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Tetralogy of Fallot: presentation, operation, predictions


The appearance of these children are immediately noteworthy: bluish skin, which are clearly visible even darker vascular, the same blue around lips, on tips of ears and fingers, on the sclera of the eye...

Specialist, just glancing briefly at such a child, I would say that the reason "cinemacast" is most likely tetralogy of Fallot - a congenital anomaly of the heart, which refer to "blue" vices due to pronounced cyanosis.

Four problems immediately

"Tetra" in Greek means "four". That's the number of anomalies immediately has a child's heart, which is prone to Vice, described by the French pathologist, Fallo in the nineteenth century.

During the examination, the expert noted the following defects:

  • Ventricular septal defect;
  • Displacement of the aorta to the right (dextroposition);
  • Narrowing of the pulmonary artery, in the mouth;
  • The increase in right ventricle.

The most important for diagnosis and prognosis of the disease are ventricular septal defect and the degree of narrowing of the pulmonary artery.

To the first year of life the child with tetralogy of Fallot is fully formed specific appearance: a bluish tinge is clearly visible not only on the skin and the sclera of the eyes, ears. In addition, the fingers look like drumsticks and the nails on them are rounded, take a convex shape and resemble glass watches.

Often tetralogy of Fallot combined with nezareatmene the top of the sky, funnel-shaped defect of the chest, feet flat.

Conventional children movement to a sick child not typical: he moves a little, gets tired quickly, but for the rest chooses the posture of squatting or lying on your side.

If the disease is severe, these children are almost up, they often develop severe breathlessness, resulting in fainting. Duration odysessy attacks from two seconds to two to three minutes. They pose the greatest danger, because it is very often the cause of death of patients due to rapidly growing hypoxic coma.

During the examination the child is often seen "heart hump" - a bulge in the region of the heart, and when listening to a short first tone at the apex and attenuated second tone in the pulmonary artery.

Detected changes in the analyses increases the number of red blood cells in the blood to 6х1012/l and above, an increase in the level of hemoglobin is its indicators exceed 130 g/L.

There are signs of heart disease are also on the ECG, phonocardiogram and x-rays. ECG shows right ventricular hypertrophy, phonocardiogram can be heard over a pulmonary artery, andx-ray picture shows the heart in the form of a wooden Shoe and a number of specific changes - depletion of lung pattern, the increase of the heart shadow and more.

Additional methods of examination - ultrasound, Doppler ultrasound, heart catheterization - allows you to specify the diagnosis, identifying the main changes in the heart and direction of blood flow, which is characteristic of tetralogy of Fallot.

The prognosis for life

Only operative treatment - that is the verdict of experts for children diagnosed with tetralogy of Fallot. And the more severe symptoms of the defect, the more often happen Odysee-cinotecnica attacks, the more likely you need to take action.

The peak of the most severe manifestations of this form of congenital heart disease accounted for the first two years of life of children: at this age, they often die from suffocation.

If they manage to survive this period, the disease gradually takes on the attributes of the adult version of course, but most patients still die by adulthood.

Operated children have a satisfactory prognosis: about 80% of them after treatment return to life - though a life with many restrictions.

What differencebut disease

Despite their "awareness" of, tetralogy of Fallot needs to clarify the diagnosis. It is usually differencebut with other "blue" heart defects:

  • The Ebstein's Anomaly;
  • Atresia (complete fusion) of the pulmonary artery;
  • Double discharge of great vascular from the right ventricle;
  • The only ventricle;
  • Common arterial trunk.

When surgery is performed

Surgery for this variety of congenital heart diseases are of two kinds - palliative and radical.

The word "palliative" means a half measure - that is, partial cure. The purpose of palliative surgery is to alleviate the condition of the sick child, and doing them in under three years.

This operation represents the application of anastomoses - additional vascular between arteries: one of the branches of the pulmonary arteries connect the subclavian with, why use a graft or an artificial graft. As a result, the anastomosis of blood from the artery goes to the lungs, and then into the left atrium.

So it is possible to reduce cyanosis and reduce the frequency of attacks of breathlessness, because the left ventricle gets more volume of oxygen-rich blood.

After three years to carry out radical surgery. For the intervention they chose this age because the blood vascular three or four year old child is already quite large and the operation is more successful.

It is performed with heart and attaching it to the apparatusartificial circulation, after which the surgeon closes the interventricular defect and eliminates the narrowing (stenosis) of the pulmonary artery.

During the operation, dying about 10 percent of children, however, successfully carried out the intervention gives patients a chance to live satisfactorily. In a later age they often develop complications in the form of various arrhythmias.

Some clinics now immediately perform a radical operation, bypassing the stage of palliative, however, this type of treatment is performed only when it is not very severe tetralogy of Fallot.