Shifting gears: the search for group 3 pulmonary hypertension treatment

Treatment options for Group 3 pulmonary hypertension, characterized as secondary to chronic hypoxia or lung disease, remain an elusive holy grail for physicians and patients alike. Despite increasing identification and investigation into this pulmonary vasculopathy group with the second-highest frequency and highest mortality, there are no therapeutic interventions that offer the significant improvements in morbidity and mortality comparable to those benefiting other pulmonary hypertension groups including pulmonary arterial hypertension. This review examines the data on available and emerging Group 3 pulmonary hypertension treatments.

Pulmonary vasodilators have yielded equivocal results in this patient population, although recent evidence shows modestly improved outcomes with inhaled treprostinil in interstitial lung disease-associated pulmonary hypertension. With pulmonary vasodilators providing limited benefit, emerging data support the right ventricle as a potential treatment target in Group 3 pulmonary hypertension.

Group 3 pulmonary hypertension is associated with significant morbidity and mortality. Pulmonary vasodilators offer only limited haemodynamic and exertional benefits, and lung transplantation remains the only cure for this deadly disease. The right ventricle may provide a novel intervention target.