{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,23]],"date-time":"2026-08-23T18:18:00Z","timestamp":1787509080819,"version":"build-2736575974"},"reference-count":47,"publisher":"American Academy of Pediatrics (AAP)","issue":"1","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[1998,7,1]]},"abstract":"<jats:p>Objective.\u2003Although cardiovascular malformations (CVMs) are well-recognized congenital anomalies in Turner syndrome, aortic dilation and dissection are less common and less familiar. Most of the relevant literature is limited to single cases reports or small series. We sought to increase the information available about the frequency and characteristics of aortic dilation in patients with Turner syndrome.<\/jats:p>\n                  <jats:p>Design.\u2003A 1-page survey of cardiac abnormalities, including aortic dilation, was mailed to \u223c1000 (1040 verified) members of the Turner Syndrome Society as an enclosure in the June 1997 newsletter. We also conducted a literature review.<\/jats:p>\n                  <jats:p>Participants.\u2003A total of 245 patients or families of patient members of the Turner Syndrome Society responded to the survey (\u223c24% response rate).<\/jats:p>\n                  <jats:p>Results.\u2003A CVM was reported in 120 of 232 (52%) respondents to this questionnaire. Obstructive lesions of the left side of the heart predominated and included bicuspid aortic valve (38%) and coarctation (41%).<\/jats:p>\n                  <jats:p>Aortic dilation was reported in at least 15 of 237 respondents (6.3%; 95% confidence interval: 3.6%-10.3%); 2 of 15 (13%) had dissection. Twelve of 15 (80%) patients had an associated risk factor for aortic dilation such as a CVM or hypertension. The 3 (20%) patients who did not have a CVM or hypertension were all younger than 21 years. In the entire group with aortic dilation, 10 of 15 (67%) patients were younger than 21 years. All patients with aortic dilation had involvement of the ascending aorta, and 2 had additional involvement of the descending aorta distal to a repaired coarctation.<\/jats:p>\n                  <jats:p>An update of the literature revealed 68 patients with aortic dilation, dissection, or rupture. An associated CVM or hypertension was reported in 53 of 59 (90%) informative patients. At least 6 (10%) had no predisposing risk factor (information was inadequate for 9 of 68 patients). The following patterns of aortic involvement were identified: ascending \u00b1 descending aorta with coarctation (14); ascending \u00b1 descending aorta without coarctation (39); descending aorta with coarctation (3); descending thoracic or abdominal aorta without coarctation (4); and unspecified (8). Dissection or rupture was reported in 42\/68 (62%). Two reported patients died suddenly from aortic dissection in the third trimester of assisted pregnancy. At least 20 (29%) patients were younger than 21 years. One of the 6 (17%) patients with isolated aortic dilation was in this younger group.<\/jats:p>\n                  <jats:p>Conclusions.\u2003More information is needed about the frequency and natural history of aortic dilation in Turner syndrome. This work contributes new patient data and increases the literature review. Despite the well-recognized limitations of self-reporting, this survey detected aortic dilation with or without dissection in \u223c6% of patients with Turner syndrome. Although rare, this is a potentially catastrophic occurrence, warranting greater awareness among health professionals.<\/jats:p>\n                  <jats:p>In this study and the literature, the vast majority of patients with aortic dilation have an associated risk factor such as a CVM, typically bicuspid aortic valve or coarctation, or systemic hypertension. These patients represent a higher risk group that should be followed appropriately, usually under the direction of a cardiologist. Patients undergoing assisted pregnancy also should be evaluated closely.<\/jats:p>\n                  <jats:p>It is generally accepted that at the time of diagnosis of Turner syndrome, all patients should have a complete cardiology evaluation including echocardiography. The small number of patients with aortic dilation without a CVM, who would not be under the long-term care of a cardiologist, makes it prudent to screen all patients with Turner syndrome for this potentially lethal abnormality. The specific timing for this screening is controversial. Our recommendations for prospective imaging do not represent a rigid standard of care.<\/jats:p>","DOI":"10.1542\/peds.102.1.e12","type":"journal-article","created":{"date-parts":[[2004,8,13]],"date-time":"2004-08-13T19:44:32Z","timestamp":1092426272000},"page":"e12-e12","source":"Crossref","is-referenced-by-count":169,"title":["Further Delineation of Aortic Dilation, Dissection, and Rupture in Patients With Turner Syndrome"],"prefix":"10.1542","volume":"102","author":[{"given":"Angela E.","family":"Lin","sequence":"first","affiliation":[{"name":"From the Genetics and Teratology Unit, Massachusetts General Hospital, Boston, Massachusetts;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Barbara","family":"Lippe","sequence":"additional","affiliation":[{"name":"Peptide Hormones Group, Pharmacia & Upjohn and the Department of Pediatrics, UCLA School of Medicine, Los Angeles, California; and the"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ron G.","family":"Rosenfeld","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Oregon Health Sciences University, Doernbecher Children's Hospital, Portland, Oregon."}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"417","published-online":{"date-parts":[[1998,7,1]]},"reference":[{"key":"2021121010174024700_B1","doi-asserted-by":"crossref","first-page":"433","DOI":"10.1136\/adc.71.5.433","article-title":"Prevalence of cardiovascular malformations and association with karyotypes in Turner's syndrome.","volume":"71","author":"Gotzsche","year":"1994","journal-title":"Arch Dis Child."},{"key":"2021121010174024700_B2"},{"key":"2021121010174024700_B3","doi-asserted-by":"crossref","first-page":"47","DOI":"10.1016\/S0022-3476(83)80284-4","article-title":"Echocardiography reveals a high incidence of bicuspid aortic valve in Turner syndrome.","volume":"102","author":"Miller","year":"1983","journal-title":"J Pediatr"},{"key":"2021121010174024700_B4","doi-asserted-by":"crossref","first-page":"820","DOI":"10.1016\/S0022-3476(86)80700-4","article-title":"Aortic dilation, dissection, and rupture in patients with Turner syndrome.","volume":"109","author":"Lin","year":"1986","journal-title":"J Pediatr"},{"key":"2021121010174024700_B5","first-page":"411","article-title":"Zum kingking des aortenbogens beim Turner-syndrom.","volume":"23","author":"Wendel","year":"1968","journal-title":"Z Ger Inn Med."},{"key":"2021121010174024700_B6","first-page":"87","article-title":"Dissection aortique et syndrome de Turner.","volume":"5","author":"Guerin","year":"1974","journal-title":"Coeur."},{"key":"2021121010174024700_B7","first-page":"771","article-title":"Sindrome de Turner y aneurisma disecante de la aorta.","volume":"44","author":"Miller","year":"1974","journal-title":"Arch Inst Cardiol 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Case report and review of the literature.","volume":"96","author":"Swapp","year":"1989","journal-title":"Br J Obstet Gynaecol."},{"key":"2021121010174024700_B44","doi-asserted-by":"crossref","first-page":"81","DOI":"10.1159\/000293307","article-title":"Turner's syndrome: review of the literature with reference to a successful pregnancy outcome.","volume":"29","author":"Kaneko","year":"1990","journal-title":"Gynecol Obstet Invest."},{"key":"2021121010174024700_B45","doi-asserted-by":"crossref","first-page":"587","DOI":"10.1093\/oxfordjournals.humrep.a019445","article-title":"The risk of aortic dissection in women with Turner syndrome.","volume":"11","author":"Birdsall","year":"1996","journal-title":"Hum Reprod."},{"key":"2021121010174024700_B46","doi-asserted-by":"crossref","first-page":"427","DOI":"10.1016\/S0022-5223(19)37770-0","article-title":"Coarctation resection in children with Turner's syndrome. A note of caution.","volume":"80","author":"Ravelo","year":"1980","journal-title":"J Thorac Cardiovasc Surg."},{"key":"2021121010174024700_B47","doi-asserted-by":"crossref","first-page":"175","DOI":"10.1007\/BF02427041","article-title":"Repair of coarctation of the aorta in children with Turner syndrome.","volume":"5","author":"Brandt","year":"1985","journal-title":"Pediatr Cardiol."}],"container-title":["Pediatrics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/publications.aap.org\/pediatrics\/article-pdf\/102\/1\/e12\/837772\/e12.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/publications.aap.org\/pediatrics\/article-pdf\/102\/1\/e12\/837772\/e12.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,1,14]],"date-time":"2024-01-14T00:06:15Z","timestamp":1705190775000},"score":1,"resource":{"primary":{"URL":"https:\/\/publications.aap.org\/pediatrics\/article\/102\/1\/e12\/65449\/Further-Delineation-of-Aortic-Dilation-Dissection"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1998,7,1]]},"references-count":47,"journal-issue":{"issue":"1","published-online":{"date-parts":[[1998,7,1]]},"published-print":{"date-parts":[[1998,7,1]]}},"URL":"https:\/\/doi.org\/10.1542\/peds.102.1.e12","relation":{},"ISSN":["1098-4275","0031-4005"],"issn-type":[{"value":"1098-4275","type":"electronic"},{"value":"0031-4005","type":"print"}],"subject":[],"published":{"date-parts":[[1998,7,1]]}}}