Systemic Sclerosis-Associated Pulmonary Hypertension: Three Cases with Distinct Hemodynamic Profiles

Pulmonary arterial hypertension (PAH) is a devastating complication of systemic sclerosis (SSc) and is a leading contributor to mortality and morbidity. This series demonstrates the clinical and hemodynamic heterogeneity within PAH-SSc and the importance of personalized care. Herein, we describe three connective tissue disease (CTD) women with SSc and PAH at various times of their disease. Patient 1: isolated postcapillary PAH in context of left heart disease and atrial flutter; patient 2: combined PAH with interstitial lung disease and systemic hypertension; patient 3: precapillary PAH with heart failure with preserved ejection fraction (HFpEF) profile. All were verified by right heart catheterization and treated with specific therapies for PAH. This series highlights the heterogeneity of PAH in SSc and the need for early recognition, multimodal assessment and individualized treatment to enhance functional status and quality of life in these complex patients.