Determining the Optimal Approach to Initiating Oral, Inhaled, and Intravenous Therapies in Clinical Practice: Maximal Upfront Therapy Is Best

Abstract

Tremendous progress has been made with pharmacologic therapy for pulmonary artery hypertension (PAH) over the past two decades. Nonetheless, we still consider this disease a deadly foe. Many PAH experts would argue that parenteral prostanoid therapy is the most effective form of therapy for patients with PAH, particularly those with advanced disease. In spite of this, and because other effective and more convenient pharmacotherapies have become available, aggressive parenteral prostanoid therapy is still nearly always reserved for the most advanced PAH cases based upon symptoms, signs, echocardiographic findings, and right-heart catheterization results demonstrating a very low cardiac index and high right atrial pressure. Intravenous therapy is cumbersome, but safe. Two approaches that require further research and exploration are, earlier initiation of parenteral prostanoid therapy and upfront triple therapy; that is, instead of waiting to see if goals are achieved on one drug, starting potent multiple therapies immediately, with consideration of weaning off parenteral therapy if certain goals are met. This approach will be expensive, but must be considered. Our current treatment approaches are improving but remain inadequate.