Surgical Management

Evaluation and Diagnosis of Pulmonary Arterial Hypertension

Idiopathic pulmonary arterial hypertension (formerly referred to as primary pulmonary hypertension) is an uncommon yet progressively fatal disease defined by the presence of mean pulmonary artery pressure greater than 25mmHg at rest or greater than 30mmHg with exercise as tested by right heart catheterization in the absence of other etiologies for pulmonary hypertension.

Pulmonary Arterial Hypertension in Adults with Congenital Heart Disease

Congenital heart disease is the most common congenital malformation and accounts for about eight cases per 1,000 births.1 Due to tremendous developments in cardiac surgery, nearly 90% of all children with congenital heart disease reach adult age. In patients with congenital heart disease, pulmonary arterial hypertension (PAH) may develop due to increased pulmonary arterial flow as a result of a left-to-right shunt. PAH may lead to a decreased functional capacity and right ventricular failure, and is often associated with early death.