Congenital Heart Defects or Pediatric Heart Problems

"Congenital" means existing at birth and congenital heart defect results when the heart or blood vessels near the heart have not developed normally before birth. These are also called congenital heart disease but calling it a defect is more appropriate as this heart ailment is caused due to a defect or abnormality and not due to a disease. Congenital heart defects may involve:

• Defects in the interior walls of the heart
• Defects in the valves inside the heart
• Defects in the arteries and veins carrying blood to and from the heart.

  

  
  

Various types of congenital heart defects are:

• Ventricular septal defect (VSD): This Congenital heart defect commonly referred to as a hole in the heart, occurs when the muscular wall known as the septum separating the right and left ventricles is not fully formed. This hole lets the oxygen-rich blood to flow from the left ventricle into the right ventricle which is filled with oxygen-poor blood instead of moving into the aortas as is the normal procedure. VSDs can be small, medium, or large. Small VSDs don't cause problems close on their own whereas medium VSDs do not close on their own and may require treatment. Large VSDs allows a large amount of blood to flow from the left ventricle to the right ventricle making the left side of the heart must work harder than normal and at the same time extra flow of blood increases blood pressure in the right side of the heart and the lungs which can lead to scarring of the arteries in the lungs and heart failure. Open-heart surgery usually is done to close a large VSD, in which a patch of fabric or pericardium is sewn over the VSD to close it completely.
• Atrial septal defect (ASD): This is a hole in the septum that separates the atria or the upper chambers of the heart. The hole lets oxygen-rich blood flow from the left atrium to into the right atrium, instead of the left ventricle as it should. Small ASDs doesn't affect how the heart works as they allow only a small amount of blood to leak from one atrium to the other and they close on their own without any treatment. Medium and large ASDs allow more blood to flow from one atrium to the other and it can be repaired using a catheter procedure or open-heart surgery.
• Patent ductus arteriosus (PDA): Before birth the aorta and the pulmonary artery are connected by a blood vessel called the ductus arteriosus which is an essential part of fetal blood circulation. After birth, the ductus arteriosus closes but in some cases they remain open allowing oxygen-rich blood from the aorta to mix with oxygen-poor blood from the pulmonary artery. This puts a strain on the heart and increases pressure in the lung arteries. PDA is treated with medicines, procedures that use catheter, and surgery.
• Pulmonary valve stenosis: The flow of the blood from the right ventricle to the pulmonary artery is slowed by narrowing at the pulmonary valve making the right ventricle to pump harder to get blood into the pulmonary artery that carries blood to the lungs. This can cause heart failure, arrhythmias or enlargement of the right heart chambers. This condition can be treated by a procedure called pulmonary valvuloplasty which uses a balloon to open the narrowed valve.
• Aortic stenosis: It is a congenital heart defect that narrows or blocks the aortic valve opening, making it harder for the heart to pump blood into the aorta which in turn reduces the flow of the blood to the rest of your body. This defect causes enlargement of the heart, or lead to abnormal heart rhythms known as arrhythmias. Surgery to repair or replace the valve and a surgical procedure called balloon valvuloplasty is used for widening of the valve in which a balloon-like device widens the valve so that blood can flow through.
• Coarctation of the aorta: It is caused by the narrowing or coarctation in a portion of the aorta which forces the heart to pump harder to get blood through the aorta and to the rest of the body. The narrowing of the aorta can cause severe high blood pressure, aortic aneurysm, infection of the heart, brain hemorrhage, stroke, heart failure. Treatment usually involves surgically removing the affected portion or widening it through a surgical procedure called balloon angioplasty and placement of a mesh tube that can hold the aorta open (stent).
• Transposition of the great arteries: In this congenital heart defect, the positions of the aorta and the pulmonary artery are reversed or transposed. In transposition the aorta arises from the right ventricle instead of the left ventricle and the pulmonary artery arises from the left ventricle instead of the right which prevents oxygen-rich blood from reaching the body. Surgery is used to repair this condition.
• Tetralogy of Fallot: This condition is a combination of four heart defects which are:
• Ventricular septal defect (VSD)
• Pulmonary valve stenosis
• A misplaced aorta
• A thickened right ventricular wall

In this defect the blood travels across the hole from the right ventricle to left ventricle and out into the body through aorta. Obstruction in the pulmonary valve prevents the normal amount of blood from being pumped to the lungs. This defect usually results in an insufficient amount of oxygen-rich blood reaching the body.

• Ebstein's anomaly: This Congenital heart defect involves a malformed tricuspid valve that does not properly close to keep the blood flow moving in the right direction resulting in the blood to leak back from the lower to upper chambers on the right side of the heart. This defect leads to heart failure and irregular heartbeats.
• Atrioventricular canal defect: This is a combination of defects which involves a large hole in the center of the heart and a single common valve instead of the separate tricuspid and mitral valves. This defect causes the heart to enlarge and the condition occurs most often in children with Down syndrome.
• Hypoplastic left heart syndrome: Usually found in babies, this condition occurs when the left side of the heart including the aorta, aortic valve, mitral valve and left ventricle are underdeveloped. As a result of this defect the baby's body doesn't receive enough oxygen causing cyanosis, difficulty in breathing and being unable to feed. Treatment option for this defect is a heart transplant.
• Truncus arteriosus: This is a congenital heart defect in which the pulmonary artery and aorta which are usually two separate arteries merge into one single large vessel arising from the two bottom of the right and left ventricles. The blood leaving the heart can go either to the heart or the lungs making the heart work very hard and over time this extra blood flow damages the blood vessels in the lungs causing pulmonary hypertension.

Pediatric congenital heart defects are followed by specialists called pediatric cardiac surgeon. These doctors diagnose heart defects and help manage the health of children before and after surgical repair of the heart problem. Specialists who treat heart problems in the operating room are known as pediatric cardio thoracic surgeons.

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