What is Falott's syndrome?

Falott syndrome, also known as Falott's tetralogy, involves four heart defects that make up the clinical picture of the disease. These are:

ventricular septal defect (also known as VSD) - an abnormal connection between the chambers of the heart occurring as an opening.
the aorta escaping from behind the defect, which is supplied with blood by both chambers of the heart - so-called aortic dislocation,
narrowing of the outflow tract- the pulmonary artery becomes narrower, so that the heart has to work harder to "outflow" the blood,
hypertrophy of the right ventricle - a result of the intensified work of the heart.

Symptoms

A symptom visible to the naked eye is cyanosis, which can be observed in a child. As the child grows older and becomes more active, cyanosis progresses. In critical situations, when there is a rapid decrease in blood flow through the lungs, a hypoxaemic attack may occur with the appearance of severe cyanosis, sudden acceleration of breathing, anxiety and loss of consciousness. In older children suffering from ToF with chronic hypoxaemia, so-called 'clubbed fingers' are observed , or abnormally developed fingers. In children with mild pulmonary artery stenosis, no cyanosis is observed, but the so-called "pink ToF" is referred to - this is because the children are pinkish as after intense exercise.

What is Fallot syndrome, photo: panthermedia

Treatment

The treatment of tetralogy of Falot is based on surgical methods. Pre-operatively, newborns with significant cyanosis are prepared for surgery by administering prostaglandin E1 (PG1) to improve pulmonary flow and thereby help oxygenate the blood. Correction of Fallot syndrome is performed under so-called extracorporeal circulation and hypothermia, usually between the ages of three months and three years. The interventricular defect is closed with a special plastic 'patch'. In children with pulmonary artery hypoplasia, coronary artery anomaly or pulmonary artery obstruction, a so-called systemic-pulmonary anastomosis is performed using the Blalock and Taussig method, i.e. an anastomosis between the right subclavian artery and the right pulmonary artery. Total anastomosis can be performed in several years of age. During the operation, the ventricular septal defect is closed, the hole in the right ventricular wall is cut and an artificial vessel is sewn into the edges of the hole to connect the right ventricle to the pulmonary artery trunk. It may become necessary, as the child grows, to have another operation to enlarge the vessel or to transplant the pulmonary artery along with the valve. In children diagnosed with defects with high pulmonary flow that are entirely dependent on the collateral circulation, the connecting vessels to the systemic circulation are severed and anastomosed to each other. "Generated" pulmonary artery is connected to the right ventricular outflow using an artificial vessel or by means of a biological graft.

Postoperative complications

The postoperative mortality rate ranges from 2 to 5%. The most common complications include bleeding, circulatory failure and cardiac arrhythmias. So-called distant complications, such as secondary or residual right ventricular outflow tract stenosis, residual ventricular septal defects, proximal pulmonary artery stenosis, may require reoperation.

Life after surgery

Most children have a chance of returning to a relatively normal life after complete correction. The 30-year survival rate is close to 90%. Children who have undergone surgery for Falott syndrome require ongoing cardiac monitoring and routine echocardiographic checks.

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Tetralogy of Fallot - diagnosis and treatment

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