A63-17 Large Atrial Septal Defect With Severe Pulmonary Arterial Hypertension: Can a “Treat-And-Repair” Approach Be Done During the Same Hospitalization?

Introduction With advanced pulmonary vasodilator therapies, previously considered “inoperable” intracardiac shunts due to severe pulmonary arterial hypertension (PAH) can undergo a “treat-and-repair” approach, provided pulmonary vascular resistance (PVR) is significantly reduced after months of aggressive treatment. We report on a patient with newly diagnosed severe PAH and a bidirectional atrial septal defect (ASD), considered for closure during the same hospitalization. Case Presentation A patient in her 40s presented with progressive dyspnea and severe hypoxemia. Initial echocardiogram revealed a markedly dilated right ventricle (RV) and RV systolic pressure of 94 mmHg (Fig.1). A bedside bubble study identified an impressive right-to-left shunt.A right heart catheterization (RHC) identified a significant oxygen step-up at the level of the RA: 64% up to 81%. Baseline hemodynamics included: mean pulmonary artery pressure (mPAP) 44 mmHg, pulmonary flow (Qp) 4.3 L/min, systemic flow (Qs) 4.19 L/min, Qp/Qs 1.2, and PVR 7.2 Wood units (WU). Intracardiac echocardiography confirmed a large 21 mm secundum ASD (Fig. 2). Lack of rim around the ASD prevented consideration for percutaneous closure. Given the patient’s lack of insurance, we initiated aggressive PAH therapies with a plan to consider inpatient surgical closure after reevaluation of hemodynamics two weeks later.Repeat RHC revealed a mean pulmonary artery pressure (mPAP) of 40 mmHg. The PVR had decreased from 7.2 to 4.1 WU, and Qp improved from 4.3 to 5.8 L/min (with no change in Qp/Qs), suggesting improvement in RV performance. There was no significant hemodynamic change post-balloon occlusion. After extensive multidisciplinary discussions, the decision was made to proceed with surgical ASD closure under continued IV and oral pulmonary vasodilator therapy. She experienced a dramatic clinical improvement post ASD closure and PAH therapy and was discharged home two weeks later on continuous subcutaneous treprostinil, macitentan/tadalafil combination, and 2 L of oxygen. A follow-up in the PH clinic 4 weeks later confirmed an excellent sustained clinical response. Discussion The consideration for a “treat-and-repair” strategy, complemented by transient ASD balloon occlusion testing, enables clinicians to identify patients who may safely benefit from definitive ASD closure. This strategy has been considered potentially safe if PVR becomes <.6.5 WU after several months of parenteral therapies. To our knowledge, our patient is the first reported case of ASD and severe pulmonary hypertension who, after only a few weeks of inpatient PAH therapy, safely underwent ASD closure during the same hospitalization. This highlights the benefits of a multidisciplinary approach in complex PH cases. This abstract is funded by: None