Study note

Surgical management of pulmonary stenosis with a ventricular septal defect

THE CONTEXT
Management includes definitive correction when feasible and shunt palliation when pulmonary artery hypoplasia prevents repair.

Definitive correction and palliation

FeatureDetails
Definitive correction
surgical treatment relieves pulmonary stenosis and closes the ventricular septal defect.
Timing
primary surgical correction may be undertaken before 5 years of age.
Indication for palliation
pulmonary arteries that are too hypoplastic for surgical repair may require a Blalock–Taussig shunt.
Shunt anatomy
an anastomosis is created between the pulmonary artery and subclavian artery.
Shunt benefit
increased pulmonary blood flow promotes pulmonary artery development and may facilitate later definitive correction.

Prognosis and surveillance

FeatureDetails
Prognosis
the outlook after total correction is good, especially when surgery is performed in childhood.
Follow-up
surveillance should identify residual shunting, recurrent pulmonary stenosis and arrhythmias.
Adult management
an implantable defibrillator is sometimes recommended.
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