This is a type of congenital defect consisting of four different abnormalities (tetralogy). These are ventricular septal defect, right ventricular outflow tract obstruction, right ventricular hypertrophy and over riding of aorta over the interventricular septum. It usually results in insufficiently oxygenated blood being pumped to the body causing bluish discoloration of the skin (cyanosis).

• Cyanosis (bluish discolouration) is the most common finding. This gets aggravated by crying or during agitation leading to a state called cyanotic spell which needs special attention.

• Clubbing of fingers (dumbbell shaped finger nails)

• Poor feeding and failure of grow

• Squatting (particularly during cyanotic spells)

Diagnosis

Clinical symptoms are characteristic but may be common in all cyanotic congenital heart disease. However echocardiography is the diagnostic test. Sometimes Cardiac Caterization may be necessary particularly to assess the feasibility of surgery. X ray shows typical boof shaped heart, dark lung field and blood hemoglobin level remains extremely high.

Treatment of Spell

• Avoid excessive crying and excitement

• Feed the child slowly

• Position the child in knee chest position

• Make the child calm by giving medications like morphine

• Different drugs like adrenaline, Beta Blockers are used to increase flow to the lung and thereby reducing the blueness

• If all measure fail, the child may have to be on ventricular and sometimes emergency Shunt operation may be necessary

B.T. Shunt (Palliative)

Surgery to repair the defect is always done in the early age group. However in case of small size of arteries of the lung, a relatively smaller operation is done before the total correction . This is called a shunt operation.  This is usually done through the side of the chest. A connection is established between blood vessel of systemic circulation and pulmonary circulation.

Done through the front of the chest (sternomy). This is an open-heart procedure. After diverting all blood to heart lung machine, the defect is approached through right atrium and pulmonary artery. The right ventricular outflow obstructions are relieved but cutting down the muscle bundle and the valve. The ventricular Septal defect then closed with a patch and outflow tract of right ventricle is reconstructed with pericardium or a homograft.

Most of the surgically treated patients enjoy good life. Sometimes right ventricular outflow tract obstruction or a sever leak in pulmonary valve may cause late right ventricular failure. It may need re-operation. Without total correction of TOF life usually ends. If there is adequate connection between the two chambers and child presents late atrial switch, operation may be done at later date.