Moderate Aortic Enlargement and Bicuspid Aortic Valve Are Associated With Aortic Dissection in Turner Syndrome: Report of the International Turner Syndrome Aortic Dissection Registry

Carlson, Misty; Airhart, Nathan; Lopez, Leo; Silberbach, Michael

doi:10.1161/circulationaha.111.088633

Cited by 181Aortic Disease and Treatment ApproachesConnective tissue disorders researchCardiovascular Issues in Pregnancy

Moderate Aortic Enlargement and Bicuspid Aortic Valve Are Associated With Aortic Dissection in Turner Syndrome: Report of the International Turner Syndrome Aortic Dissection Registry

Carlson, Misty, Airhart, Nathan, Lopez, Leo, Silberbach, Michael·2012·Circulation

DOI 10.1161/circulationaha.111.088633

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Abstract

BACKGROUND: Girls and women with Turner syndrome are at risk for aortic dissection and rupture. However, the size of the aorta and the clinical characteristics among those with Turner syndrome and dissection have received little attention. METHODS AND RESULTS: We obtained medical records from 20 individuals who voluntarily participated in the International Turner Syndrome Aortic Dissection Registry. Type A dissections occurred in 17 of 20 (85%) cases, and type B occurred in 3 cases of which 1 occurred after coarctation stent placement. Of those with spontaneous aortic dissections, 18 of 19 (95%) had an associated cardiac malformation that included a bicuspid aortic valve. In 1 individual there was no predisposing finding other than the presence of Turner syndrome. Associated pregnancy was documented in 1 of 19 (5%). More than half (13/19, 68%) came to medical attention >24 hours after the onset of symptoms. For those with type A dissections, the mean ascending aortic size index was 2.7±0.6 cm/m(2) (n=9). CONCLUSIONS: Aortic dissection in Turner syndrome occurs in young individuals at smaller aortic diameters than in the general population or other forms of genetically triggered aortopathy. The absence of aortic valve or other cardiac malformations appears to markedly reduce the risk of aortic dissection However, aortic dissection can occur in Turner syndrome without cardiac malformations or hypertension. Individuals with Turner syndrome who are >18 years of age with an ascending aortic size index >2.5 cm/m(2) should be considered for an aortic operation to prevent aortic dissection.

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Dissections occur at a relatively young age in TS (mean 30-35 years), like other genetically triggered aortopathies.
“Dissections occur at a relatively young age in TS (mean 30-35 years), like other genetically triggered aortopathies.435”
From Clinical practice guidelines for the care of girls and women with Turner syndrome. (2024) · cites this paper as [435]

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Authors: Carlson, M., Airhart, N., Lopez, L., & Silberbach, M.
Affiliations: Oregon Health & Science University (US)
ORCID: Airhart, Nathan
Lopez, Leo
Silberbach, Michael
Venue: Circulation
Publisher: Ovid Technologies (Wolters Kluwer Health)
Published: 2012-10-30
Volume: 126
Issue: 18
Pages: 2220-2226
ISSN: 0009-7322 (ISSN-L)
1524-4539
DOI: 10.1161/circulationaha.111.088633
OpenAlex: W1986790597
Cited by: 181
References: 24
Citations by year: 2026: 5 · 2025: 7 · 2024: 8 · 2023: 15 · 2022: 14 · 2021: 11 · 2020: 15 · 2019: 13 · 2018: 21 · 2017: 12 · 2016: 14 · 2015: 19 · 2014: 13 · 2013: 11 · 2012: 2
FWCI: 11.59
Type: journal article
Language: en
Open access: closed
Locations: Circulation (publishedVersion)
PubMed (publishedVersion)
Funders: National Center for Research Resources (UL1RR024140)
Topics: Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Aortic Disease and Treatment Approaches
Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Connective tissue disorders research
Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiovascular Issues in Pregnancy
Keywords: Turner syndrome, aortic dissection, bicuspid aortic valve, aortic dilatation, aortopathy, cardiac anomalies, pregnancy, aortic surgery, aortic rupture
MeSH terms: Abnormalities, Multiple, Adolescent, Adult, Aortic Dissection, Anthropometry, Aorta, Aortic Aneurysm, Aortic Coarctation, Aortic Valve, Female, Heart Defects, Congenital, Cardiac Surgical Procedures, Humans, Hypertension, Middle Aged, Postoperative Complications, Pregnancy, Pregnancy Complications, Surveys and Questionnaires, Registries, Retrospective Studies, Risk Factors, Turner Syndrome, Ultrasonography, Young Adult

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Moderate Aortic Enlargement and Bicuspid Aortic Valve Are Associated With Aortic Dissection in Turner Syndrome: Report of the International Turner Syndrome Aortic Dissection Registry

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