Tetralogy of Fallot

Tetralogia Fallot's pulmonary stenosis associated with highly placed septuni ventricular defect. In this way, the right ventricular empties into the aorta instead of into the pulmonary artery and is therefore highly unsaturated aortic blood oxygen. Early leads to cyanosis, and polioitemije batičastih fingers. Fatigue causes cyanosis. 

The clinical picture

A. The signs and symptoms in severe cases lagging growth. He is a frequent dyspnea, fatigue and facilitates čučanjemse dyspnea; occasionally, until syncope. Express the signs are cyanosis ibatičasti fingers, moderate protrusion desnogventrikla and absence of apical impulse, as ikratak, harsh noise sfstolni if ​​trirduž left edge of the sternum. The heart was not enlarged. In medium-sized openings hear a loud second sound, while jepocepan with pulmonary component of reduced amplitude.


B. Radiographic findings: sučista lung fields. Apex of the heart with a dull konkavnošću in a segment.pulmonary (heart shaped wooden shoes). Right aortic arch is set U25% cases.

C. ECG: umarena desnogventrikla hypertrophy is almost always present. Povremenose are expressed and P waves.

D. Special tests: diagnosis is based on kateterijzacije heart and from the right ventricle angiokardiografije. In this way, determined and morphological appearance. Aortography is recommended as a routine method In patients who are preparing for surgery. Time to show some unexpected aortic Granell associated defects.

Treatment

Tetralogia Fallot is treated surgically with the use of the machine for extracorporeal circulation.Operative mortality was moderately low. Patients with underdeveloped pulmonary arteries previously recommended Blalockov type of surgery. Propranolol (Inderal) is used in syncopal attacks which, in fact, caused by contraction of the infundibulum.

Forecast

In adults the most common tetralogia cainotic congenital heart defects, but children do not reach adulthood so often. The most common cause of death was pronounced hypoxemia. It is also common and vascular thrombosis caused by polycythemia. Strength syndrome is depending on the degree of stenosis and. pulmonary, if the stenosis is greater the higher the desnolevi shunt and pulmonary blood flow decreased. 

Tetralogy of Fallot

Tetralogy of Fallot is the most common congenital heart defect cijanozna and represents 10% of all congenital anomalies. Means the combination of: 1) the defect međukomorske compartments, 2) riding aorta, which is an opening above the defect to receive blood from both chambers, 3) pulmonary stenosis (obstruction of the outflow tract of the right ventricle), and 4) right ventricular hypertrophy.

The clinical picture

Systemic hypoxemia (low oxygen levels) is the cause of most of the symptoms and signs in children and adults. Children lag behind in growth. During physical activity appear dyspnea (disturbed, shortness of breath), and dizziness, and relief appearances at zauuimanju characteristic position - squatting. Although there are forms of tetralogy acijanotični (pale Fallot), which can be found in adults, the majority of patients with this defect is characterized by cyanosis (blue discoloration) with maljičastim fingers and polycythemia (increased number of cells in the blood). Cyanosis can be of varying intensity. Attacks worsening cyanosis occur due to increased tone of excitement in the infundibulum (fear, crying) or taking drugs that increase myocardial contractility. Anoksemične crisis (crisis without oxygen) with sudden weakness, loss of consciousness, seizures, and even death, attributed to spasm - the deterioration of infundibular stenosis. Not seen in adults and in children are a sign of serious defects and indications for prompt surgical correction. Natural history of conservatively treated patients is poor.Frequent kompikacije such as heart attack and brain abscess, and the risk of infective endocarditis is great. Heart failure and arrhythmias are the most common causes of death in most patients until 30 appearances year.

Diagnosis

It is based on clinical presentation, examination, ECG, X-ray, ultrasound and cardiac catheterization.

Treatment

Cijanotički paroxysmal attacks is urgently treated by placing the child in the position with the knees to the chest, giving oxygen and opiates. With prolonged anoxic attacks should be included infusion and acidosis corrected bicarbonate. Once the required general anesthesia or giving beta blockers to stop the attack.

Surgical treatment is indicated in all patients. The ideal is a total correction of defects in the first years of life, which consists in closing the defect međukomorske barriers and reducing obstructions in the outlet of the right ventricle. Most operated almost normal lives, but some occur arrhythmias and sudden death.