Clinical and epidemiological description of aortic dissection in Turner's syndrome
Identifieur interne : 005084 ( Istex/Curation ); précédent : 005083; suivant : 005085Clinical and epidemiological description of aortic dissection in Turner's syndrome
Auteurs : Claus H Jbjerg Gravholt [Danemark] ; Kerstin Landin-Wilhelmsen [Suède] ; Kirstine Stochholm [Danemark] ; Britta Eilersen Hjerrild [Danemark] ; Thomas Ledet [Danemark] ; Christian Born Djurhuus [Danemark] ; Lisskulla Sylvén [Suède] ; Ulrik Baandrup [Danemark] ; Bent Stergaard Kristensen [Danemark] ; Jens Sandahl Christiansen [Danemark]Source :
- Cardiology in the Young [ 1047-9511 ] ; 2006-10.
Abstract
Background: Women with Turner's syndrome have an increased risk of congenital cardiac malformations, ischaemic heart disease, hypertension and stroke. Aortic dissection seems to occur with increased frequency. Aim: To describe in more detail aortic dissection as encountered in Turner's syndrome, giving attention to clinical, histological and epidemiological aspects. Materials and methods: Based on a retrospective study, we describe the clinical, karyotypic, and epidemiological aspects of aortic dissection as encountered in cases of Turner's syndrome seen in Denmark and Sweden. Results: The median age at onset of aortic dissection in 18 women was 35 years, ranging from 18 to 61 years. Fourteen of 18 women had a 45,X karyotype, while 2 patients had 45,X/45,XY, and 2 had the 45,X/46,X+r(X) complement, respectively. Echocardiography was performed in 10 of 18 patients before their acute illness, and showed signs of congenital cardiac disease, with either bifoliate aortic valves, dilation of the aortic root, or previous aortic coarctation evident in most patients. In 5 patients evidence of a bifoliate aortic valve was conclusive. Hypertension was present in 5 of 18 patients, while 10 of the patients died from aortic dissection, of so-called type A in 6, type B in 3, while in the final case the origin of dissection could not be determined. Biochemical analysis showed altered ratio between type I and type III collagen. Histology showed cystic medial necrosis in 3 of 7 cases. We estimated an incidence of dissection of 36 per 100,000 Turner's syndrome years, compared with an incidence of 6 per 100,000 in the general population, and a cumulated rate of incidence of 14, 73, 78, and 50 per 100,000 among 0–19, 20–29, 30–39, and 40+ year olds, respectively. Conclusion: Aortic dissection is extremely common in the setting of Turner's syndrome, and occurs early in life. Patients with Turner's syndrome should be offered a protocol for clinical follow-up similar to that provided for patients with Marfan syndrome, and each clinic should embrace a programme for follow-up.
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DOI: 10.1017/S1047951106000928
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Denmark</mods:affiliation><country xml:lang="fr">Danemark</country><wicri:regionArea>Medical department M (Endocrinology and Diabetes) and Medical Research Laboratories, Århus Sygehus, Århus University Hospital</wicri:regionArea></affiliation></author><author><name sortKey="Eilersen Hjerrild, Britta" sort="Eilersen Hjerrild, Britta" uniqKey="Eilersen Hjerrild B" first="Britta" last="Eilersen Hjerrild">Britta Eilersen Hjerrild</name><affiliation wicri:level="1"><mods:affiliation>Medical department M (Endocrinology and Diabetes) and Medical Research Laboratories, Århus Sygehus, Århus University Hospital, Denmark</mods:affiliation><country xml:lang="fr">Danemark</country><wicri:regionArea>Medical department M (Endocrinology and Diabetes) and Medical Research Laboratories, Århus Sygehus, Århus University Hospital</wicri:regionArea></affiliation></author><author><name sortKey="Ledet, Thomas" sort="Ledet, Thomas" uniqKey="Ledet T" first="Thomas" last="Ledet">Thomas Ledet</name><affiliation wicri:level="1"><mods:affiliation>Laboratory of Biochemical Pathology, Århus Sygehus, Århus University Hospital, Denmark</mods:affiliation><country xml:lang="fr">Danemark</country><wicri:regionArea>Laboratory of Biochemical Pathology, Århus Sygehus, Århus University Hospital</wicri:regionArea></affiliation></author><author><name sortKey="Born Djurhuus, Christian" sort="Born Djurhuus, Christian" uniqKey="Born Djurhuus C" first="Christian" last="Born Djurhuus">Christian Born Djurhuus</name><affiliation wicri:level="1"><mods:affiliation>Medical department M (Endocrinology and Diabetes) and Medical Research Laboratories, Århus Sygehus, Århus University Hospital, Denmark</mods:affiliation><country xml:lang="fr">Danemark</country><wicri:regionArea>Medical department M (Endocrinology and Diabetes) and Medical Research Laboratories, Århus Sygehus, Århus University Hospital</wicri:regionArea></affiliation></author><author><name sortKey="Sylven, Lisskulla" sort="Sylven, Lisskulla" uniqKey="Sylven L" first="Lisskulla" last="Sylvén">Lisskulla Sylvén</name><affiliation wicri:level="1"><mods:affiliation>Department of Woman and Child Health, Karolinska University Hospital, Solna, Stockholm, Sweden</mods:affiliation><country xml:lang="fr">Suède</country><wicri:regionArea>Department of Woman and Child Health, Karolinska University Hospital, Solna, Stockholm</wicri:regionArea></affiliation></author><author><name sortKey="Baandrup, Ulrik" sort="Baandrup, Ulrik" uniqKey="Baandrup U" first="Ulrik" last="Baandrup">Ulrik Baandrup</name><affiliation wicri:level="1"><mods:affiliation>Department of Pathology, Århus University Hospital, Århus Sygehus, Denmark</mods:affiliation><country xml:lang="fr">Danemark</country><wicri:regionArea>Department of Pathology, Århus University Hospital, Århus Sygehus</wicri:regionArea></affiliation></author><author><name sortKey=" Stergaard Kristensen, Bent" sort=" Stergaard Kristensen, Bent" uniqKey=" Stergaard Kristensen B" first="Bent" last=" Stergaard Kristensen">Bent Stergaard Kristensen</name><affiliation wicri:level="1"><mods:affiliation>Department of Cardiology, Skejby Hospital, Århus University Hospital, Denmark</mods:affiliation><country xml:lang="fr">Danemark</country><wicri:regionArea>Department of Cardiology, Skejby Hospital, Århus University Hospital</wicri:regionArea></affiliation></author><author><name sortKey="Sandahl Christiansen, Jens" sort="Sandahl Christiansen, Jens" uniqKey="Sandahl Christiansen J" first="Jens" last="Sandahl Christiansen">Jens Sandahl Christiansen</name><affiliation wicri:level="1"><mods:affiliation>Medical department M (Endocrinology and Diabetes) and Medical Research Laboratories, Århus Sygehus, Århus University Hospital, Denmark</mods:affiliation><country xml:lang="fr">Danemark</country><wicri:regionArea>Medical department M (Endocrinology and Diabetes) and Medical Research Laboratories, Århus Sygehus, Århus University Hospital</wicri:regionArea></affiliation></author></analytic><monogr></monogr><series><title level="j">Cardiology in the Young</title><idno type="ISSN">1047-9511</idno><idno type="eISSN">1467-1107</idno><imprint><publisher>Cambridge University Press</publisher><pubPlace>Cambridge, UK</pubPlace><date type="published" when="2006-10">2006-10</date><biblScope unit="volume">16</biblScope><biblScope unit="issue">5</biblScope><biblScope unit="page" from="430">430</biblScope><biblScope unit="page" to="436">436</biblScope></imprint><idno type="ISSN">1047-9511</idno></series></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">1047-9511</idno></seriesStmt></fileDesc><profileDesc><textClass></textClass><langUsage><language ident="en">en</language></langUsage></profileDesc></teiHeader><front><div type="abstract">Background: Women with Turner's syndrome have an increased risk of congenital cardiac malformations, ischaemic heart disease, hypertension and stroke. Aortic dissection seems to occur with increased frequency. Aim: To describe in more detail aortic dissection as encountered in Turner's syndrome, giving attention to clinical, histological and epidemiological aspects. Materials and methods: Based on a retrospective study, we describe the clinical, karyotypic, and epidemiological aspects of aortic dissection as encountered in cases of Turner's syndrome seen in Denmark and Sweden. Results: The median age at onset of aortic dissection in 18 women was 35 years, ranging from 18 to 61 years. Fourteen of 18 women had a 45,X karyotype, while 2 patients had 45,X/45,XY, and 2 had the 45,X/46,X+r(X) complement, respectively. Echocardiography was performed in 10 of 18 patients before their acute illness, and showed signs of congenital cardiac disease, with either bifoliate aortic valves, dilation of the aortic root, or previous aortic coarctation evident in most patients. In 5 patients evidence of a bifoliate aortic valve was conclusive. Hypertension was present in 5 of 18 patients, while 10 of the patients died from aortic dissection, of so-called type A in 6, type B in 3, while in the final case the origin of dissection could not be determined. Biochemical analysis showed altered ratio between type I and type III collagen. Histology showed cystic medial necrosis in 3 of 7 cases. We estimated an incidence of dissection of 36 per 100,000 Turner's syndrome years, compared with an incidence of 6 per 100,000 in the general population, and a cumulated rate of incidence of 14, 73, 78, and 50 per 100,000 among 0–19, 20–29, 30–39, and 40+ year olds, respectively. Conclusion: Aortic dissection is extremely common in the setting of Turner's syndrome, and occurs early in life. Patients with Turner's syndrome should be offered a protocol for clinical follow-up similar to that provided for patients with Marfan syndrome, and each clinic should embrace a programme for follow-up.</div></front></TEI></record>Pour manipuler ce document sous Unix (Dilib)
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