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Rolling With The Punches: Successful Management Of High-risk Single Ventricle Anatomy With Heterotaxy Syndrome Requiring Multiple Atrioventricular Valve Reinterventions
William Blake Swicord, MD, James M. Meza, MD, MSc, Winfield Wells, MD, John D. Cleveland, MD.
Children's Hospital of Los Angeles, Los Angeles, CA, USA.
Objective(s): Heterotaxy in the single ventricle population is complex and associated with adverse outcomes. Here we present the course of a child with heterotaxy and several high-risk anatomical features, who underwent a total of five atrioventricular valve (AVV) interventions before developing heart failure requiring a ventricular assist device (VAD) and heart transplantation.
Methods: We review of the clinical course of a patient with heterotaxy/asplenia syndrome, unbalanced right-dominant atrioventricular canal defect, side-by-side great arteries, bilateral superior vena cavae, and infradiaphragmatic total anomalous pulmonary venous return (TAPVR), and recurrent AVV regurgitation.
Results: The patient initially underwent neonatal repair of infradiaphragmatic TAPVR and pulmonary artery (PA) banding at 1 week old followed by bidirectional Glenn at 5 months old. He developed AVV regurgitation one month later, requiring first AVV repair. At four years old, he underwent Fontan and a second AVV repair. He again developed significant AVV regurgitation, resulting in bioprosthetic AVV replacement ten months later. He then required mechanical then bioprosthetic AVV re-replacements before VAD support for 285 days and cardiac transplantation at seven years old.
Conclusions: Heterotaxy presents distinct clinical challenges within the single ventricle population and requiring both persistence and the nimbleness to adjust management plans as dictated by the child’s clinical condition. Despite a constellation of high-risk features (neonatal TAPVR repair, multiple AVV reintervention, and VAD support), he is doing well after eight total cardiac surgeries and ultimate cardiac replacement.
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