The role of calcium channel blockers, steroids, anticoagulation, antiplatelet drugs, and endothelin receptor antagonists

The rationale for the drug therapy of pulmonary artery hypertension is to reduce mortality and morbidity caused by failure of right ventricular adaptation to an elevated pulmonary vascular resistance. We review the evidence for the use of calcium-channel blockers, steroids, anticoagulation, antiplatelet drugs, and endothelin receptor antagonists in the management of pulmonary artery hypertension. The drugs we discuss are more suited to long-term outpatient therapy. These drugs have not found a routine place in intensive care management, and calcium-channel blockers are contraindicated in patients with right-heart failure. The efficacy of many agents has been extrapolated from data acquired in adult patients and applied to children. All of us involved in the care of young patients with pulmonary artery hypertension should advocate for both the inclusion of younger patients in clinical trials and the design of distinctly pediatric trials with pharmaceutical and drug administration agencies. It is only with data derived from pediatric inclusive studies that we shall be able to recommend therapy with strong evidence. However, it is important to point out that the use of newer agents for the treatment of chronic pulmonary artery hypertension (prostacyclin, endothelin receptor antagonists, nitric oxide, and sildenafil) have not been shown to improve survival unequivocally and have relied on surrogates, such as exercise capacity. There are no long-term studies of survival benefit. Recent studies have included data on time to clinical worsening, which may be a more predictive surrogate of survival.