Balloon Atrial Septostomy (BAS) in the Management of Severe Idiopathic PAH

Abstract

SESSION TITLE: Fellow Case Report Poster - Obstructive Lung Disease SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM INTRODUCTION: PAH is a progressive condition that can produce right heart failure (RHF), multiple organ dysfunctions and death, cure is not available yet and mortality rates are high. BAS is a percutaneous treatment employed to create a communication in the interatrial septum. It can be a palliative alternative or bridge-therapy towards lung transplantation (LT)1. We present the case of a young woman with severe PAH whose clinical condition was stabilized using BAS and then maintained in functional class (FC) II for more than 5 years with maximal combined therapy, including IV Epoprostenol (Epo) CASE PRESENTATION: The patient was a 24-years-old woman with three-years history of tachycardia, chest pain, syncope and secondary convulsive syndrome. At physical examination: mild cyanosis, auscultation with P2 reinforcement but no murmurs, crackles or wheezing, and evidence of hepatomegaly and inferior limb edema. Chest Rx showed prominent pulmonary artery without lung infiltrates. Echocardiogram with PASP 124 mmHg, severe dilatation of RV, tricuspid insufficiency and small-size LV. Chest CT (-) for pulmonary embolism without lung infiltrates. Spirometry and CO diffusion were normal. Right heart catheterization reported EFLV of 65%, PAP 134 mm Hg, severe dilatation of RV, no intracardiac shunt and normal wedge pressure. Combined treatment was initiated but multiple admissions occurred due to decompensation with RHF. On 2009, SAB was performed followed by adequate symptoms’ control. The patient was discharged with triple therapy, Bosentan, Sildenafil and IV Epo, since 2010, with clinical relief and good quality of life to the date. DISCUSSION: Elective BAS is recommended in PAH FC III/IV with refractory RHF or syncope and SpO2 >85% as a bridge for LT or as palliative therapy when LT is not available. The procedure is perform under mild sedation, fluoroscopy and SpO2 surveillance while progressive dilatation is done CONCLUSIONS: In this case, elective BAS was useful to stabilize clinical status of the patient while IV Epo was initiated. This management has maintained her condition stable until today. Reference #1: 1.Chiu JS, et al Balloon atrial septostomy in pulmonary arterial hypertension: effect on survival and associated outcomes. J Heart Lung Transplant 2015 Mar,34(3):376-80 DISCLOSURE: The following authors have nothing to disclose: Liliana Fernandez, Juan Martinez, Juan Gomez, Diana Carrillo, Leidys Gutierrez No Product/Research Disclosure Information