Consecutive Sessions of Rescue Balloon Atrial Septostomy for an Idiopathic Pulmonary Arterial Hypertension Patient with Refractory Right Heart Failure – Usefulness of Intracardiac Echocardiography Guidance

摘要

For idiopathic pulmonary artery hypertension (PAH) patients with end-stage right heart failure who received maximal medical therapy, balloon atrial septostomy (BAS) is recommended by most guidelines as a palliative therapy or a bridging treatment before lung transplantation. In this report, we described a 32-year-old woman with idiopathic PAH, who received maximal PAH-specific medical treatment, including intravenous prostacyclin, but still suffered from refractory right heart failure. The markedly enlarged right atrium (RA), high mean RA pressure of 23 mmHg, low systemic arterial oxygen saturation of 86% and concomitant pancytopenia all increased the patient’s risk for BAS. We used intracardiac echocardiography (ICE) guidance to facilitate trans-septal puncture, and performed graded BAS four times within 7 months to stabilize the patient. Our case showed that with dedicated PAH treatment, an experienced structural heart interventionist and ICE guidance, BAS could be done safely even in a patient in unfavorable clinical and hemodynamic condition.