treatment
Last reviewed 10/2020
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.
management of hypercyanotic attacks