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Aarskog syndrome with aortic root dilatation and sub-valvular aortic stenosis: surgical management

S Mahmoud Nouraei1, Asif Hasan, Milind P Chaudhari

  • 1Regional Cardiothoracic Centre, Freeman Hospital, Newcastle upon Tyne, NE7 7DN, UK.

Interactive Cardiovascular and Thoracic Surgery
|August 3, 2007
PubMed

Insights

Aarskog syndrome increases the risk of congenital heart disease (CHD). This case highlights successful surgical management of aortic root dilatation and stenosis in an affected child, emphasizing the need for cardiac surveillance.

Area of Science:

  • Genetics and developmental biology
  • Pediatric cardiology
  • Syndromology

Background:

  • Aarskog syndrome is a genetic disorder characterized by craniofacial anomalies, genital malformations, and short stature.
  • Children with Aarskog syndrome have a higher prevalence of congenital heart disease (CHD) compared to the general population.

Purpose of the Study:

  • To report a case of progressive aortic root dilatation and sub-valvular aortic stenosis in a child with Aarskog syndrome.
  • To highlight the successful surgical management of this cardiovascular complication.
  • To emphasize the importance of cardiac surveillance in Aarskog syndrome.

Main Methods:

  • Case report detailing clinical presentation and management.
  • Surgical intervention involving aortic root and valve replacement.

Main Results:

  • The patient with Aarskog syndrome presented with progressive aortic root dilatation and sub-valvular aortic stenosis.
  • Successful surgical management was achieved through aortic root and valve replacement.

Conclusions:

  • Aarskog syndrome is associated with significant cardiovascular risks, including aortic abnormalities.
  • Early and regular cardiac surveillance is crucial for children diagnosed with Aarskog syndrome to detect and manage potential CHD.
  • Surgical intervention can be effective for managing severe aortic complications in this population.

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