Cardiopulmonary Exercise Testing Reveals Onset of Disease and Response to Treatment in a Case of Heritable Pulmonary Arterial Hypertension

Abstract

Patients affected by pulmonary arterial hypertension (PAH) show a typical pattern of abnormalities on cardiopulmonary exercise testing (CPET). However, CPET is not routinely used as a screening method. We discuss a patient with hereditary PAH in whom CPET revealed onset of disease. Furthermore, we show that the abnormalities observed can improve in part by PAH-specific treatment.