1
Figure 1. – Flowchart of patients throughout the study. PAH: pulmonary arterial hypertension; CTEPH: chronic thromboembolic pulmonary hypertension.
Figure 2
Figure 2. – Change of WHO functional class over time in patients with pulmonary hypertension. A) Data from all 41 patients in baseline and follow-up periods. B) Data from the 31 patients who completed 3 years of follow-up.
Figure 3
Figure 3. – Change of 6-min walking test (6MWT) over time in patients with pulmonary hypertension. *p-value< 0.05; +p-value< 0.10; Paired Student’s t-test compared to Baseline.
Figure 4
Figure 4. – Change of N-terminal pro-brain natriuretic peptide (NT-proBNP) over time in patients with pulmonary hypertension.
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