SESSION TITLE: Medical Student/Resident Cardiovascular Disease Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Atrial septal defects (ASD) is a common congenital abnormality discovered in adulthood but ostium primum ASDs are more rare with an incidence of ∼15% of ASDs.2 ASDs with coexisting pulmonary valve stenosis is rare but has been described in Noonan Syndrome.1 About 66% of Noonan Syndrome patients exhibit congenital heart defects.3,4 ASDs and coexisting valvular pulmonary stenosis in the absence of Noonan syndrome is very rare. CASE PRESENTATION: 62 year-old Hispanic male with CAD with prior PCI, hypertension, and hypothyroidism who presented with progressive dyspnea and palpitations. He was found to be in new-onset atrial fibrillation with ventricular rates in the 160’s BPM. Labs revealed normal electrolytes and TSH, elevated BNP (1226pg/ml), and negative troponin-I. CXR was unremarkable. He was rate controlled, anti-coagulated, and underwent successful TEE with 200J of DCCV with restoration of sinus rhythm. His TEE showed a normal EF but a RA measuring 5.1cm (ID) in end-systole along with a membrane in the posterior aspect of the RA (prominent eustachian valve versus cor triatriatum dextrum), mildly dilated RV, mild MR, and a large primum ASD measuring 4.91cm2 by planimetry. A TTE (in sinus rhythm) showed mild-mod pulmonary stenosis and mild pulmonary regurgitation by doppler (mean systolic gradient 17.00mmHg, peak systolic gradient 30.00mmHg) along with dilated pulmonary arteries with possible flow reversal in the right pulmonary artery. Left heart cath showed non-obstructive CAD. CTA of the pulmonary veins showed no anomalous pulmonary vein return but did show a 32mm main pulm vein, 42mm left pulm artery, 28mm right pulm artery, and known ASD measuring 24x30mm. He remained in sinus rhythm and was discharged. DISCUSSION: The natural progression of ASDs with left-to-right shunting leads to Eisenmenger syndrome. This case represents interesting hemodynamics, such that his valvular pulmonary stenosis served as a protective factor against significant left-to-right shunting due to the outflow obstruction. The patient presented with new AFib, which is common in patients with ASD due to the atrial dilation over time from the shunting. This patient likely presented with symptoms later in life due to his valvular pulmonary stenosis minimizing his left-to-right shunting through his ASD, and thereby delaying his right atrial dilation. This combination is seen with Noonan syndrome, but this case is unique in that the patient did not have any other known congenital syndrome.1,3 Although not formally genetically tested, he exhibited no phenotypic features associated with Noonan syndrome and did not have a family history of any chromosomal abnormalities, therefore the diagnosis is unlikely. CONCLUSIONS: We report a case of coexisting ostium primum ASD and valvular pulmonary stenosis (in absence of Noonan Syndrome) demonstrating valvular outflow obstruction preventing left-to-right shunting through the ASD. Reference #1: Ma LK, Ma PTS, Leung AKC. Pulmonary Valve Stenosis with Atrial Septal Defect. In: Lang F, ed. Encyclopedia of Molecular Mechanisms of Disease. Berlin, Heidelberg: Springer Berlin Heidelberg; 2009:1779-80. Reference #2: Webb G, Gatzoulis MA. Atrial Septal Defects in the Adult. Circulation 2006;114:1645-53 Reference #3: Allanson JE. Noonan syndrome. Journal of medical genetics 1987;24:9. DISCLOSURES: No relevant relationships by Emmanuel Bassily, source=Web Response No relevant relationships by Fahad Hawk, source=Web Response no disclosure on file for Bibhu Mohanty