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Standardized Technique of Aortic Valve Re-implantation for Valve-sparing Aortic Root Replacement
Published on: December 11, 2017
Valve-Sparing Aortic Root Replacement in an 8-Month-Old Infant With Loeys-Dietz Syndrome
Konrad Wisniewski1, Sybille Singer2, Hans-Gerd Kehl2
1Division of Pediatric Cardiac Surgery, University Hospital Muenster, Muenster, Germany.
Abstract:
The clinical experience with Loeys-Dietz syndrome (LDS) reveals fateful natural history with intracerebral incidents and aortic dissections. A newborn child was referred to our hospital with significantly dilated aortic root and clinical signs of LDS phenotype later genetically confirmed as LDS type I. A therapy with antihypertensive medicines was initiated to postpone the surgery. Despite that, aortic root dilatation progressed to 33 mm (z-score +9.3). To avoid aortic rupture, a valve sparing aortic root replacement was performed at patient age of 8 months. The purpose of this report is to share our dilemmas and experience in the treatment of this child.
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