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Riociguat for CTEPH: A retrospective cohort study from a single reference center in Brazil
Caio Julio Cesar Dos Santos Fernandes1, William Salibe-Filho1, José Leônidas Alves-Jr1
1Instituto do Coração, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (HCFMUSP), São Paulo, SP, Brazil.
Insights
Riociguat treatment improved functional capacity and reduced BNP levels in Chronic Thromboembolic Pulmonary Hypertension (CTEPH) patients in Brazil. Hemodynamic improvements were not significant, highlighting the need for further research.
Area of Science:
- Cardiology
- Pulmonology
- Pharmacology
Background:
- Chronic Thromboembolic Pulmonary Hypertension (CTEPH) is a severe condition often requiring specialized treatment.
- Riociguat is a medication approved for pulmonary hypertension, but its effects in CTEPH patients in Brazil require specific evaluation.
Purpose of the Study:
- To assess the efficacy of Riociguat in improving clinical outcomes and risk stratification in Brazilian CTEPH patients.
- To evaluate changes in functional capacity, biomarker levels, and hemodynamic parameters following Riociguat treatment.
Main Methods:
- An observational, retrospective study involving 8 CTEPH patients treated with Riociguat at a Brazilian reference center.
- Assessment of NYHA functional class, BNP levels, 6-minute walk distance, and hemodynamic parameters pre- and post-treatment.
- Risk stratification using the COMPERA 2.0 score.
Main Results:
- Significant improvements in NYHA functional class, 6-minute walk distance, and BNP levels were observed (p < 0.05).
- Risk stratification scores (COMPERA 2.0) showed significant improvement.
- No significant changes were noted in hemodynamic parameters such as mean pulmonary arterial pressure or cardiac index.
Conclusions:
- Riociguat treatment positively impacts functional capacity and biomarker levels in CTEPH patients, aligning with international findings.
- The lack of significant hemodynamic changes suggests a need for larger prospective studies.
- Limited Riociguat availability in Brazil presents a challenge for CTEPH management.
Objective:
To evaluate the effects of Riociguat treatment in patients with Chronic Thromboembolic Pulmonary Hypertension (CTEPH) at a Brazilian reference center.
Methods:
This observational study was conducted at INCOR-SP and included 8 patients diagnosed with CTEPH between 2016 and 2024 who were treated with Riociguat. Clinical parameters (NYHA functional class, BNP levels, and 6-minute walk distance) and hemodynamic measurements (right atrial pressure, cardiac index, and pulmonary vascular resistance) were assessed pre- and post-treatment, along with risk stratification using COMPERA 2.0.
Results:
The retrospective cohort (mean age 56 ± 17 years; 100 % female) comprised 2 inoperable patients and 6 with residual PH post-PEA. Significant improvements were observed in functional class (p < 0.05), 6-minute walk distance (increased from 398.5 ± 45 m to 413 ± 110 m; p < 0.05), and BNP levels (decreased from 140 ± 191 pg/mL to 28 ± 235 pg/mL; p < 0.05). Additionally, risk stratification according to COMPERA 2.0 improved significantly. However, no significant changes were detected in the hemodynamic parameters (mean pulmonary arterial pressure, right atrial pressure, cardiac index, or pulmonary vascular resistance).
Conclusion:
Riociguat treatment significantly enhanced functional capacity, reduced BNP levels, and improved risk stratification in CTEPH patients, corroborating international evidence. The absence of significant hemodynamic changes underscores the need for larger, prospective studies to fully elucidate the cardiovascular impact of Riociguat. Importantly, the limited availability of Riociguat in Brazil remains a critical challenge that hinders the effective management of CTEPH.
