Cardiovascular findings, and clinical course, in patients with Williams syndrome

@article{Bruno2003CardiovascularFA,
  title={Cardiovascular findings, and clinical course, in patients with Williams syndrome},
  author={E. Bruno and N. Rossi and {\'O}. Th{\"u}er and R. C{\'o}rdoba and L. Alday},
  journal={Cardiology in the Young},
  year={2003},
  volume={13},
  pages={532 - 536}
}
Aims: We investigated the incidence and outcome of cardiac malformations in 53 patients with Williams syndrome. Methods and results:The mean age, and period of follow-up, were 3.6 and 5.3 years, with standard deviations of 4.0 and 5.6 years, respectively. Of the patients, 45 (85%) had cardiovascular anomalies, often combined. Males presented earlier than females, at the ages of 2.1 years, with standard deviation of 2.8 years, as opposed to 4.5 years, with standard deviation of 4.2 years (p < 0… Expand
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TLDR
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Pulmonary Artery Diverticulum: An Angiographic Marker for Williams Syndrome
TLDR
The angiographic appearance of a diverticulum as an extension of the main pulmonary artery is a consistent finding in patients with Williams syndrome, and can be considered as a pathognomonic feature of Williams syndrome. Expand
Diffuse hypoplasia of the aortic arch and isthmus in a patient with Williams syndrome.
TLDR
The case of a 16-month-old female with Williams syndrome and diffuse hypoplasia of the aortic arch and isthmus, and concomitant pulmonary stenosis and a ventricular septal defect is described. Expand
Cardiovascular disease in Williams syndrome.
TLDR
Hemizygosity of the ELN gene coding for elastin has been demonstrated to be responsible for the vascular pathology in WS and the remaining 25 to 27 deleted genes contribute to the phenotypic findings in patients with WS and have recently been reviewed in detail elsewhere. Expand
Cardiovascular disease in Williams syndrome
  • R. Collins
  • Medicine
  • Current opinion in pediatrics
  • 2018
TLDR
Advances in surgical techniques, medical therapeutic options, and periprocedural management hold promise for significant improvements in the cardiovascular outcomes of patients with Williams syndrome. Expand
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