Curable Syncope in Primary Pulmonary Hypertension with Novel Atrial Flow Regulator

Pulmonary artery hypertension (PAH) occurs when mean pulmonary artery pressure (PAP) is higher than 25 mmHg in rest or 30 mmHg during activity. Idiopathic pulmonary artery hypertension (IPAH) is defined as PAH without a definite reason. The IPAH is a rare disease with a high mortality rate if left untreated. To date, there has been no definite cure for this entity, and most treatments are for symptom relief or improvement in the quality of life. For many years, decompressing the right heart through a hole in the interatrial septum has been advised to alleviate some of their symptoms, specifically syncope. Atrial flow regulator (AFR) is a device to make an iatrogenic interatrial hole and finally a unidirectional shunt. It has been used for some PAH patients for symptom relief. Herein, we report a 36-year-old female case with diagnosed IPAH for 6 years. In the last 3 years, the case had frequent syncope with shortening intervals. The AFR was implanted for her without any complications. Then, within 6 months of follow-up, she had only one syncope episode. A significant change was observed in her 6-minute walk and PAP.