Aortic dissection in Turner's syndrome

Lie, J.T.

doi:10.1016/0002-8703(82)90577-4

Cited by 39 publications

(9 citation statements)

References 6 publications

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“…Although the literature reports a possibly higher surgical risk in Turner girls, caused by the possible association of this syndrome with hystopathologic changes within the aortic wall [2,4,6,11,14,16,25,26], especially in those patients with AoCo or aortic dilatation [3,18], we did not encounter surgical complications in our cases. Only in two patients with BicAo did we observe a mild dilatation of the aortic root, which remained unchanged at follow-up; nevertheless, the possible associa- Table 3.…”

Section: Discussionmentioning

confidence: 63%

Turner's Syndrome: Cardiologic Profile According to the Different Chromosomal Patterns and Long-Term Clinical Follow-Up of 136 Nonpreselected Patients

et al. 1999

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The preferential association between Turner's syndrome and congenital heart defects (CHD) have been well known since the first description by Morgagni. There are few studies about the different cardiologic problems stemming from different chromosomal patterns of X monosomies. We reviewed a large series of 136 patients with Turner syndrome without cardiologic preselection, 29 of whom had some kind of CHD (21.5%). Partial anomalous pulmonary venous drainage (PAPVD; 2.9%), aortic valve disease (stenosis and/or incompetence) (AoVD; 5. 1%), aortic coarctation (AoCo; 4.4%), and bicuspid aortic valve (BicAo; 14.7%) are much more frequent in Turner's syndrome than in the normal population, with the difference being statistically highly significant. In our cases, only the 45, X subjects showed severe CHD and multiple lesions, whereas the X-ring pattern was associated with an elevated prevalence of BicAo. Patients with X-deletion showed no signs of congenital heart malformations. Eleven patients, all with 45, X pattern, and significant CHD, underwent cardiac surgery at a mean age of 7.7 +/- 5.3 years (range 7 days-18 years) without complications. At follow-up of 3-18 years (8.6 +/- 5. 2), we were unable to observe any type of evolution of the remaining untreated cardiovascular anomalies.

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Section: Discussionmentioning

confidence: 63%

Turner's Syndrome: Cardiologic Profile According to the Different Chromosomal Patterns and Long-Term Clinical Follow-Up of 136 Nonpreselected Patients

et al. 1999

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“…1516 However, cystic medial necrosis is not a specific disease; it is found at autopsy in approximately 2% of the general population. 17 Moreover, cystic medial necrosis has been associated with Ehlers-Danlos syndrome, 1819 Turner's syndrome, 20 …”

Section: Marfan's Syndrome and Cystic Medial Necrosismentioning

confidence: 99%

Coexistence of fibromuscular dysplasia and cystic medial necrosis in a patient with Marfan's syndrome and bilateral carotid artery dissections.

Schievink

Björnsson

Piepgras

1994

Stroke

118

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A primary arteriopathy is suspected in most patients with spontaneous dissections of the carotid artery, although the nature of this arteriopathy usually remains elusive. Angiographic changes of fibromuscular dysplasia (FMD), however, are found in 10% to 20% of patients with carotid dissections. A 26-year-old woman with Marfan's syndrome presented with bilateral amaurosis fugax after surgical repair of an aortic dissection. Angiography revealed a dissection extending from the ascending aorta into the right internal carotid artery and, separate from the aortic dissection, a dissection of the left internal carotid artery. After surgery, microscopic examination of the right carotid artery revealed a medial dissection but no evidence of an underlying arteriopathy, while the left internal carotid artery displayed the typical features of FMD. Eighteen months later the patient died after resection of a large thoracoabdominal aortic dissecting aneurysm. Microscopic examination of the aorta revealed moderately extensive cystic medial necrosis. Carotid dissections associated with Marfan's syndrome may be the result of an extension of an aortic dissection or occur isolated from the aorta, even in the same patient. The occurrence of FMD in a patient with Marfan's syndrome in conjunction with previous reports of FMD in a variety of connective tissue disorders suggests that FMD, like cystic medial necrosis, may be a nonspecific disease entity.

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“…Clearly, this lesion and coarctation of the aorta frequently occur either alone or in combination. In this syndrome, aortic aneurysms and idiopathic dilatation of the aorta are less commonly found (Asch, 1979;Lie, 1982;Miller et al, 1983). In those cases in which aortic pathology was described, cystic medial necrosis was found (Lie, 1982).…”

Section: Case Reportmentioning

confidence: 97%

“…In this syndrome, aortic aneurysms and idiopathic dilatation of the aorta are less commonly found (Asch, 1979;Lie, 1982;Miller et al, 1983). In those cases in which aortic pathology was described, cystic medial necrosis was found (Lie, 1982). In addition, there was a high correlation of either associated coarctation of the aorta, bicuspid aortic valve, or hypertension (Asch, 1979;Lie, 1982).…”

Section: Case Reportmentioning

confidence: 99%

Aortic dilatation resulting in chronic aortic regurgitation and complicated by aortic dissection in a patient with turner's syndrome

Goldberg

Pizzarello

Goldman

et al. 1984

Clinical Cardiology

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Summary:A patient with XO Turner's syndrome with a 12-year history of progressive aortic root dilatation resulting in chronic aortic regurgitation is presented. Her case is unique in that it occurred in the absence of coarctation of the aorta, bicuspid aortic valve, or hypertension. Idiopathic dilatation of the aorta may be an additional risk factor to the development of aortic dissection in the setting of Turner's syndrome.

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Aortic dissection in Turner's syndrome

Cited by 39 publications

References 6 publications

Turner's Syndrome: Cardiologic Profile According to the Different Chromosomal Patterns and Long-Term Clinical Follow-Up of 136 Nonpreselected Patients

Turner's Syndrome: Cardiologic Profile According to the Different Chromosomal Patterns and Long-Term Clinical Follow-Up of 136 Nonpreselected Patients

Coexistence of fibromuscular dysplasia and cystic medial necrosis in a patient with Marfan's syndrome and bilateral carotid artery dissections.

Aortic dilatation resulting in chronic aortic regurgitation and complicated by aortic dissection in a patient with turner's syndrome

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