8th March 2024

Management

Most patients with TOF undergo complete repair by 1 year of age. A small number of neonates require initial palliative repair in the form of shunts or stents due to severe RVOT obstruction. Surgical repair aims to relieve RVOT obstruction, separate the systemic and pulmonary circulations by closure of the VSD, preserve RV function and minimise post-surgery valvular complications. 

Ventricular arrhythmias are an important cause of mortality in TOF patients after surgery. Secondary prevention with an implantable cardioverter defibrillator (ICD) is appropriate in patients with documented sustained VT or those who have survived an episode of resuscitated cardiac arrest. The indication for a primary prevention ICD is individualised.

Follow up

The long-term prognosis is generally good in repaired TOF, with studies reporting survival rates exceeding 94% at 25 years.

Routine follow-up with a congenital cardiologist is required to monitor post-operative complications. Echocardiography and ECG are performed yearly until the age of 10 and every two years thereafter. Other investigations, such as an exercise stress test, CMR and Holter monitor, are performed roughly every 3-4 years but sooner if the need arises.

Antibiotic prophylaxis is recommended in the first 6 months after TOF surgery or in those with residual defects after intervention.  

Pregnancy is not recommended in women with unrepaired TOF. The decision for pregnancy after surgery should be individualised based on the careful evaluation made by the congenital cardiac team in order to adequately risk-stratify the patient.