Tetralogy of Fallot, or Fallot syndrome, is a complex, cyanotic, congenital heart defect that causes constant hypoxia of the body - any physical effort, e.g. crying, causes cyanosis, i.e. a bluish color of the skin. "Blue babies", as newborns and infants with this heart defect are called, require immediate surgery. What is the tetralogy of Fallot and what are its causes. What symptoms indicate Fallot's syndrome? What is cardiac surgery treatment?

Tetralogy of Fallot (TOF) , or the team of Fallot, is a complex,cyanosis ,congenital heart defect, which has 4 different heart defects. The tetralogy of Fallot accounts for approximately 3-5% of all congenital heart defects. It occurs in about 10% of infants with congenital heart disease, making it the most popular cyanotic heart defect in children. It is also the most common form of cyanotic heart disease in adults. The term "cyanosis" refers to one of the symptoms of this heart defect - a bluish color, e.g. mouth, tongue, fingers due to lack of oxygen.

Tetralogy of Fallot - reasons

TheFallot teamconsists of fourdefects in the structure of the heart(the so-called Fallot tetrad). These arecausesdysfunction of the entire circulatory system.

1. Ventricular septal defect - abnormal connection between the right and left ventricles.

2. Narrowing of the mouth of the pulmonary artery (pulmonary valve) that carries blood from the heart to the lungs. The heart must work with redoubled force so that the blood from the heart flows into the vessels in the lungs. Therefore, it appears:

3. Right ventricular hypertrophy (thickening)

4. Movement of the aorta (that is, the main artery that carries blood from the heart to the entire body) to the right over the vent in the ventricular septum. Then it is said that the aorta "sits astride" both chambers and the opening between them.

In a properly functioning circulatory system, oxygen-depleted venous blood (deoxygenated blood) is pumped from the heart through the pulmonary artery to the lungs to obtain oxygen. It then returns to the heart and is pumped from the aorta to the body. The ventricular septal defect allows oxygen-poor blood to flow from the right to the left ventricle and then into the aorta and systemic circulation(large), bypassing the lungs. In addition, blood flow from the right ventricle to the pulmonary arteries is obstructed by pulmonary valve constriction. Therefore, the part of the blood that reaches the tissues does not contain the oxygen necessary for them. Therefore, we are dealing with constant hypoxia of the body, which is manifested by cyanosis.

Tetralogy of Fallot - symptoms

Cyanosis

Cyanosis is very severe in the case of complete narrowing of the pulmonary valve or the outflow tract of the right ventricle. Lips and nails become blue, and the skin of the whole body turns dark blue, especially during increased activity of the toddler, e.g. during feeding, crying or defecation. This is because, during exercise, the oxygen demand of the body's tissues increases.

In the rare cases where the obstruction of the mouth of the pulmonary artery is small, cyanosis may not appear at all. Then we talk about the "pink" Fallot band.

Other symptoms of Fallot's syndrome:

  • shortness of breath
  • fatigue of the baby during exercise, e.g. while feeding the baby
  • insufficient weight gain
  • stick fingers (unusually rounded fingertips)

Young children (usually between 6 and 24 months of age) often develop hypoxic (anoxic) seizures - critical drops in blood oxygen levels. Most often they appear in the morning, after a good night's sleep. The characteristic symptoms are rapid breathing and increasing cyanosis.

Squatting is also a very characteristic symptom. The baby's crouching position makes it easier for the blood to reach the lungs (when the baby squats, it is more difficult for the blood to flow through the aorta. Therefore, it chooses an easier path and goes to the lungs, and the whole body is oxygenated).

In adults, except for the above-mentioned symptoms, a tendency to bleeding (gums, haemoptysis) and even thrombotic changes are observed.

Tetralogy of Fallot - diagnosis

In addition to the physical examination, during which the doctor can hear a systolic murmur above the heart, an EKG and a chest X-ray are also performed. Electrocardiography can identify features of right ventricular hypertrophy. On the other hand, in a chest X-ray, the shape of the heart resembles a shoe. The package of necessary examinations also includes echocardiography, which shows right ventricular hypertrophy, usually with an altered pulmonary valve, and angiocardiography - examination of the coronary vessels.

Tetralogy of Fallot - treatment

Tetralogy of Fallot is a serious heart defect, and if left untreated, it can lead to a delay in physical developmenta child and even his death. Therefore, the treatment consists of a cardiac surgery, the aim of which is to completely correct the defect. During open-heart surgery, the doctor closes the ventricular septal defect and widens the narrowing of the pulmonary valve. Usually the operations are carried out in stages until the defect is completely eliminated. This type of operation is performed in the so-called in extracorporeal circulation, usually in the first year of life.

Patients in whom, for various reasons, the complete correction of the defect could not be performed, usually only survive for 30 years.

Important

Complications after the procedure

Post-operative patients may develop atrial and ventricular arrhythmias or pulmonary valve leakage. Then another operation is needed. In some cases, it is possible to implant a cardioverter - defibrillator, which protects against dangerous arrhythmias.

In order to eliminate infectious endocarditis, antibiotic prophylaxis is also necessary before various types of procedures, e.g. dental procedures.

Category:

Cardiology