BLADE BALLOON ATRIAL SEPTOSTOMY IN PATIENTS WITH SEVERE PRIMARY PULMONARY-HYPERTENSION

Background Patients with severe primary pulmonary hypertension have a poor prognosis, but those with a patent foramen ovale may survive long er. A few reports of clinical improvement after blade balloon atrial s eptostomy in patients with severe pulmonary vascular disease have appe ared. The purpose of this study was to systematically evaluate the eff ects of blade balloon atrial septostomy on clinical signs and symptoms , hemodynamics, and survival in patients with severe primary pulmonary hypertension. Methods and Results Blade balloon atrial septostomy was performed on 15 children and young adults with severe primary pulmona ry hypertension. Despite maximal medical therapy, prior to septostomy all patients had recurrent syncope and 8 had severe right heart failur e. Thirteen patients survived the procedure. After blade balloon atria l septostomy, no patient experienced further syncope, and signs and sy mptoms of right heart failure improved in all New York Heart Associati on Class IV patients. Within 24 hours after the procedure and at follo w-up catheterization 7 to 27 months after septostomy, there was a sign ificant increase in cardiac index, resulting in an increase in systemi c oxygen transport. There was improved long-term survival in the 13 pa tients who survived blade balloon atrial septostomy compared with simi lar groups of primary pulmonary hypertension patients who received sta ndard therapy (P<.05). Conclusions Blade balloon atrial septostomy res ulted in clinical and hemodynamic improvement and improved survival in selected patients with severe primary pulmonary hypertension.