Winning With Four: Hemodynamic and Symptomatic Improvement of Pulmonary Arterial Hypertension With Contemporary Quadruple Therapy.

BACKGROUND

Pulmonary arterial hypertension (PAH) is a progressive disease characterized by high morbidity and mortality. We present a case highlighting significant clinical improvement of severe PAH with severe right ventricular dysfunction with contemporary quadruple PAH therapies.

CASE PRESENTATION: A 68-year-old man presented with exertional dyspnea and hypoxia. Right heart catheterization confirmed precapillary pulmonary hypertension with right ventricular dysfunction and low cardiac output. Initial upfront traditional triple therapy with uptitration of treprostinil led to modest improvement. After Food and Drug Administration approval, addition of sotatercept further significantly improved hemodynamics, right ventricular function, biomarkers, and functional status.

DISCUSSION: This case highlights the evolving management of PAH and the importance of rapid diagnosis. Initiation of quadruple therapy with monitoring was key to the remarkable improvement in symptoms and objective parameters seen in our patient.

TAKE-HOME MESSAGE: Managing pulmonary arterial hypertension in patients with multiple comorbidities is challenging and requires a multidisciplinary approach with aggressive upfront therapy leading to significant symptomatic and hemodynamic improvement.