Discussion Author(s): Primary author: Jason Messinger
Contributing author: Eric Jones
Tetralogy of Fallot (TOF) is a rare condition, but is the most common cyanotic congenital heart malformations. TOF makes up about 10% of all congenital heart diseases and has an incidence of around 1 in 3000 live births. There is no gender preference.
TOF has four defining characteristics: a large ventricular septal defect, pulmonary stenosis, right ventricular hypertrophy , and an overriding aorta.
The signs and symptoms of TOF vary, but most commonly patients will present with cyanosis and failure to thrive. TOF can be diagnosed prenatally with obstetrical ultrasound. Multiple imaging modalities are used for postnatal diagnosis.
The underlying cause for TOF, and many congenital heart defects, is not known, however, there is a strong association with TOF and genetic syndromes like trisomy 21 and DiGeorge syndrome. Other genetic associations include deletions of 22q11.2 and 13q13.1â€“q13.2 deletion.
The general course of treatment for TOF is corrective surgery because of the severe deformities of the heart. However, there is debate as to how surgery should be performed. A staged repair is more traditional but there are now proponents of early total repair.
The staged repair technique has the initial step of palliation with systemic arterial to pulmonary shunt before total TOF repair. The staged approach has the benefits of allowing the the infundibulum and pulmonary valve to grow, reducing morbidity and mortality on the operating table, and reducing neurological sequelae.
With early total repair the functional status of the right ventricle is protected and right ventricular hypertrophy can be avoided. There is also reduced incidence of arrythmias as well as reduction in cyanosis and pulmonary artery shunting. The risks of early repair involve failure of the reconstructed infundibulum and pulmonary.
With surgical intervention 40 year survival rate can be 75%.
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