Imaging of cardiovascular risk in patients with Turner's syndrome

Marin, Aleksander; Weir‐McCall, Jonathan R.; Webb, David J.; Beek, Edwin Jacques Rudolph van; Mirsadraee, Saeed

doi:10.1016/j.crad.2015.03.009

Cited by 30 publications

(12 citation statements)

References 82 publications

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“…CHD is present is 20%–50% of patients with TS. The most frequent CHD are BAV (11%–50%), CoA (11%–35%) followed by HLHS, PAPVR, and elongation of transverse aortic arch . Atrial and ventricular septal (1%–2%) defects are usually small and not frequent in patients with TS, and if present, they do not usually require surgical repair .…”

Section: Turner Syndromementioning

confidence: 99%

“…The most frequent CHD are BAV (11%-50%), 27,33,[36][37][38] CoA (11%-35%) [25][26][27]33,36,37,39 followed by HLHS, PAPVR, and elongation of transverse aortic arch. 23,40 Atrial and ventricular septal (1%-2%) 30 defects are usually small and not frequent in patients with TS, and if present, they do not usually require surgical repair. 23 There are other less commonly described types of CHD lesions in this population, 23 most of them in case reports, such as hypertrophic cardiomyopathy, 41 tetralogy of Fallot, 42,43 AVSD, 44 patent arterial duct, and aortic or pulmonary artery valve stenosis, among others.…”

Section: Spectrum Of Chd In Patients With Tsmentioning

confidence: 99%

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Congenital heart disease and cardiac procedural outcomes in patients with trisomy 21 and Turner syndrome

Morales-Demori

2017

Congenital Heart Disease

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Congenital heart disease (CHD) is present in approximately 50% of patients with trisomy 21 (T21) and Turner syndrome (TS). According to the American Academy of Pediatrics, every patient with these genetic disorders should have a postnatal echocardiogram. T21 is usually associated with atrioventricular (30%-60%), atrial (16%-21%), or ventricular septal defects (14%-27%). TS is usually associated with left-sided heart disease. However, the spectrum of CHD in these genetic disorders is wider than those mentioned lesions. More cardiac surgical procedures are offered to these patients and that has influenced positively their life expectancy for some CHD conditions. Single ventricular anatomy is associated with high mortality in these genetic disorders (49% in T21 and 83%-91% in TS). The goal of this article is to describe the spectrum of CHD, screening guidelines, and cardiac surgical outcomes in patients with T21 or TS with CHD.

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Section: Turner Syndromementioning

confidence: 99%

Section: Spectrum Of Chd In Patients With Tsmentioning

confidence: 99%

Congenital heart disease and cardiac procedural outcomes in patients with trisomy 21 and Turner syndrome

Morales-Demori

2017

Congenital Heart Disease

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“…It exclusively affects females and has a frequency of approximately 40/100 000. Moreover, the cardiovascular mortality rate for these patients is one to three times higher [58]. Apart from aortic isthmus stenosis (8 %), bicuspid aortic valve (10 -25 %) is the most common cardiovascular malformation [6].…”

Section: Turner Syndromementioning

confidence: 99%

Current and Emerging Imaging Techniques in Patients with Genetic Aortic Syndromes

Weinrich

Lenz

Girdauskas³

et al. 2019

Fortschr Röntgenstr

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Background Patients with genetic aortic syndromes such as Marfan or Loeys-Dietz syndrome have a decreased life expectancy due to the risk of aortic dissection and rupture. Imaging plays an important role in the acute setting but also in the initial diagnosis and image-based monitoring. In this article, we provide an overview of the most common genetic aortic syndromes and recommended imaging strategies. Furthermore, we highlight modern imaging methods allowing for the quantification of hemodynamic changes in aortic disease. Method This is a narrative review article on genetic aortic syndromes and recommended imaging strategies, where we take into account expert opinions and standard-of-care practices from our own center. Results and Conclusion Radiological imaging plays a key role in the initial diagnosis and surveillance of patients with genetic aortic syndromes. Radiologists contribute significantly to the multi-disciplinary setting of genetic aortic syndromes with knowledge of special features and recommended imaging methods. Accurate measurement of the aorta is crucial, particularly in terms of diameter-based surgical treatment algorithms. Modern imaging methods like 4D-flow MRI and pulse wave velocity have a potential to further improve individualized risk stratification in patients with genetic aortic syndromes. Key points: Citation Format

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“…Es betrifft aus-schließlich das weibliche Geschlecht und hat eine Prävalenz von etwa 40 : 100 000. Darüber hinaus haben die Betroffenen eine bis zu 3-fach erhöhte kardiovaskuläre Mortalitätsrate [58]. Neben einer Aortenisthmusstenose (8 %) ist die bikuspide Aortenklappe mit 10 -25 % die häufigste kardiovaskuläre Malformation [6].…”

Section: Turner-syndromunclassified