Diagnosis of Pulmonary Hypertension

Pulmonary hypertension (PH) is a chronic disease of elevated pulmonary artery pressure that can result from pulmonary vascular diseases or complicate left heart and lung disease. Pulmonary arterial hypertension (PAH) is a rare pulmonary artery vasculopathy that leads to progressive right heart failure and death. Timely and accurate diagnosis of PH is paramount, given the increased morbidity and mortality, but can be challenging given the nonspecific nature of the presenting symptoms and the many potential causative or contributing conditions. The diagnosis of PH remains clinical and the initial workup uses history, physical exam, and echocardiography to evaluate likelihood of disease, followed by characterization of left heart and lung disease and the appropriate evaluation for chronic thromboembolic disease. A right heart catheterization is requisite for the diagnosis and thus early referral to a PH expert center is strongly recommended, particularly for patients with high-risk features and in high-risk populations.