Further Delineation of Aortic Dilation, Dissection, and Rupture in Patients With Turner Syndrome

Although 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 availa...

Full description

Saved in:
Bibliographic Details
Published in:Pediatrics (Evanston) 1998-07, Vol.102 (1), p.e12-e12
Main Authors: Lin, Angela E, Lippe, Barbara, Rosenfeld, Ron G
Format: Article
Language:English
Subjects:
Adolescent
Adult
Aged
Aneurysm, Dissecting - epidemiology
Aneurysm, Dissecting - etiology
Aneurysm, Dissecting - therapy
Aorta, Thoracic
Aortic Rupture - epidemiology
Aortic Rupture - etiology
Aortic Rupture - therapy
Cardiovascular Diseases - epidemiology
Cardiovascular Diseases - etiology
Child
Confidence Intervals
Female
Heart Defects, Congenital - epidemiology
Humans
Incidence
Male
Middle Aged
Pediatrics
Population Surveillance
Pregnancy
Pregnancy Complications, Cardiovascular - epidemiology
Pregnancy Complications, Cardiovascular - etiology
Surveys and Questionnaires
Treatment Outcome
Turner Syndrome - complications
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Although 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. A 1-page survey of cardiac abnormalities, including aortic dilation, was mailed to approximately 1000 (1040 verified) members of the Turner Syndrome Society as an enclosure in the June 1997 newsletter. We also conducted a literature review. A total of 245 patients or families of patient members of the Turner Syndrome Society responded to the survey ( approximately 24% response rate). A 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%). 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. 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 +/- descending aorta with coarctation (14); ascending +/- 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. More information is needed about the frequ
ISSN:0031-4005
1098-4275
DOI:10.1542/peds.102.1.e12