SURPASSING THE FIFTH DECADE: EBSTEIN’S ANOMALY CASE REPORT

Abstract

SESSION TITLE: Cardiovascular Disease 2 SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/09/2018 01:15 PM - 02:15 PM INTRODUCTION: Ebstein's anomaly (EA) is a rare congenital heart defect in which the septal and posterior leaflets of the tricuspid valve are displaced towards the apex of the right ventricle. Although it accounts for only 0.5% of congenital heart defects, only 5% of patients survive beyond the fifth decade.1,5 We report a newly diagnosed patient with this condition in the seventh decade of life. CASE PRESENTATION: A 72-year-old female with a history of hypertension and prior stroke without residual deficits presented with new-onset seizures. She was hypertensive, tachycardic, and tachypneic, requiring intubation. Physical exam showed jugular venous distention, bilateral crackles, and bilateral pedal edema. X-ray showed cardiomegaly with pulmonary vasculature cephalization. EKG indicated right axis deviation, atrial fibrillation, complete RBBB, and prolonged QTc. 2D-Echo confirmed features of EA including severe tricuspid regurgitation (TR) with tethering of leaflets; right ventricular and atrial dilation; and vena cava dilatation. The displacement index (DI) was recorded at 0.90 mm/m2. Left ventricular EF∼50% with moderate wall thickening. Her hospital course involved resolution of her seizure activity, acute respiratory failure, and acute kidney injury. Her atrial fibrillation and hypertension were treated with AV nodal blocking agents, ARBs, loop diuretics, and oral anticoagulation. Despite her severe TR, she responded well to medical management and continued to improve after the incident. DISCUSSION: EA ranges from minimal to severe TR, consequently affecting the age and degree of clinical presentation.4 Because TR causes right atrial dilatation and reduced right ventricular function, arrhythmias typically occur as well. Other diagnoses we considered based upon echo findings included carcinoid syndrome and Uhl’s anomaly. In carcinoid syndrome, the leaflets are thickened and retracted. In Uhl’s anomaly, the right ventricular myocardium is thin with normal valvular function and preserved left ventricular function.6 The presence of the characteristic displacement of the septal leaflet of the tricuspid valve, in tandem with the abnormal DI and persistent dilated vena cava, supports an Ebstein diagnosis.5 For older patients, medical management and reassessments of functional capacity are indicated.2 Though no formalized guidelines exist, candidacy for tricuspid repair, replacement, or ablation is reserved for those with poor functional reserve or intractable arrhythmias.2,4 Even with this stratification, surgical interventions have remained equivocal and yielded more cardiac re-presentations.3 CONCLUSIONS: Our patient is atypical for being asymptomatic even after surpassing the fifth decade of life with EA, likely attributable to her decreased DI within the spectrum of EA. Our case strengthens the scarce evidence available for the role of conservative management in an asymptomatic elderly. Reference #1: D’ Andrea et al. An atypical assessment of Ebstein’s Anomaly in an elderly patient, report of case. Monaldi Arch Chest Dis. 2011;76:104–1052. Reference #2: Kawase I et al. Repair of Ebstein’s Anomaly in an elderly patient; report of a case. Kyobu Geka. 2008;61:887–890. Reference #3: Kim HYI et al. Natural Course of Adult Ebstein’s Anomaly When Treated according to Current Recommendation. Korean Med Sci. 2016 Nov;31(11):1749-1754.https://doi.org/10.3346/jkms.2016.31.11.1749. Reference #4: Yesin et al. Ebstein’s Anomaly in an asymptomatic nonagenarian: A case report. Curr Res: Cardiol 2015;2(1):40-42. Reference #5: Moradi et al. Complex Ebstein’s Anomaly in an 86-Year-Old Iranian Man: A Case Report. J Tehran Heart Cent. 2017 Jan;12(1):39-41. Reference #6: Ammash et al. Mimics of Ebstein’s Anomaly. Am Heart J. 1997 Sep;134(3):508-13. DISCLOSURES: No relevant relationships by Haleem Abdul, source=Web Response No relevant relationships by Ramsey Ataya, source=Web Response No relevant relationships by Rahul Dasgupta, source=Web Response No relevant relationships by Walter Ramirez, source=Admin input