Pulmonary arterial hypertension in adults with atrial septal defect

Atrial septal defect (ASD) is a congenital heart defect often encountered in the adult population, because many patients are free from overt symptoms for the first few decades and may show limited physical signs. Pulmonary arterial hypertension (PAH) complicating to left-to-right shunting at the atrial level is seen in 6–35% of untreated patients in later adulthood [1–3]. The presence of PAH is associated with older age, female sex, and defect size and causes progressive dyspnea, ascites, edema, presyncope or syncope, functional limitations, persistent right ventricular dilation and significant tricuspid regurgitation after closure of ASD, and increased mid-to-long-term morbidity and mortality [4–8].