Sequential Add-On Therapy Modifies Mortality Risk Stratification in Group 1.4 Pulmonary Arterial Hypertension: A...

Area of Science:

• Cardiology
• Pulmonology
• Pharmacology

Background:

• Dynamic risk stratification is crucial for managing pulmonary arterial hypertension (PAH).
• Limited data exists on sequential add-on therapy's impact in Group 1.4 PAH, especially in Latin American populations.
• This study focuses on macitentan + sildenafil combination therapy.

Purpose of the Study:

• To evaluate changes in risk classification using COMPERA 2.0 and REVEAL Lite 2 scores.
• To assess the impact of sequential add-on endothelin receptor antagonist (ERA) and phosphodiesterase type 5 inhibitor (PDE5i) therapy in Group 1.4 PAH patients.
• To analyze clinical, functional, and biochemical parameters before and after 24 weeks of treatment.

Main Methods:

• Single-center, retrospective cohort study of 25 PAH patients.
• Treatment involved macitentan + sildenafil combination therapy.
• Risk scores (COMPERA 2.0, REVEAL Lite 2) and clinical parameters were assessed at baseline and 24 weeks.

Main Results:

• Significant improvement in World Health Organization functional class (p = 0.002).
• Significant reduction in brain natriuretic peptide levels (p = 0.003).
• Both COMPERA 2.0 and REVEAL Lite 2 scores showed a shift towards lower-risk categories with strong concordance.

Conclusions:

• Sequential add-on ERA + PDE5i therapy leads to meaningful risk stratification improvement in Group 1.4 PAH.
• Simplified, noninvasive risk assessment tools are clinically useful in real-world, resource-constrained settings.
• Findings support the use of macitentan + sildenafil for PAH management.