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Treatment

Last reviewed dd mmm yyyy. Last edited dd mmm yyyy

Authoring team

Treatment is essentially surgical although the paroxysmal hypercyanotic attacks must be managed appropriately.

Surgery is often in two stages with a palliative procedure to improve symptoms followed later by a total repair:

  • palliative surgery involves creating a shunt between the aorta and pulmonary artery so that sufficient blood can enter the lungs. In a Blalock operation the subclavian artery is joined to the pulmonary artery and in a Waterston operation the ascending aorta is joined to the right pulmonary artery. Alternatively pulmonary valvotomy and infundibular resection may give relief in the Brock operation

  • total repair is undertaken as the child approaches school age

  • the use of an implantable cardioverter defibrillator should be considered following repair (1)

Reference:

  • 1) NICE (September 2000). Implantable cardioverter defibrillators for arrhythmias.

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