Ace the Case: Pulmonary Arterial Hypertension (PAH) Presentation and Evaluation by Pulmonary Specialist

Pulmonary hypertension (PH) comprises a heterogeneous group of disorders that share a common clinical feature - mean pulmonary artery pressure greater than 20 mm Hg. PH may involve multiple clinical conditions and may be associated with a variety of cardiovascular and respiratory diseases. Early diagnosis is critical to ensure patients are eligible for the widest spectrum of treatment options and that treatment is initiated as early as possible in the disease process. PH is classified into 5 clinical groups based on etiology, pathophysiology, and treatment.

Pulmonary arterial hypertension (PAH) is 1 of the 5 groups of PH. Hemodynamically, PAH is defined by (a) a right heart catheterization showing a mean pulmonary artery pressure >20 mm Hg; (b) a pulmonary artery wedge pressure ≤15 mm Hg; and (c) a pulmonary vascular resistance of ≥3 Wood units. PAH is further divided into subgroups based on underlying etiology: idiopathic PAH, heritable PAH, drug- and toxin-associated PAH, pulmonary veno-occlusive disease, PAH in long-term responders to calcium channel blockers, and persistent PH of the newborn, as well as PAH associated with other medical conditions including connective tissue disease, HIV, and congenital heart disease.

The activities in this series are designed for the primary and pulmonary care team (physician, APP, nurse, pharmacist) engaged in the care of patients with PAH.