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.
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