Page 26 - Journal of Structural Heart Disease Volume 4, Issue 1

Case Report
Journal of Structural Heart Disease, February 2018, Volume 4, Issue 1:17-20
DOI: https://doi.org/10.12945/j.jshd.2018.031.17
Received: July 04, 2017 Accepted: July 25, 2017 Published online: February 2018
The First Case of Successful Stenting of the
Dissection of the Ascending Aorta and the Aortic
Arch that Occurred During Surgical Correction
of the Supralvular Aortic Stenosis in a Child with
the Williams Syndrome
Manolis Georgievich Pursanov, MD, Professor*,Konstantin Valentinovich Shatalov, Professor, Andrey Vasiljevitch Sobolev, PhD, Irina Vladimirovna Arnautova, PhD
Department of Endovascular and Intraoperative Diagnostic and Treatment of Cardiovascular Disease, Bakoulev Scienti c Center for Cardiovascular Surgery, Moscow, Russian Federation
Abstract
Congenital supravalvular aortic stenosis (SVAS), in vast majority of cases is a manifestation of Williams syn- drome. Surgical correction of this pathology is a "gold standard" for treatment in these patients. One of the most dangerouse potential complications in surgical repair of this disease is acute dissection of the ascend- ing aorta, arising in 0.7% of cases. This complication can be attributed to both surgical errors and or due to the anatomical features of the aortic wall. We report on a pediatric patient with Williams syndrome, 2.3 yr, 11.9 kg, who underwent surgical repair for supravalvar aor- tic stenosis using the Doty technique. Aortic dissection was diagnosed using aortography. This was managed by implanting two Valeo stents.
Copyright © 2018 Science International Corp.
Key Words
Williams syndrome • Aortic dissection • Stenting
Introduction
Congenital supravalvular aortic stenosis (SVAS), in the vast majority of cases, is a manifestation of Wil- liams syndrome or other pathological condition as- sociated with mutation of the 7q11.23 gene [1-4]. In
patients with Williams syndrome or SVAS, other ob- structive vascular lesions are often present such as coarctation of the aorta and peripheral stenoses of the pulmonary arteries. Patients with Williams syn- drome also often have a bicuspid aortic valve and mitral valve prolapse, as well as various heart rhythm disturbances [6-8].
SVAS is a complex pathology that may present in a wide variety of clinical and morphological forms. The most common form is aortic lumen obstruction local- ized directly above the aortic valve [5, 9]. The severity of stenosis is the main factor determining the severity of hemodynamic burden and thus the clinical mani- festation of this pathology. In most cases, the clinical picture of the disease manifests during childhood or adolescence, and most patients require surgical cor- rection of the defect, which is performed under car- diopulmonary bypass [1, 10, 11].
Although surgery is safe and successful in most cases, there is the rare potential complication of dis- section of the ascending aorta after its surgical recon- struction. This complication can be attributed to both surgical errors and anatomical features of the aortic wall. The incidence of this complication is 0.6% [12- 13]. Here, we describe a child with Williams syndrome
* Corresponding Author:
Manolis Georgievich Pursanov, MD
Department of Endovascular and Intraoperative Diagnostic and Treatment of Cardiovascular Disease
Bakoulev Scienti c Center for Cardiovascular Surgery
Rublevskoe Shosse 135, Россия, Moscow, 121552, Russian Federation
Tel. +8 495 414 78 89; Fax: +8 926 529 32 23; E-Mail: mpursanov@gmail.com
Fax +1 203 785 3346
E-Mail: jshd@scienceinternational.org http://structuralheartdisease.org/
© 2018 Journal of Structural Heart Disease Published by Science International Corp. ISSN 2326-4004
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