Valve Sparing Aortic Root Replacement in Children with Loeys-Dietz Syndrome
نویسندگان
چکیده
Loeys-Dietz syndrome (LDS) is an autosomal dominant connective tissue disorder that is characterized by aggressive arterial and aortic disease, often involving the formation of aortic aneurysms. We describe the cases of two children with LDS who were diagnosed with aortic root aneurysms and successfully treated by valve-sparing aortic root replacement (VSRR) with a Valsalva graft. VSRR is a safe and suitable operation for children that avoids prosthetic valve replacement.
منابع مشابه
Valve-sparing aortic root replacement in Loeys-Dietz syndrome and a novel mutation in TGFBR2
BACKGROUND Loeys-Dietz syndrome (LDS) is a recently recognized aggressive aortic disorder characterized by root aneurysm, arterial tortuosity, hypertelorism, and bifid uvula or cleft palate. The results of prophylactic root replacement using valve-sparing procedures (valve-sparing root replacement [VSRR]) in patients with LDS is not known. METHODS We reviewed all patients with clinical and ge...
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We describe a successful surgical treatment in a 2.5-year old boy with Loeys-Dietz syndrome, in whom we performed aortic arch and ascending aorta replacement with a valve-sparing operation (VSO) of the aortic root because of significant aortic insufficiency and dilation of the aortic root. We believe that VSO is ideal for treating young patients with aortic root aneurysm with normal or minimall...
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OBJECTIVES We aimed at evaluating the results of aortic valve-sparing root replacement (AVSRR) in children with aortic root aneurysm (ARA) due to genetic disorders in terms of mortality, reoperation and recurrent aortic valve regurgitation (AVR). METHODS Thirteen patients (mean age 9.7 ± 6.5 years, 10 months-18 years) underwent AVSRR for ARA between 2002 and 2011. Six of the 13 patients had M...
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Loeys-Dietz syndrome (LDS) is a recently recognized connective tissue disorder caused by mutations of the transforming growth factor (TGF)-β receptors. It is an autosomal dominant syndrome characterized by the triad of arterial tortuosity and aneurysms, hypertelorism, and bifid uvula or cleft palate. We treated an 18-year-old woman with a 100-mm-diameter aortic root aneurysm and severe aortic v...
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Thoracic aortic aneurysm (TAA) is a genetically mediated disease with variable age of onset. In the pediatric age range, nonsyndromic TAA frequently has a milder course than syndromic forms of TAA, such as Marfan syndrome or Loeys-Dietz syndrome. Herein, we describe 17-year-old identical twin brothers with severe progressive TAA due to a novel de novo ACTA2 mutation. Interestingly, both boys we...
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