Current Concepts in Management of Pulmonary Hypertension: Fighting the Old Demon with Modern Weapons

Pulmonary hypertension in children is a rare disease associated with high morbidity and mortality. The pathogenesis is not fully understood. Diagnostic evaluation focuses on ruling out other etiologies of pulmonary hypertension and prognosticating the disease. Congenital heart disease and left sided heart disease associated pulmonary hypertension are more common in children. 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 classes of drugs shown to be useful in pulmonary hypertension. However, use of these drugs in children is based on extrapolation of adult usage and on expert consensus rather than based on randomized controlled trial evidence. Despite these advances, the outcomes of various forms of pulmonary hypertension remain poor, especially in India, where some forms of therapy are not available and children often are diagnosed at an advanced stage of disease.