It is a congenital disorder.It occurs as a result of developmental anomaly.The heart wont be able to function normally due to lack of it’s proper functional structures.
Tetralogy of FALLOT is characterized by obstruction to the right ventricular outflow,hypertrophy of right ventricle,ventricular septal defect and dextroposition of aorta with septal override.
CLINICAL MANIFESTATIONS:-
It depends on the degree to which right ventricle is obstructed,time of onset of symptoms and degree of cyanosis.Cyanosis(bluish colour on lips,oral cavity,face) may be present or absent at birth.It precipitates usually by one year.The other clinical manifestations are clubbing,dyspnea(difficulty to breath)on exertion,squatting at times of attack.Cyanotic attacks precipitate as the child becomes active in his later stages of life and lasts for minutes to hours.The growth of the child will be delayed.The associated symptoms are convulsions,restlessness,hyperpenic,gasping respiration,hypoxia and acidosis.It lasts for minutes to hours.
On examination the heart beat will be normal.On x ray examination a concavity can be seen on the left heart border and elevation of heart border.
Major Complications are:-
Cyanotic spells,cerebral thrombosis,brain abcess,infection of the heart wall with bacteria,Congestive cardiac failure.
TREATMENT:-
Treatment is done by a systematic approach.Severe obstruction to the outflow of right ventricle is treated to increase pulmonary blood flow by keeping pulmonary duct by administration of PGE1 and this is a temporary measure till surgery.In less severe cases,medical management is by prevention and prompt treatment of dehydration.oral propanolol administration.
CYANOTIC SPELL is managed by eeiping the patient in a knee-chest positon,administering oxygen and glucose,morphine .2mg/kg,propanolol I.V 0.1mg/kg,correction of acidosis.
Complications like brain absess is treated by administration of antibiotics for 6 weeks,polycytemia by adequate hydration,phlebotomy and volume replacement,bacterial endocarditis by administration of antibiotics for 6 weeks.
SURGICAL MANAGEMENT:-
1.PALLIATIVE PROCEDURE:-decrease hypoxia,improves growth,augments pulmonary artery growth and increae pulmonary artery blood flow.
2.CORRECTIVE SURGERY:-Often aimed at curing.The patient can lead a near normal life.It is performed in early infancy.
