Tetralogy of Fallot

Tetralogy of Fallot



Michael E. Mcconnell, MD



Basic Information



Definition


Tetralogy of Fallot (TOF) is a form of congenital heart disease characterized by a ventricular septal defect (VSD), an “overriding” aorta, right ventricular outflow tract obstruction (RVOTO), and right ventricular hypertrophy.



Synonyms



Fallot’s

Fallot’s tetralogy

Tet

TOF


ICD-9-CM Code



745.2 Tetralogy of Fallot


Epidemiology & Demographics



• TOF is the most common of the cyanotic congenital heart diseases.

• TOF affects 10% of children with congenital heart disease.

• The prevalence is 3.53 cases per 10,000 live births.

• There is a slight predominance of TOF in boys.


Clinical Presentation



• Presentation depends on the severity of the RVOTO and the amount of pulmonary blood flow.

• Loud murmurs of pulmonic stenosis at the upper left sternal border associated with a single second heart sound are common.

• If the pulmonary blood flow is significantly limited, the patient will be cyanotic.

• Soft, continuous murmurs from aorta-pulmonary collateral arteries are common in patients with TOF and pulmonary atresia.


Etiology



• The cause of congenital heart disease is presumably heterogeneous and may depend on the interaction between genetic predisposition and environmental factors.

• Microdeletions of chromosome region 22q11 are frequently found in patients with TOF who have certain craniofacial anomalies.
image Velocardiofacial syndrome

image Sometimes found in patients with TOF not suspected to have a chromosome abnormality
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Jul 2, 2016 | Posted by in PEDIATRICS | Comments Off on Tetralogy of Fallot

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