Multifactorial Pulmonary Hypertension: Converging Roles of Sarcoidosis, Heart Failure With Reduced Ejection Fraction,...

Pulmonary hypertension (PH) is a multifactorial condition associated with elevated pulmonary arterial pressures. Treatment focuses on the identification and treatment of underlying causes. Here, we present the case of a 50-year-old man with advanced pulmonary sarcoidosis, recent pulmonary embolisms, and heart failure with reduced ejection fraction (HFrEF) of 35% who was found to have severe PH. This case describes a 50-year-old man with severe, multifactorial PH arising from three distinct World Health Organization (WHO) classifications. The patient, who has a history of advanced pulmonary sarcoidosis and HFrEF, presented with gastrointestinal bleeding and hemodynamic instability. Diagnostic workup, including a right heart catheterization, revealed significantly elevated pressures, including a mean pulmonary arterial pressure of 71 mmHg and a transpulmonary gradient of 54 mmHg. The complexity of his condition stems from the overlap of Group 2 PH (venous congestion from HFrEF), Group 4 PH (chronic thromboembolic disease), and Group 5 PH (sarcoidosis-related fibrosis and vascular compression). Management of such mixed-etiology PH requires a highly tailored approach. For the Group 2 component, treatment focuses on goal-directed medical therapy (GDMT) for heart failure, including beta-blockers and sodium-glucose cotransporter 2 (SGLT2) inhibitors. The Group 4 component necessitates long-term anticoagulation and evaluation for surgical endarterectomy or riociguat therapy. Finally, the Group 5 component, driven by sarcoidosis, requires controlling systemic inflammation. As this case illustrates, when PH is multifactorial and refractory to standard interventions, lung transplantation remains the definitive consideration for long-term survival.