Cessation of pulmonary vasodilators in PAH related to congenital heart disease

Pulmonary arterial hypertension secondary to congenital heart diseases is a subset of patients who need pulmonary vasodilators (PVDs). We are reporting an adult female who continued to have severe pulmonary hypertension after corrective surgery for the atrial septal defect. She showed improvement in symptoms and effort tolerance assessed by 6-min walk distance, but on cessation of therapy, showed worsening of exercise tolerance which improved after reintroduction. This report stresses the importance of the need for long-term combination of PVDs without interruption.