Abstract
BackgroundAortic valve regurgitation leading to coronary steal phenomenon can severely impair cardiac function in hypoplastic left heart syndrome, thus worsening long-term outcome.Case presentationA German infant with borderline aortic and mitral valve, hypoplastic left ventricle, ventricular septal defect, and hypoplastic aortic arch with critical coarctation initially underwent aortic arch reconstruction and aortic valve dilation with the aim of biventricular correction later on. Unfortunately, severe cardiac dysfunction necessitated a change in strategy entailing modified stage I Norwood palliation. Increasing aortic regurgitation with coronary steal was revealed postoperatively, which required redo surgery to oversew the valve. However, pronounced aortic regurgitation recurred, causing severe cardiac decompensation with repeated resuscitation. As a bailout strategy, we performed aortic valve closure via transfemoral retrograde implantation of an Amplatzer Duct Occluder II device. This led to the patient’s rapid stabilization while circumventing highly risky renewed surgery in such a critically ill infant.ConclusionsRetrograde transcatheter aortic valve closure may be considered a feasible alternative in infants with a failing single ventricle due to aortic regurgitation, with critical device evaluation being crucial for successful device implantation in this young age group.
Highlights
Hypoplastic left heart syndrome (HLHS) is characterized by variably underdeveloped left-sided heart structures, mostly leading to single-ventricle physiology
We present a novel, retrograde approach for transcatheter aortic valve (AoV) closure as a bailout strategy for aortic regurgitation (AR) in HLHS
We observed recurrent, significant AR (Fig. 1; Additional file 1: Video S1), with the patient demonstrating signs of impaired coronary perfusion in stressful situations, including repeated events of cardiopulmonary decompensation requiring resuscitation. Due to this unstable hemodynamic situation, we opted against further surgery in favor of transcatheter AoV closure
Summary
Retrograde transcatheter AoV closure is a viable bailout option in the context of failing single-ventricle palliation with significant AoV regurgitation. Angiogram of the ascending aorta demonstrating severe regurgitation of the native aortic valve. Angiogram of the ascending aorta demonstrating unobstructed coronary arteries during positioning of the device. Angiogram of the ascending aorta after release of the device showing mild residual aortic regurgitation. Informed consent was provided by the patient’s parents for all operations and interventions mentioned in the report. A copy of the written consent is available for review by the Editor-in-Chief of this journal. Author details 1Department of Congenital Heart Disease and Pediatric Cardiology, University Heart Center Freiburg – Bad Krozingen, Medical Center – University of Freiburg, Faculty of Medicine, Mathildenstrasse 1, 79106 Freiburg, Germany. Author details 1Department of Congenital Heart Disease and Pediatric Cardiology, University Heart Center Freiburg – Bad Krozingen, Medical Center – University of Freiburg, Faculty of Medicine, Mathildenstrasse 1, 79106 Freiburg, Germany. 2Department of Cardiovascular Surgery, University Heart Center Freiburg – Bad Krozingen, Medical Center – University of Freiburg, Faculty of Medicine, Hugstetter Strasse 55, 79106 Freiburg, Germany
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