Dissection of the aorta in Turner syndrome: two cases and review of 85 cases in the literature

Abstract

Patients with Turner syndrome (TS) are at risk for aortic dissection, but the clinical profile for those at risk is not well described. In addition to reporting two new cases, we performed an electronic search to identify all reported cases of aortic dissection associated with TS. In total, 85 cases of aortic dissection in TS were reported between 1961 and 2006. Dissection occurred at a young age, 30.7 (range 4–64) years, which is significantly earlier than its occurrence in the general female population (68 years). Importantly, in 11% of the cases, neither hypertension nor congenital heart disease were identified, suggesting that TS alone is an independent risk factor for aortic dissection; however, the cases where no risk factors were identified were very poorly documented. A TS aortic dissection registry has been established to determine the natural history and risk factors better (http://www.turnersyndrome.org/).

Zaburzenia w budowie aorty u pacjentek z zespołem Turnera — nowe metody diagnostyki i leczenia

“…In contrast, the International Registry of Acute Aortic Dissection found that the mean age of dissection in non-TS women is 68 years [36,38].…”

Editor's Choice – Management of Descending Thoracic Aorta Diseases

“…These studies should be repeated every 5e10 years, or earlier if clinically indicated by pregnancy or by known risk factors for AD such as hypertension, bicuspid aortic valve, or coarctation. 288 A recent study investigating the effect of supraphysiological doses of growth hormone to increase adult height in patients with TS, which may have direct effects on the cardiovascular system, revealed that this regimen neither seems to affect ascending or DTA diameter beyond the increase related to the larger body size nor does it disproportionately affect cardiac dimensions. 289 3.7.1.5.…”

“…While apparently all TS patients younger than 20 years have some structural congenital heart disease, about 10% of adults with TS that have died from aortic dissection have no evidence of major structural defects on autopsy. 288 Evidence for regular screening for aortic disease in women with TS is not available. A baseline cardiovascular evaluation should be obtained at the time of diagnosis and includes MRI in addition to echocardiography.…”

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

“…У пациенток с СШТ наиболее частой находкой является двустворчатый аортальный клапан (12-30%) [16,17]. Коарктацию аорты обнаруживают у 7-18% больных с СШТ.…”

“…Аневризма аорты развивается у данной группы пациентов в 100 раз чаще, чем в популяции [18][19][20][21]. 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.…”