Types of Cardiac Conditions

Tetralogy of FALLOT (TOF):

Children with Fallot’s Tetralogy have 4 defects in their heart:

1). The pulmonary valve which is the valve in the heart that controls the flow of blood from the right side of the heart to the lungs is very narrow. This restricts the blood flow into the lungs. This is called pulmonary stenosis.

2). There is a hole in the wall or septum  between the right and left  ventricles. This is called  a ventricular septal defect

3). The aorta overrides the hole which means it gets blood form both the ventricles rather than form just the left ventricle. The aorta normally supplies oxygen filled blood to the rest of the body but in a child with TOF the blood is contaminated with de-oxygenated or blue blood from the right ventricle. This leads to lower oxygen levels that normal.

4). Since the right ventricle has to pump blood through a narrow pulmonary valve it becomes thick and abnormal overtime and fails if the child is not operated upon.

Symptoms: The child gradually become bluer and more breathless. The child does not grow as its normal peers and also has limited physical abilities.

Treatment: An operation is required to increase the amount of blood going to the lungs

There are two operations that can be done:
1). Shunt is a ‘holding’ operation that buys the child time until a corrective operation is performed. In this operation an extra tube is inserted to divert blood form the body into the lungs. This helps to make the child less breathless.

2). Corrective operation: the operation involves removing the narrowing of the pulmonary valve  and closing of the hole between the 2 pumping chambers or ventricles.
The operation is performed using a heart lung bypass machine and the child can be quiet ill after surgery requiring expert intensive care

In the UK, this corrective operation is performed by about 6 months of age whereas in India there are children who are much older who have not benefited from corrective surgery because of the lack of local expertise.

Ventricular Septal defect (VSD):

Children with this condition have a hole in the wall or partition between the two pumping chambers or ventricles. As a result, the blood flows from the left to the right ventricle and into the lungs which means that there is abnormal blood flow into the lungs which causes increases chest infections, breathlessness and an abnormal increase in the pressure of the lungs.

Surgery: The operation is ideally carried out before the child is two years old to prevent a build up of abnormal pressure in the child’s lungs.A Heart Lung machine is used during the operation and the surgeon closes the hole with a patch.

Coarctation of the Aorta:

In this condition the aorta which is the biggest artery in the body and is responsible for taking blood from the heart to the rest of the body is narrow. That means that the left ventricle which normally pumps blood through the aorta has to pump at a higher pressure to force blood through the narrow aorta. Eventually it will fail.

Surgery: Must be performed early on to prevent the left ventricle failing and involves opening the left side of the chest, exposing the aorta  by dissection .The narrow part of the aorta is then cut and removed and then the two remaining ends of the aorta are joined together. This operation is carried out without having to use a heart lung bypass machine.

Anomalous pulmonary venous connection:

In the normal heart, the pulmonary veins (the veins that drain blood from the lungs back to the heart) are connected to the left atrium. In this condition, these veins are connected to the right side of the heart or the blood vessels emptying into the right side of the heart.    

Symptoms: Since the red or oxygenated blood is not getting back to the left side or the correct side to be pumped around the rest of the body, the child is often blue or cyanosed particularly if these blood vessels are also narrow. If however, the abnormally draining pulmonary vessels are wide open then the child will present with breathlessness rather than blueness.

Surgery: An operation is required soon after the diagnosis is made. The operation is to redirect the blood flow from the lungs to the left atrium. A heart lung bypass machine will be required during the operation. This can be quite a difficult operation and the child might need prolonged stay in the intensive care unit after the operation.

Pulmonary stenosis:

Pulmonary stenosis is narrowing of the pulmonary valve which is the valve in artery that takes blood to the lungs. The valve normally works by preventing leaking back to the right side of then heart. When the valve is narrow or ‘stenosed’ the right side of the heart or right ventricle has to work harder to force the blood through the narrowed area. The narrowing maybe mild, moderate or severe.

Symptoms: Over time the right ventricle will start to struggle under the strain necessitating surgery.

Surgery: Usually this condition can be managed without surgery but by a balloon inserted via a groin vein to stretch the valve open. Rarely surgery maybe required.

Atrioventricular Septal defect:

Children with this defect have a large hole in the middle of the heart  between the receiving chambers and  the pumping chambers. This hole leads  to excessive blood flowing  from the left side of the heart to the right side and through into the lungs.

This increased flow of blood at high pressures gives the heart more work to do which will ultimately cause the heart to fail. In addition, the high pressure in the lungs damages the blood vessels. To complicate matters further, the valves leak and blood flows the wrong way further adding to the strain

Symptoms: Breathlessness, repeated chest infections, difficulty feeding and poor weight gain.

Surgery: Needs to be done soon before the pressure in the lungs increases.It is major surgery requiring a heart lung machine.During the operation, the large hole is closed with one or two patches.The leaking valves are also repaired.The intensive care stay can be difficult and prolonged and is best done by doctors and nurses who have specific training in looking after children following major complex heart surgery.

Transposition of the Great Arteries:

In this condition, the two  main arteries of the body  the pulmonary artery and the aorta come form the wrong side of the heart. In other words the aorta which takes the blood to the body arises from the right ventricle instead of the left ventricle. This is effect means that blue blood which has not been oxygenated is pumped around the body. Meanwhile the pulmonary artery which normally takes blood to the lungs comes from the left ventricle instead of the right ventricle. This means that red oxygenated blood  keeps going to the lungs again and again. This means the left and right sides of the heart are working in isolation of each other.

The baby can only survive if some of the red blood somehow mixes with the blue blood that is going around the body. This can happen naturally if there is the child also has a hole between the two ventricles or the through a normal communication between the pulmonary artery and the aorta called the ductus arteriosus which is normally present in the foetus but usually closes soon after birth.

Symptoms:The symptoms appear very early in life,usually in the first few days. The baby is blue and breathless and unable to feed. If there is a good communication between the two side of the heart that allows blood to mix then symptoms will not appear until later.

Surgery:The first procedure that is required is a procedure to allow mixing of blood between the two sides of the heart. This is a relatively minor procedure which entails creating a hole between the left and right atria.

The procedure is called Balloon Atrial Septostomy and involves passing a tube or catheter through the baby’s groin upto into the heart and literally tearing a hole in the common atrial wall. This artificial hole then allows red blood to reach then body and  keeps the baby going until definitive surgery.

Definitive surgery is called the arterial switch operation where the aorta and pulmonary artery are disconnected and ‘switched ‘or swapped to the correct place.The coronary arteries which are the arteries that supply blood to the heart also have to be switched onto the Aorta.
Any holes within the heart will also have to be repaired at the same time. The best time to do the operation is in then first month of life. The surgery is major open heart surgery requiring a Heart Lung Bypass machine and meticulous intensive care afterwards.

If the child is not fortunate enough to be operate upon in the first month of life, then doing an Arterial Switch becomes more difficult.There are other procedures available like the Senning or Mustard operations which involve switching the Atria rather than the Great Arteries .Neither of these procedures is as effective as the Arterial Switch operation but will allow the child a reasonable quality of life.

Aortic Stenosis:

The Aortic valve is the valve between the Left Ventricle(the pumping chamber on the left side of the Heart) and the Aorta(the blood vessel which takes oxygenated blood around then body.In Aortic Stenosis, the valve is usually narrow which means  the left side of the heart or left ventricle has to pump harder to push blood through the narrow valve. Over time this causes significant strain on the left ventricle and it will fail.

Symptoms: In the newborn periodn the condition can be very serious and requires either cathetewr intervention ir  (Missing/ truncated text from word document - please supply.)

 

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