Aortic dissection and turner’s syndrome: case report and review of the literature

Bordeleau, Louise; Cwinn, A. Adam; Turek, Michèle; Barron-Klauninger, Kathy; Victor, Gary

doi:10.1016/s0736-4679(98)00041-9

Cited by 41 publications

(22 citation statements)

References 20 publications

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“…As noted earlier, an altered compliance of the aorta exists in TS, 19,29 as well as enlargement of other large vessels, including brachial and carotid arteries, 18,29 suggesting a general vasculopathy. In a few cases for which pathology was available, cystic medial necrosis was reported, 23,24,30 similar to that found in Marfan syndrome.…”

Section: Discussionsupporting

confidence: 53%

Aortic Dilatation and Dissection in Turner Syndrome

et al. 2007

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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.

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Section: Discussionsupporting

confidence: 53%

Aortic Dilatation and Dissection in Turner Syndrome

et al. 2007

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“…Bicuspid aortic valve, coarctation of the aorta, and systemic hypertension have been identified in approximately 90% of patients with aortic dilatation and dissection. 38,39,43 These are the same risk factors that are associated with aortic root dilatation in the general population. However, because of the current lack of knowledge about the natural history of aortic dilatation, additional research will be required to identify the best strategy for timely discovery of the problem.…”

Section: Cardiovascular Abnormalitiesmentioning

confidence: 90%

Health Supervision for Children With Turner Syndrome

Frías¹,

Davenport²

2003

Pediatrics

109

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ABSTRACT. This report is designed to assist the pediatrician in caring for the child in whom the diagnosis of Turner syndrome has been confirmed by karyotyping. The report is meant to serve as a supplement to the American Academy of Pediatrics' "Recommendations for Preventive Pediatric Care" and emphasizes the importance of continuity of care and the need to avoid its fragmentation by ensuring a medical home for every girl with Turner syndrome. The pediatrician's first contact with a child with Turner syndrome may occur during infancy or childhood. This report also discusses interactions with expectant parents who have been given the prenatal diagnosis of Turner syndrome and have been referred for advice.

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“…In addition, there is an altered distensibility of the aorta in TS [32,33] and enlargement of other large vessels including brachial and carotid arteries [33,35], suggesting a general vasculopathy perhaps related to some connective tissue defect in the syndrome. In many cases where pathology is available, cystic medial necrosis and tissue friability have been reported [19,31,28] similar to that found in Marfan syndrome and the altered compliance of the arterial wall has also been likened to findings in Marfan syndrome [32]. Other signs of connective tissue disorder, such as joint laxity, have not been found in TS.…”

Section: Vasculopathymentioning

confidence: 72%

“…To date there have been 73 reported cases of aortic dissection or rupture in individuals with TS ages 4-64 years [19,[26][27][28][29][30][31]. The best epidemiological information is from a very recent Danish Registry study [30] that reports an overall incidence of 36 cases/ 100,000 patient-years for TS vs. 6/100,000 general population, and for the 30-40 years age group the rate is 78 cases/100,000 patient-years for TS vs. <1 for all others.…”

Section: Aortic Dissectionmentioning

confidence: 99%