Aortic Dilatation and Dissection in Turner Syndrome

Abstract

Background-The risk for aortic dissection is increased among relatively young women with Turner syndrome (TS). It is unknown whether aortic dilatation precedes acute aortic dissection in TS and, if so, what specific diameter predicts impending deterioration. Methods and Results-Study subjects included 166 adult volunteers with TS (average age, 36.2 years) who were not selected for cardiovascular disease and 26 healthy female control subjects. Ascending and descending aortic diameters were measured by magnetic resonance imaging at the right pulmonary artery. TS women were on average 20 cm shorter, yet average aortic diameters were identical in the 2 groups. Ascending aortic diameters normalized to body surface area (aortic size index) were significantly greater in TS, and Ϸ32% of TS women had values greater than the 95th percentile of 2.0 cm/m 2 . Ascending diameter/descending diameter ratios also were significantly greater in the TS group. During Ϸ3 years of follow-up, aortic dissections occurred in 3 women with TS, for an annualized rate of 618 cases/100 000 woman-years. These 3 subjects had ascending aortic diameters of 3.7 to 4.8 cm and aortic size indices Ͼ2.5 cm/m 2 . Conclusions-The risk for aortic dissection is greatly increased in young women with TS. Because of their small stature, ascending aortic diameters of Ͻ5 cm may represent significant dilatation; thus, the use of aortic size index is preferred. Individuals with a dilated ascending aorta defined as aortic size index Ͼ2.0 cm/m 2 require close cardiovascular surveillance. Those with aortic size index Ն2.5 cm/m 2 are at highest risk for aortic dissection.

Cardiovascular findings and management in Turner syndrome: insights from a French cohort

“…Aortic diameters were measured at the level of the aortic cusps, the aortic sinuses, the supra-aortic ridge, the proximal ascending aorta, the aortic arch, and the descending aorta. Standardized imaging included morphologic ECG-gated sequences in the axial, coronal, and left-anterior-oblique axes and cine-MR sequences in a coronal view of the ascending aorta and through the aortic valve to check for aortic regurgitation or stenosis and to determine valve morphology (9,10,11).…”

Turner syndrome in childhood and adolescence

“…A major goal of the NIH protocol is detection of congenital cardiovascular defects or anomalies that might identify those at risk for life-threatening complications, such as aortic aneurysm, which foreshortens the lives of too many patients with TS [6,7]. All participants undergo indepth cardiovascular evaluations, including echocardiography and MRI.…”

“…The presence of neck webbing is usually obvious in children and leads to the diagnosis of TS in most cases; it may be more difficult to discern in obese adults. We did not attempt to assess short stature in this group because 75% received or had received GH treatment, nor did we assess the prevalence of premature ovarian failure because of the young age of our study group.A major goal of the NIH protocol is detection of congenital cardiovascular defects or anomalies that might identify those at risk for life-threatening complications, such as aortic aneurysm, which foreshortens the lives of too many patients with TS [6,7]. All participants undergo indepth cardiovascular evaluations, including echocardiography and MRI.…”

Shereshevsky-Turner Syndrome in Aortic Surgery: An Example of Hybrid Aneurism Treatment and Dissection of Thoracoabdominal Aorta

“…Основными факторами риска для увеличения раз-мера аорты при СШТ являются артериальная гипер-тензия, наличие бикуспидального аортального кла-пана, коарктации аорты, и 45, Х кариотип. Также необходимо отметить, что расслоение при СШТ раз-вивается при значимо меньшем диаметре аорты, что в некоторой степени объясняется небольшой ком-плекцией больных [22]. При этом 2/3 случаев рассло-ения приходится на А тип (с вовлечением прокси-мальной части аорты) и чаще развивается у пациен-ток молодого возраста, особенно при беременности [23].…”