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Shone's Anomaly - A Case Report.

M Palaniappan1, B Soundravalli, R Sundar

  • 1G. Kuppusamy Naidu Memorial Hospital, Pappanaikanpalyalam,Coimbatore, India. drpal@eth.net.

Annals of Cardiac Anaesthesia
|September 14, 2007
PubMed
Summary
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Shone's anomaly, a rare congenital heart defect, involves multiple left heart obstructions. Successful surgical repair is possible, even in adults, with a two-stage procedure in a single sitting.

Area of Science:

  • Cardiology
  • Congenital Heart Disease
  • Cardiac Surgery

Background:

  • Shone's anomaly is a rare congenital cardiac malformation characterized by multiple left heart obstructions.
  • These obstructions include supravalvular mitral ring, parachute mitral valve, subaortic stenosis, and coarctation of the aorta.
  • While presenting a spectrum of abnormalities, most patients can achieve satisfactory outcomes.

Purpose of the Study:

  • To report a case of Shone's anomaly presenting in the third decade of life.
  • To highlight the successful surgical management of a complex case.
  • To emphasize the feasibility of multi-stage repair in a single sitting.

Main Methods:

  • Surgical correction of multiple left heart obstructions.
  • A two-stage operative approach was employed.

Related Experiment Videos

  • The procedure was completed successfully in a single sitting.

    • Main Results:

    • The patient, presenting in the third decade, underwent successful surgical correction.
    • The two-stage procedure in a single sitting yielded a satisfactory outcome.
    • Operative mortality and outcome are influenced by mitral valve disease severity and the number of procedures.

    Conclusions:

    • Shone's anomaly, despite its complexity, can be managed successfully with surgical intervention.
    • A staged, single-sitting repair is a viable option for adult patients.
    • Early and comprehensive surgical management is crucial for optimal outcomes in Shone's anomaly.