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Murine Fetal Echocardiography
08:04

Murine Fetal Echocardiography

Published on: February 15, 2013

The clinical course and echocardiographic features of Marfan's syndrome in childhood

T Geva1, J Hegesh, M Frand

  • 1Department of Pediatrics C, Chaim Sheba Medical Center, Tel-Hashomer, Israel.

American Journal of Diseases of Children (1960)
|November 1, 1987
PubMed

Insights

Childhood Marfan

Area of Science:

  • Cardiology
  • Genetics
  • Pediatrics

Background:

  • Marfan's syndrome is a genetic disorder affecting connective tissue.
  • Early diagnosis and understanding of cardiovascular manifestations are crucial in pediatric patients.

Purpose of the Study:

  • To evaluate the clinical and echocardiographic features of Marfan's syndrome in infants and children.
  • To identify risk factors for progressive cardiovascular involvement and mortality.

Main Methods:

  • Retrospective analysis of 25 pediatric patients diagnosed with Marfan's syndrome.
  • Clinical examination and echocardiography were used to assess cardiovascular status.
  • Follow-up evaluation of disease progression and patient outcomes.

Main Results:

  • All patients exhibited mitral valve prolapse; 80% had aortic root dilatation.
  • Significant rates of mitral regurgitation (44%) and aortic regurgitation (28%) were observed.
  • Progressive cardiovascular disease occurred in 22% of patients, with higher incidence in those diagnosed before age 10 and sporadic cases.

Conclusions:

  • Cardiovascular involvement is a major cause of morbidity and mortality in childhood Marfan's syndrome.
  • Early diagnosis (before age 10) and sporadic cases indicate a higher-risk group requiring close monitoring.

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