CHSS - Intrinsic Morphology Influences The Natural History Of Neo-aortic Root Dilatation And Pathologic Neo-aortic Regurgitation In Transposition Of The Great Arteries

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Intrinsic Morphology Influences The Natural History Of Neo-aortic Root Dilatation And Pathologic Neo-aortic Regurgitation In Transposition Of The Great Arteries
Aditya Sengupta, MD, MPH, Kimberlee Gauvreau, ScD, Chrystalle K. Carreon, MD, Ji M. Lee, BS, Stephen P. Sanders, MD, Steven D. Colan, MD, Pedro J. del Nido, MD, John E. Mayer, Jr., MD, Meena Nathan, MD, MPH.
Boston Children's Hospital, Boston, MA, USA.

Objective(s): We sought to delineate the natural history of root dilatation in repaired dextro-transposition of the great arteries (dTGA) and histologically characterize the great arteries in unrepaired dTGA.
Methods: A single-center review of patients who underwent the arterial switch operation (ASO) from 07/1981-09/2022 was performed. Morphology was categorized as dTGA with intact ventricular septum (dTGA-IVS), dTGA with ventricular septal defect (dTGA-VSD), and double outlet right ventricle-TGA type (DORV-TGA). Echocardiographically-determined diameters and derived z-scores were measured at the annulus, sinus of Valsalva (SoV), and sinotubular junction (STJ) immediately before the ASO and throughout follow-up. Linear mixed-effects models assessed trends in dimensions over time. We also histologically examined the aortic and pulmonary roots of 10 cases of unrepaired dTGA from our Cardiac Registry.
Results: Of 1359 patients meeting entry criteria, 593 (44%) had dTGA-IVS, 666 (49%) had dTGA-VSD, and 100 (7%) had DORV-TGA. At a median follow-up of 8.3 years (range 0.1-39.3 years), 129 (9.5%) patients had ≥moderate AR or required a valve/root reoperation (Fig-A). Overall growth of the root is shown in Fig-B. At 30 years, DORV-TGA patients had significantly larger diameters at the annulus (p<0.001), SoV (p=0.039), and STJ (p=0.041) relative to dTGA-IVS patients (Fig-C). Specimens of the aortic and pulmonary roots from a representative case of unrepaired dTGA are shown (Fig-D).
Conclusions: Longitudinal surveillance of the neo-aortic root is warranted long after the ASO.

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