TETRALOGY OF FALLOT (ToF)

 

                     *  TETRALOGY OF FALLOT *

Four anomalies that impact the heart's structure are the hallmarks of Tetralogy of Fallot (TOF), a congenital cardiac condition. Here's what you should be aware of:

 

A rare cyanotic congenital cardiac condition known as tetralogy of Fallot is brought on by a combination of four cardiac defects and causes insufficient blood flow to the lungs.

 

                                                 

                 

 

ü  Tetralogy of Fallot includes four defects:

•       Ventricular septal defect (VSD)

•       Overriding aorta

•       Pulmonary stenosis 

•       Right ventricular hypertrophy (RVH) 

•       Blood with insufficient oxygen is released from the heart and distributed throughout the body as a result of these structural heart defects.

 

Ventricular Septal Defect (VSD)

• A hole in the septum, the wall dividing the heart's two lower chambers (left and right ventricles), is known as a ventricular septal defect.

 

Overriding of Aorta.

• The Aorta is positioned incorrectly. It has been moved to the right and now sits directly above the ventricular septal defect, a hole in the heart wall. Consequently, both the right and left ventricles supply the aorta with a mixture of blood that is oxygen-rich and blood that is oxygen-poor.

Pulmonary stenosis.

Blood flow to the lungs is decreased when the valve that divides the pulmonary artery, the major blood vessel that supplies the lungs, and the lower right chamber of the heart, or right ventricle, narrows.

Right Ventricular Hypertrophy.

The heart's right ventricle's muscular wall thickens when the heart pumps too hard. This could eventually lead to the heart stiffening, weakening, and failing.

Epidemiology

Ten percent of all congenital heart diseases are of the rare cyanotic heart condition known as tetralogy of Fallot, which affects approximately five out of every 10,000 babies.

Causes of ToF.

Most babies with tetralogy of Fallot have unknown causes.

Some babies are born with heart defects due to chromosomal or genetic abnormalities. A combination of genes and other risk factors, such as items the mother or fetus come into contact with in the environment, food or drink the mother consumes, or medications she takes, are also thought to be responsible for heart defects like tetralogy of Fallot.

Signs & Symptoms of ToF

ü  Tetralogy of Fallot signs and symptoms can include:

•       Cyanosis

•       Shortness of breath

•       Slow weight gain

•       Tiring easily during play or exercise

•       Loud & Harsh heart murmur

•       Squatting Posture

•       Clubbing

•       Some children with a more severe form of tetralogy of Fallot may also develop pulmonary atresia.

•       The baby's skin, nails, and lips turn blue all of a sudden after feeding, crying, or waking up from sleep.
We refer to this as "TET SPELLS."

•       Diagnosis of ToF.

ü  Tests to diagnose tetralogy of Fallot soon after the baby is born:

•       Oxygen level measurement (pulse oximetry)

•       Foetal Echocardiogram

•       Chest X-ray

•       Cardiac catheterization

•       Risk Factors of ToF

•       The following are risk factors for tetralogy of Fallot:
a viral disease, like German measles or rubella, that strikes pregnant women

•       Mothers who drink alcohol

•       inadequate dietary intake during gestation

•       a mother who is over 40

•       A parent with Fallot tetralogy

•       The baby has either DiGeorge syndrome or Down syndrome.

•       Management.

•       oxygen availability in cases of cyanosis.

•       Meals should be small and frequent.

•       Give yourself a kneeling or squatting position during "Tet spells" to improve blood flow to your lungs.

•       Pulmonary and valve spasm are reduced by propranolol 1 mg/kg QID.

•       Intravenous prostaglandin therapy improves arterial blood oxygenation and pulmonary blood flow.

•       Surgical Management.

•       TEMPORARY SHUNT SURGERY: To increase the lungs' blood flow
If your child was born too soon or has underdeveloped pulmonary arteries, this procedure might be necessary.
A bypass, or shunt, is made by the surgeon between the pulmonary artery and a sizable artery that divides from the aorta.
The shunt is removed by the surgeon during the intracardiac repair procedure once your baby is ready.

•       Shunts: instruments used to lessen cyanosis
Pott shunt
The Waterston Shut
Blalock-Taussig shunt: still used in certain circumstances

•       INTRACARDIAC REPAIR (Complete Repair): to seal the opening between the heart's lower chambers (ventricles), a synthetic Dacron patch is placed over the ventricular septal defect.
The pulmonary valve needs to be repaired or replaced in order to improve blood flow to the lungs.

•       Complications from ToF surgery.

 

•       Chronic pulmonary regurgitation, or leaking pulmonary valve, causes blood to seep back through the valve and into the right ventricle, the pumping chamber.

•       tricuspid valve leak

•       Ventriculo ventricular septal defects are holes in the wall between the ventricles that might need to be repaired again or might leak even after they are fixed.

•       enlarged right ventricle or malfunctioning left ventricle Abnormal heartbeats (arrhythmias)

•       Heart disease

•       Dilation of the aortic root, or enlargement of the ascending aorta