Fallo's tetrad

Description:

Fallo's tetrad — the so-called "blue" heart disease combining by definition of the French pathologist Fallo, four anomalies:
stenosis of output department of a right ventricle (valve, subvalvular, a stenosis of a pulmonary trunk and (or) branches of a pulmonary artery or combined);
high (subaortal) defect of an interventricular partition;
aorta dextroposition;
hypertrophy of a right ventricle (as a result of the complicated outflow of blood from a ventricle).

Symptoms of the Tetrad of Fallo:

Cyanosis – the main symptom of a tetrad of Fallo. Degree of cyanosis and time of its emergence depends on expressiveness of a stenosis of a pulmonary artery. Only severe forms of defect – a "extreme" form of a tetrad of Fallo are diagnosed for children of the first days of life on the basis of cyanosis. Gradual development of the cyanosis (by 3 months - 1 year) having various shades is generally characteristic (from gentle-bluish to "blue and crimson" or "pig-iron-blue"): at first there is cyanosis of lips, then mucous membranes, finger-tips, face skin, extremities and a trunk. Cyanosis accrues with growth of activity of the child. Early "drum sticks" and "hour glasses" develop.

Constant sign is an asthma on type диспноэ (profound arrhythmic breath without the expressed increase in a respiration rate), noted at rest and sharply increasing at the slightest exercise stress. Gradually the delay of physical development develops. Practically since the birth rough systolic noise along the left edge of a breast is listened. The terrible clinical symptom at Fallo's tetrad causing weight of a condition of patients are odyshechno-tsianotichesky attacks. There are they, as a rule, aged from 6 up to 24 months against the background of absolute or relative anemia. The pathogeny of emergence of an attack is connected with a sharp spasm of infundibulyarny department of a right ventricle therefore all venous blood comes to an aorta, causing the sharpest hypoxia of TsNS. Blood saturation by oxygen during an attack falls to 35%. Intensity of noise at the same time sharply decreases up to total disappearance. The child becomes uneasy, a look scared, pupils are expanded, short wind and cyanosis accrue, extremities cold; then the loss of consciousness, spasms follows and development of a hypoxemic coma and a lethal outcome is possible. Attacks are various on weight and duration (from 10-15 seconds to 2-3 minutes). In the poslepristupny period patients it is long remain sluggish and adynamic. Development of a hemiparesis and severe forms of disturbance of cerebral circulation is sometimes noted. By 4-6 years the frequency of emergence and intensity of attacks considerably decreases or they disappear. It is connected with development of collaterals through which there is more or less adequate blood supply of lungs.

Depending on features of clinic allocate three phases of a course of defect:

The I phase – relative wellbeing (from 0 to 6 months) when the condition of the patient rather satisfactory, is not present lag in physical development;

The II phase - odyshechno-tsianotichesky attacks (6-24 months) of which the large number of brain complications and lethal outcomes is characteristic;

The III phase – transitional when the clinical picture of defect begins to accept adult lines;

Reasons of the Tetrad of Fallo:

Fallo's tetrad one of the most often meeting blue (followed by cyanosis) inborn heart diseases. Its frequency is 10-15% among all inborn heart diseases. Perhaps, defect has the hereditary nature.

Treatment of the Tetrad of Fallo:

Indications to operation have absolute character. In our country most of surgeons adhere to a stage surgical method of treatment of children with Fallo's tetrad.
Up to 3 years the palliative operations significantly facilitating life of patients are carried out. The purpose of performance of palliative operations — to increase inflow of blood to a small circle of blood circulation.

1. Endocardiac operative measures.
pulmonary valvulotomy — a section of shutters by means of the valvulotome entered through a pulmonary trunk through the avascular site of a right ventricle;
infundibulyarny resection on R. Brock (1948), excision of the muscular roller at a subvalvular stenosis by means of Brock's rezektor.

2. Extracardiac interventions — creation of a vascular anastomosis — shunts for increase in inflow of blood to a small circle of blood circulation
the pulmonary and subclavial shunt on Bleloku-Taussing (1945, the right subclavial artery is sewed sideways the right pulmonary artery)
the arterial and pulmonary shunt on Vaterstounu-Kuli (the ascending part of an aorta anastomose with the right pulmonary artery a side - in - a side from a front and side thoracotomy in the IV mezhreberye on the right),
the aorto-pulmonary shunt according to Potts-Smith (an anastomosis a side - in - a side between the descending aorta and the left pulmonary artery from a left-side front and side thoracotomy).

Second stage.
It is carried out in 2-4-6 months after the first. Radical operation is carried out in the conditions of IK with a careful cardioplegia. Perfusion is begun with the perfusate cooled to 10-12 °C in the device IK. The general hypothermia at a temperature of 21,6 °. After crossclamping of an aorta open a cavity of a right ventricle and depending on character of a stenosis under control of sight carry out operational receptions on elimination of a stenosis of output department of a right ventricle. Defect of an interventricular partition is eliminated with a patch podshivaniye P-shaped seams. In a front surface of a right ventricle sew a patch for increase in width of output department of a right ventricle
aorto-pulmonary shunting by a prosthesis from polytetrafluoroethylene.