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Major vascular anomalies in Turner syndrome: prevalence and magnetic resonance angiographic features.
- Source :
-
Circulation [Circulation] 2004 Sep 21; Vol. 110 (12), pp. 1694-700. Date of Electronic Publication: 2004 Sep 07. - Publication Year :
- 2004
-
Abstract
- Background: Turner syndrome (TS) is associated with aortic coarctation and dissection; hence, echocardiographic evaluation of all patients is currently recommended. X-ray angiography in clinically symptomatic patients has suggested a range of other vascular anomalies, but the true prevalence of such lesions in TS is unknown. To better understand the prevalence and pathogenesis of cardiovascular defects in TS, we prospectively evaluated a group of asymptomatic adult volunteers with TS using magnetic resonance (MR) angiography.<br />Methods and Results: A total of 85 adults with TS and 27 normal female adult volunteers underwent gadolinium-enhanced 3D MR angiography. A high prevalence of aortic anomalies was seen in women with TS, including elongation of the transverse arch (49%), aortic coarctation (12%), and aberrant right subclavian artery (8%). Venous anomalies were also prominent, including persistent left superior vena cava (13%) and partial anomalous pulmonary venous return (13%). None of these anomalies were found in healthy female controls. The constellation of elongation of the transverse arch, aortic coarctation, and persistent left superior vena cava was significantly associated with women with TS. Neck webbing and increased thoracic anterior-to-posterior dimension diameters were strong predictors for arterial and venous anomalies.<br />Conclusions: Thoracic vascular anomalies are common in TS, occurring in approximately 50% of a group not preselected for cardiovascular disease. The highly significant association between neck webbing, increased chest diameter, and these vascular anomalies suggests that in utero, centrally localized lymphatic obstruction may contribute to these cardiovascular deformities in TS. Improved recognition of these often-undetected vascular lesions may be important for identification of patients in need of closer cardiovascular monitoring.
- Subjects :
- Adult
Anthropometry
Aortic Arch Syndromes diagnosis
Aortic Arch Syndromes epidemiology
Aortic Arch Syndromes genetics
Aortic Coarctation diagnosis
Aortic Coarctation epidemiology
Aortic Coarctation genetics
Blood Pressure
Cardiovascular Abnormalities diagnosis
Cardiovascular Abnormalities epidemiology
Female
Humans
Imaging, Three-Dimensional
Middle Aged
Neck abnormalities
Prevalence
Pulmonary Veins abnormalities
Single-Blind Method
Subclavian Artery abnormalities
Thorax pathology
Turner Syndrome epidemiology
Vena Cava, Superior abnormalities
Cardiovascular Abnormalities genetics
Magnetic Resonance Imaging
Turner Syndrome pathology
Subjects
Details
- Language :
- English
- ISSN :
- 1524-4539
- Volume :
- 110
- Issue :
- 12
- Database :
- MEDLINE
- Journal :
- Circulation
- Publication Type :
- Academic Journal
- Accession number :
- 15353492
- Full Text :
- https://doi.org/10.1161/01.CIR.0000142290.35842.B0