Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Experiment Videos

Myocardial disarray in Noonan syndrome.

M Burch1, J M Mann, M Sharland

  • 1Department of Clinical Genetics, St George's Hospital Medical School, London.

British Heart Journal
|December 1, 1992
PubMed
Summary

Children with Noonan syndrome exhibit significant left ventricular myocyte disarray, similar to hypertrophic cardiomyopathy. This finding suggests a potential shared pathological mechanism between these conditions.

Related Concept Videos

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

Myocyte nuclear area as a measure of left ventricular hypertrophy in transplant patients.

Cardiovascular pathology : the official journal of the Society for Cardiovascular Pathology·2015
Same author

Congenital and genetic disorders in the Sultanate of Oman. First attempt to assess healthcare needs.

Journal of community genetics·2014
Same author

Mutations in FAM20C also identified in non-lethal osteosclerotic bone dysplasia.

Clinical genetics·2009
Same author

Brain penetration effects of microelectrodes and DBS leads in STN or GPi.

Journal of neurology, neurosurgery, and psychiatry·2009
Same author

A mutation in NFkappaB interacting protein 1 causes cardiomyopathy and woolly haircoat syndrome of Poll Hereford cattle.

Animal genetics·2008
Same author

Homozygous mutation of desmocollin-2 in arrhythmogenic right ventricular cardiomyopathy with mild palmoplantar keratoderma and woolly hair.

Cardiology·2008

Area of Science:

  • Cardiovascular Pathology
  • Pediatric Cardiology
  • Genetic Syndromes

Background:

  • Noonan syndrome is a genetic disorder often associated with cardiac abnormalities, including left ventricular hypertrophy.
  • Idiopathic hypertrophic cardiomyopathy is characterized by unexplained thickening of the heart muscle.

Observation:

  • Histological analysis of six pediatric hearts with Noonan syndrome and ventricular hypertrophy was compared to age- and sex-matched controls.
  • Myocyte disarray was quantified using image analysis under light microscopy.

Findings:

  • Patients with Noonan syndrome showed significantly greater myocyte disarray in both the ventricular septum (24%) and free wall (22.2%) compared to controls (3.8% and 2.4%, respectively).
  • The difference in disarray extent was statistically significant (p < 0.0005 for septum, p < 0.005 for free wall).

Related Experiment Videos

Implications:

  • The histopathological findings in Noonan syndrome-associated left ventricular hypertrophy resemble those of idiopathic hypertrophic cardiomyopathy.
  • Further research is needed to determine if these conditions share an underlying genetic etiology.