The Evolving Landscape of Pulmonary Hypertension Treatment: From Vasodilation to Disease Modification

Pulmonary hypertension (PH) is a complex disorder associated with significant morbidity and mortality. PH is characterized by an elevated pulmonary vascular load, leading to right ventricular dysfunction and remodeling, with right heart failure and premature death if left untreated. PH is classified into five groups (WHO Groups 1-5) based on etiology and pathophysiology. Group 1 PH, known as pulmonary arterial hypertension (PAH), has seen recent therapeutic advances with life-extending treatments, including a new therapeutic option that targets the underlying vascular abnormalities. PAH treatment guidelines now emphasize early diagnosis, risk stratification, and targeting multiple pathophysiologic pathways with upfront combination therapy. In contrast to PAH treatment, strategies for other forms of PH remain limited and focus on managing pathophysiologic processes outside the pulmonary vasculature, including left heart disease (Group 2), lung disease and/or hypoxia (Group 3), or pulmonary artery obstructions (Group 4). Nevertheless, advances in research have introduced novel therapeutic targets and investigational agents, offering hope for future expanded treatment options. In this review, we explore recent advances in PH treatments, highlighting the developments poised to expand the therapeutic landscape for this complex disease.