Tetralogy of Fallot Illustration

(Blue=Non-oxygenated Blood, Red=Oxygenated Blood, Purple= Mixed)

Signs and Symptoms of Tetralogy of Fallot:

Tetralogy of Fallot is often diagnosed in the first few weeks of life with presenting symptoms including the presence of cyanosis or a loud heart murmur. The degree of cyanosis is proportional to the lung blood flow which is determined by the degree of narrowing of the outflow of the pulmonary arteries. Other common signs and symptoms of Tetralogy of Fallot include:

• Paroxysmal dyspnea (with loss of consciousness)
• Clubbing of the fingers (seen in older children)
• Parasternal heave
• Precordial prominence
• Systolic murmur (often absent in the first few days of life)
• A single S2 can be heard
• Low arterial oxygen content
• Irritability
• Sleepiness

Treatment Options for Tetralogy of Fallot:

Surgical intervention to repair the four specific cardiac anomalies associated with Tetralogy of Fallot have proven to be very effective in the past decade. The surgery can be completed in two operations (the first being a temporary fix and the second being a complete repair). This two step approach is usually recommended for small or premature infants who may be suffering from other conditions. The following describes both surgical repairs:

Temporary Operation – in small blue infants a shunt operation that provides adequate blood flow to the lungs can first be performed. This allows time for the infant to grow enough for a full repair. The shunt is placed between the pulmonary artery and the aorta in an attempt to improve blood oxygenation and is then removed when the complete repair operation is undertaken.

Complete Operation – to do a complete repair, the ventricular septal defect is closed with a patch and the right ventricular outflow tract is opened by removing some thickened ventricular muscle below the pulmonary valve. Repairing or removing the pulmonary valve and enlarging the peripheral pulmonary arteries (that go to the lungs) is also involved in the complete operation. In most cases a Rastelli repair (which is when a tube is placed between the right ventricle and pulmonary artery) completes this operation.