Valve sparing aortic root replacement in an 8-month-old infant with Loeys-Dietz syndrome.
Valve sparing aortic root replacement in an 8-month-old infant with Loeys-Dietz syndrome.
Ann Thorac Surg. 2018 Oct 10;:
Authors: Wisniewski K, Singer S, Kehl HG, Nawrocki P, Januszewska K, Malec E
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 33mm (z-score +9.3). To avoid aortic rupture a valve sparing aortic root replacement was performed at the age of 8 months. The purpose of this report was to share our dilemmas and experience in the treatment of this child.
PMID: 30315797 [PubMed - as supplied by publisher]
Source: The Annals of Thoracic Surgery - Category: Cardiovascular & Thoracic Surgery Authors: Wisniewski K, Singer S, Kehl HG, Nawrocki P, Januszewska K, Malec E Tags: Ann Thorac Surg Source Type: research
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