Treatment options for patients with advanced pulmonary hypertension (PH) are limited. Iatrogenic creation of an interatrial communication (i.e., atrial septostomy [AS]) has been suggested as a possible treatment option or as a bridge to transplantation in selected patients and has been incorporated into current PH guidelines. Uptake of the procedure has been slow and the worldwide experience with AS is limited to approximately 280 published cases, over a period of more than 25 years. The rationale for creating an AS has been provided by the observation that patients with congenital heart disease, shunt lesions and PH have a better survival compared with patients with idiopathic PH. We review pathophysiologic data and the published clinical experience and discuss the rationale, indication and potential pitfalls of AS in patients with severe PH.