Emerging therapies in pulmonary hypertension

posted Jan 26, 2017, 1:41 AM by sourav ghosh
S. Ramakrishnan
Professor of Cardiology,All India Institute of Medical Sciences, New Delhi,India

Idiopathic pulmonary hypertension (IPAH) is a rare disease associated with high morbidity and mortality. The pathogenesis is not fully understood. Diagnostic evaluation focuses on ruling out other aetiologies of pulmonary hypertension and prognosticating the disease. Congenital heart disease and left sided heart disease associated pulmonary hypertension are more common in India. Therapies for idiopathic pulmonary hypertension have evolved over the past decade. Phosphodiesterase 5 inhibitors (sildenafil, tadalafil), endothelin antagonists (Bosentan and ambrisentan) and prostanoids are the class of drugs shown to be useful in pulmonary hypertension. An initial combination of ambrisentan and tadalafil may be beneficial in selected patients with IPAH. Riociguat and macitentan are the newer agents approved for use in PAH following landmark clinical trials confirming their efficacy. After prostanoids, initial combination therapy, riociguat and macitentan have shown improvement in hard clinical end points. Balloon atrial septostomy, Pott’s shunt, stem cell therapy, and radiofrequency ablation are the other therapies that could benefit an IPAH patient with refractory symptoms. Despite these advances, the outcomes of various forms of pulmonary hypertension remains poor especially in India, where some forms of therapy are not available and patients often are diagnosed at an advanced stage of disease.