Pulmonary Hypertension Clinical Trial
Official title:
CompRehensive Phenotypic Characterization of Patients With Scleroderma-Associated Interstitial Lung DiseasE and Pulmonary Hypertension (PH): The CRuSADE PH Study
Patients with interstitial lung disease (ILD) and scleroderma who develop pulmonary hypertension (PH) do not fit well into the current classification system and treatments for pulmonary hypertension. This study aims to better understand patients with ILD-PH and scleroderma and to determine if treatment with Macitentan is beneficial.
The investigators aim to use pressure-volume loop derived right ventriculo-vascular coupling,
pulmonary impedance, and invasive cardiopulmonary exercise testing (CPET) to:
1. Comprehensively phenotype patients with scleroderma ILD-PH and pulmonary vascular
exercise limitation (PVL) relative to scleroderma ILD-PH without PVL.
2. Compare the efficacy of chronic Macitentan therapy in improving 1) right ventricular
hemodynamics 2) exercise capacity and 3) symptoms in scleroderma ILD-PH patients with
and without PVL.
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