SESSION TITLE: Pulmonary Vascular Disease SESSION TYPE: Affiliate Case Report Slide PRESENTED ON: Tuesday, October 31, 2017 at 04:30 PM - 05:30 PM INTRODUCTION: A Gerbode defect is a ventricular septal defect (VSD) communicating directly between the left ventricle (LV) and right atrium (RA). The shunt can be either congenital or acquired. Acquired cases are secondary to cardiothoracic surgeries. CASE PRESENTATION: A 66 year-old woman was hospitalized with acute on chronic diastolic heart failure. She had a history of mitral valve papillary muscle rupture replaced with a bioprosthesis and bypass for coronary artery disease. BP was 99/69 (MAP 70), HR 70 bpm, with normoxia at rest and with exertion. Exam showed an internal jugular vein with prominent v-wave, loud holosystolic murmur, right sided heave and 3+ edema. Lab results showed a hemoglobin of 17 mg/dL, creatinine 1.6 mg/dL and BNP 2584 pg/mL. Surface echocardiogram revealed preserved LV function, markedly dilated right ventricle with flattening of the intraventricular septum, severe tricuspid regurgitation (TR), tricuspid annular plane systolic excursion of 1.6 cm, TR peak velocity jet of 3.3 m/sec and a negative bubble study. A transesophageal echocardiogram showed a large left to right communication in the posterior area of the atria with a high velocity systolic flow. Several right heart catherizations showed persistent oxygen step up at the level of the right atrium. DISCUSSION: The presence of the shunt complicated hemodynamic interpretation. This elevated the pulmonary artery saturation falsely increasing the estimated Fick cardiac output (CO). The low CO by thermodilution and renal insufficiency were consistent with a hemodynamic shock state, and improved with furosemide, sildenafil and midodrine. LV-RA shunt correction was not possible in this patient due to severe RV dysfunction and high peri-operative risk. CONCLUSIONS: Gerbode defect is a VSD communicating directly between the LV and RA. Sildenafil has been shown to reduce pulmonary pressures and improve hemodynamics in patients with combined PH. Longstanding hydrostatic pressure within the pulmonary capillaries with persistent high flow produced venulae damage and vascular remodeling similar to other causes of pulmonary artery hypertension. We describe a patient with combined pre and post-capillary PH with progressive right heart failure successfully treated with medical therapy. Reference #1: Opitz CF, Hoeper MM, Gibbs SR, et al. Pre-capillary, combined, and post-capillary pulmonary hypertension: a pathophysiological continuum. J Am Coll Cardiol 2016 Reference #2: Yuan Shi-Min. A systematic review of acquired left ventricle to right atrium shunt. Hellenic J Cardiol 2015 DISCLOSURE: The following authors have nothing to disclose: Domingo Franco-Palacios, Patrick Kicker, Oksana Shlobin, Qiong Zhao, Mardi Gomberg-Maitland No Product/Research Disclosure Information