Valve-Sparing Aortic Root Replacement in Loeys-Dietz Syndrome

Johns Hopkins Division of Cardiac Surgery, Department of Surgery, The Johns Hopkins Hospital, Baltimore, Maryland 21287, USA.
The Annals of thoracic surgery (Impact Factor: 3.85). 08/2011; 92(2):556-60; discussion 560-1. DOI: 10.1016/j.athoracsur.2011.04.003
Source: PubMed


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.
We reviewed all patients with clinical and genetic (transforming growth factor-β receptor mutations) evidence of LDS who underwent VSRR at our institution. Echocardiographic and clinical data were obtained from hospital and follow-up clinic records.
From 2002 to 2009, 31 patients with a firm diagnosis of LDS underwent VSRR for aortic root aneurysm. Mean age was 15 years, and 24 (77%) were children. One (3%) patient had a bicuspid aortic valve. Preoperative sinus diameter was 3.9±0.8 cm (z score 7.0±2.9) and 2 (6%) had greater than 2+ aortic insufficiency. Thirty patients (97%) underwent reimplantation procedures using a Valsalva graft. There were no operative deaths. Mean follow-up was 3.6 years (range, 0 to 7 years). One patient required late repair of a pseudoaneurysm at the distal aortic anastomosis, and 1 had a conversion to a David reimplantation procedure after a Florida sleeve operation. No patient suffered thromboembolism or endocarditis, and 1 (3%) patient experienced greater than 2+ late aortic insufficiency. No patient required late aortic valve repair or replacement.
Loeys-Dietz syndrome is an aggressive aortic aneurysm syndrome that can be addressed by prophylactic aortic root replacement with low operative risk. Valve-sparing procedures have encouraging early and midterm results, similar to those in Marfan syndrome, and are an attractive option for young patients.

Download full-text


Available from: Diane E Alejo, Aug 07, 2015
39 Reads
  • Source
    • "Several centers have accumulated sizeable experience with aortic valve sparing operations [23,33,34,37,53-55]. Table 1 summarizes freedom from reoperation on the aortic valve and freedom from moderate or severe aortic insufficiency in large series with follow-up of at least a decade. "
    [Show abstract] [Hide abstract]
    ABSTRACT: Aortic valve sparing operations were developed to preserve the native aortic valve during surgery for aortic root aneurysm as well as surgery for ascending aortic aneurysms with associated aortic insufficiency. There are basically two types of aortic valve sparing oprations: remodeling of the aortic root and reimplantation of the aortic valve. These operations have been performed for over two decades and the clinical outcomes have been excellent in experienced hands. Although remodeling of the aortic root is physiologically superior to reimplantation of the aortic valve, long-term follow-up suggests that the latter is associated with lower risk of developing aortic insufficiency. Failure of remodeling of the aortic root is often due to dilatation of the aortic annulus. Thus, this type of aortic valve sparing should be reserved for older patients with ascending aortic aneurysm and normal aortic annulus whereas reimplantation of the aortic valve is more appropriate for young patients with inherited disorders that cause aortic root aneurysms. This article summarizes the published experience with these two operations. They are no longer experimental procedures and should be part of the surgical armamentarium to treat patients with aortic root aneurysm and ascending aortic aneurysms with associated aortic insufficiency.
    Korean Journal of Thoracic and Cardiovascular Surgery 08/2012; 45(4):205-12. DOI:10.5090/kjtcs.2012.45.4.205
  • Source

    European journal of cardio-thoracic surgery: official journal of the European Association for Cardio-thoracic Surgery 02/2012; 41(5):1186. DOI:10.1093/ejcts/ezs003 · 3.30 Impact Factor
  • Source

    Journal of the American College of Cardiology 05/2012; 60(5):404-7. DOI:10.1016/j.jacc.2012.03.032 · 16.50 Impact Factor
Show more