Abstract

Tetralogy of Fallot (ToF) is the most common cyanotic congenital heart disease, frequently diagnosed prenatally. To enhance understanding, we reviewed 18 years of our institutional outcomes for patients with classic ToF. There were 410 patients of whom 233/410 (56.8%) were diagnosed prenatally. A genetic abnormality was diagnosed in 38/410 (9.3%), most commonly 22q11.2 microdeletion (43.6%). There were 10/410 (2.4%) deaths in the neonatal period prior to any cardiac intervention. Prior to complete ToF repair, 66/400 (16.5%) of patients required cardiac procedures, in 15 in the first week after birth. Babies with an antenatal diagnosis were more likely to require at least one procedure before complete ToF repair than those with a postnatal diagnosis (p = 0.039). There was a trend to shorter median length of stay in patients with RVOT stent (median 5.4 days) or duct stenting (median 4.2 days) versus BTT shunt (median 8 days). Complete ToF repair was undertaken in 396/410 at a median age of 6 months (IQR: 4.0–8.0 months), with a 30-day postoperative survival rate of 99.5%. The actuarial survival for the whole group (n = 410) of 410 patients was 98.5% at 30 days, 96.2% at 1 year, and 95.2% at 3 years of age. Following complete surgical repair, reintervention was required in 25% of cases: 3.3% at 30 days, 10.6% at 1 year, and 19.2% at 5 years. The freedom from reintervention was 89.4% after 1 year and 80.8% after 5 years. There were no deaths beyond 2.5 years post-repair. In conclusion, ToF can be repaired with a low procedural mortality rate and promising long-term survival outcomes, but there may be pre-procedural deaths and there is a likelihood of requiring reinterventions during follow-up.