ACC ACPC Council Reviews D-TGA Hot Topics

Since it was first successfully performed in 1975, the arterial switch operation (ASO) has been the definitive means to manage D-loop transposition of the great arteries (D-TGA), with survival rates only increasing with the refinement of surgical techniques and improved medical management. Today, the majority of patients who are treated by the procedure live to adulthood, with a 20-year survival rate of nearly 90 percent.

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In a state-of-the-art review published July 28 in the Journal of the American College of Cardiology, Juan Villafañe, MD, FACC, Department of Pediatrics, University of Kentucky, and his colleagues on behalf of the ACC's Adult Congenital and Pediatric Cardiology Council, attempt to update clinicians on the "hot topics" in the management of patients with D-TGA and an intact – or virtually intact—ventricular septum who undergo the ASO. They summarize the current knowledge on genetics, pre-natal diagnosis, surgical timing, balloon atrial septostomy, prostaglandin E1 therapy, intraoperative techniques, imaging, coronary obstruction, arrhythmias, sudden death, neoaortic regurgitation and dilation, neurodevelopmental (ND) issues, and lifelong care of D-TGA patients.

According to the authors, the key takeaways include: a low risk of familial recurrence; improved cognitive skills in children diagnosed pre-natally compared with those diagnosed post-natally; the success of echocardiography in identifying risk factors; routine use of balloon atrial septostomy and prostaglandin E1 may not be indicated in all cases; early ASO improves outcomes and reduces costs with a low mortality; single or intramural coronary arteries remain risk factors; post-ASO arrhythmias and cardiac dysfunction should raise suspicion of coronary insufficiency; coronary insufficiency and arrhythmias are rare but are associated with sudden death; early- and late-onset ND abnormalities are common; aortic regurgitation and aortic root dilation are well tolerated; and aging ASO patients may benefit from "exercise-prescription" rather than restriction.

"Significant strides have been made in understanding risk factors for cardiac, ND, and other important clinical outcomes after ASO," the authors note. They add that while the lifelong consequences of ASO remain speculative and will require time to test its updated management, overall the therapy is a landmark surgical procedure, one that will continue to benefit innumerable patients with D-TGA.

Keywords: Cognition, Ventricular Septum, Pediatrics, Survival Rate, Death, Sudden, Transposition of Great Vessels, Risk Factors, Echocardiography

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