Exercise Pulmonary Hypertension and Beyond: Insights in Exercise Pathophysiology in Pulmonary Arterial Hypertension (PAH) from Invasive Cardiopulmonary Exercise Testing.

Pulmonary arterial hypertension (PAH) is a rare, progressive disease of the pulmonary vasculature that is associated with pulmonary vascular remodeling and right heart failure. While there have been recent advances both in understanding pathobiology and in diagnosis and therapeutic options, PAH remains a disease with significant delays in diagnosis and high morbidity and mortality. Information from invasive cardiopulmonary exercise testing (iCPET) presents an important opportunity to evaluate the dynamic interactions within and between the right heart circulatory system and the skeletal muscle during different loading conditions to enhance early diagnosis, phenotype disease subtypes, and personalize treatment in PAH given the shortcomings of contemporary diagnostic and therapeutic approaches. The purpose of this review is to present the current applications of iCPET in PAH and to discuss future applications of the testing methodology.