THE COMPLICATION-RIDDEN DESTINY OF THE SYSTEMIC RIGHT VENTRICLE IN L-TRANSPOSITION OF THE GREAT ARTERIES: MANAGEMENT DILEMMAS

Document Type

Conference Proceeding

Publication Date

3-7-2023

Publication Title

J Am Coll Cardiol

Abstract

Background: Congenitally corrected levo-transposition of the great arteries (L-TGA) is a congenital heart disease in which the ventricles and great arteries are transposed from their typical anatomy. In L-TGA, the double discordance, atrioventricular and ventriculoarterial, create an acyanotic milieu which allows patients to survive their early decades, however, progressive systemic right ventricle (sRV) dysfunction creates complications later on.

Case: A 40-year-old male with L-TGA presented with symptoms of acute decompensated heart failure (ADHF). In childhood, he had surgical repair of a ventricular septal defect. In adulthood, he developed sRV dysfunction, systemic tricuspid valve (sTV) regurgitation, and left-bundle branch block for which he underwent cardiac resynchronization therapy. Transthoracic echocardiogram obtained during the admission showed a sRV ejection fraction of 40%, severe sTV regurgitation, and a newly identified sRV apical thrombus; the thrombus was confirmed by ultrasound-enhancing agents and transesophageal echocardiography.

Decision-making: Our patient was optimized with guideline-directed medical therapy and diuresis. The presence of a sRV thrombus posed a dilemma given the limited literature. Guidelines for intracardiac thrombus in patients with structurally typical hearts recommend anticoagulation with a vitamin K antagonist (VKA) followed by echocardiography to assess for resolution. However, multiple case reports and small-scale studies support the use of direct oral anticoagulants. It is unknown whether these principles can be extrapolated to patients with congenital heart disease. Review of literature identified no cases of sRV thrombus making this one of the first reports. Our patient was anticoagulated with a VKA and later referred for evaluation by advanced heart failure and heart transplant services.

Conclusion: We describe one of the first reported cases of sRV thrombus in L-TGA presenting with ADHF. This case illustrates the natural history of L-TGA and highlights the importance of surveillance and monitoring in these patients with dedicated cardiac imaging including advanced imaging modalities to identify complications.

Volume

81

Issue

8

First Page

3056

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