Patent Ductus Arteriosus (PDA)
Before birth, there is a natural opening between the aorta (the main artery to the body) and the pulmonary artery (the main artery to the lungs) called the ductus arteriosus. This opening usually closes shortly after birth. PDA occurs when this opening fails to close; PDA occurs in about 10% of infants.
PDA is often treated initially with a medication called indomethacin. If the ductus fails to close on its own or with indomethacin, surgery is performed. A small incision is made on the left side of the chest. The ductus is either ligated (tied off) or cut.
Coarctation of the Aorta (COA)
Coarctation of the aorta occurs when a segment of the aorta has a very narrow spot, like that in an hourglass. To repair this defect, an incision is made on the left side of the chest.
There are a few different techniques used in this repair. One approach is to remove the narrowed segment of the aorta and stitch the remaining ends together. This can usually be done in older children due to the size of the aorta.
Repair in infants is usually achieved by using a subclavian flap. An incision is made in the narrowed portion of the aorta. A patch is made from a portion of the left subclavian artery (the artery to the arm) to enlarge the diameter of the aorta.
Atrial Septal Defect (ASD)
The atrial septum is the wall between the left and right atria (upper chambers) of the heart. There is a natural opening prior to birth that usually closes spontaneously. When the defect fails to close, a child is left with an ASD.
ASDs may possibly be closed in the heart catheterization lab with two small umbrella-shaped “clamshell” devices placed on both the right and left side of the septum. The two devices are attached together, closing the hole in the heart. This procedure is still in the experimental phase, and is performed only in select centers.
Surgical repair is a well-accepted treatment for ASD. The septum is closed using sutures or covered with a patch made of membrane or synthetic material.
Ventricular Septal Defect (VSD)
The ventricular septum is the wall between the left and right ventricles (lower chambers) of the heart. A hole in the ventricular septum is called a VSD.
Small defects usually close on their own — 90% by age 8 — and do not require surgery. Larger defects or those causing heart failure require surgical repair with a synthetic patch.
Tetralogy of Fallot (TET/TOF)
Tetralogy of Fallot is a complex congenital heart defect with a range of severity that requires surgical correction, typically between 6 months and 2 years of age.
Types of repairs vary related to the specific defects. The ventricular septal defect is closed as described above. The pulmonary valve is opened and the thickened muscle (stenosis) is removed. A patch may be placed on the right ventricle to improve circulation to the lungs.
Transposition of the Great Vessels (TGA)
The aorta normally comes from the left side of the heart, and the pulmonary artery normally comes from the right side. Transposition of the great vessels occurs when these arteries come from the opposite sides of the heart from where they should.
Transposition of the great vessels requires surgical correction. If possible, this surgery is performed shortly after birth. The most common repair is an arterial switch. The aorta and pulmonary artery are divided. The pulmonary artery is connected to the right ventricle, where it belongs, and the aorta is connected to the left ventricle, where it belongs.
Truncus Arteriosus
Truncus arteriosus is a rare condition that occurs when the aorta and the pulmonary artery arise from one common trunk. This very complex defect requires a complex surgical repair.
If the baby is very sick, the repair is done in the first few months of life. If the baby is not so sick, surgeons wait to operate until the baby is between 9 and 15 months of age.
The pulmonary arteries are separated from the aortic trunk and any defects are patched. There is always an associated VSD, which is patched. A conduit is then placed between the right ventricle and the pulmonary arteries.
Tricuspid Atresia
Tricuspid atresia is a condition in which the tricuspid valve (the valve between the upper and lower chambers on the right side of the heart) is severely narrowed. This condition severely restricts blood flow from the right side of the heart to the lungs.
Other defects may exist with tricuspid atresia and can aid in the oxygenation of blood and flow to the lungs. A medication called prostaglandin E may be given to maintain a patent ductus arteriosus as an alternate channel to the lungs until corrective surgery can be performed.
A series of shunts and surgeries may be necessary to correct this defect. The goal of this surgery is to join the right atrium or occasionally the right ventricle to the pulmonary artery.
Total Anomalous Pulmonary Venous Return (TAPVR)
TAPVR is a condition in which the pulmonary veins bring oxygenated blood from the lungs back to the right side of the heart, rather than to the left side of the heart, where it should be.
This condition requires surgical correction, the timing of which depends on how sick the baby is. The surgery may be done in the newborn period if the infant has severe symptoms. If not done immediately, it is done in the first six months of life.
TAPVR repair is an open-heart procedure. The pulmonary veins are attached to the left atrium, where they belong, and any abnormal connections are closed. If a PDA is present, it is tied off.
Hypoplastic Left Heart or Right Heart Syndrome (HLHS/HRHS)
This is a very severe heart defect in which the left side or right side of the heart is severely underdeveloped.
The best reported results are with a series of 3 heart operations. Prostaglandin is usually administered once the baby is born to keep the PDA open. The first operation for HLHS or HRHS may be the BT Shunt or PA Banding. It may also be referred to as the Norwood procedure. The first surgery is usually done in the first week of life.
The remaining 2 surgeries are the same for both HLHS and HRHS. At about 4-6 months of age, the second operation, the Glenn, is usually performed. A third operation, the Fontan, is usually required before Kindergarden.
A heart transplant may be performed to treat this condition, but donor hearts for infants are rare and the procedure can only be performed at a small number of centers.
Double Outlet Right Ventricle (DORV)
DORV is a very rare congenital heart defect in which both of the great arteries of the heart (the aorta and the pulmonary artery) are connected to the right ventricle instead of separate ventricles. The degree of the defect can vary.
Although the surgical procedures vary, they all share the same basic goal: connecting the left ventricle to the aorta while keeping the pulmonary artery connected with the right ventricle. One approach is called intraventricular repair. During this operation, the surgeon creates a tunnel from the ventricular septal defect connecting the left ventricle to the aorta. This allows blood to flow from the left ventricle to the aorta.
Another surgical procedure is called the arterial switch operation (ASO). This surgery involves two steps. In the first step, a tunnel is created from the VSD to the pulmonary artery. At that point, the heart is wired completely backwards because the left ventricle is now connected to the pulmonary artery, and the right ventricle is connected to the aorta – the reverse of the normal heart. In the second step, the two great arteries are disconnected and reconnected to the correct ventricles. The coronary arteries are transferred to the newly positioned aorta for proper blood supply to the heart. Based on tests conducted before surgery, the surgeon may correct other heart conditions during surgery.
Other options include the Fontan procedure and the Rastelli procedure.
Double Inlet Left Ventricle (DILV)
Double Inlet Left Ventricle is a congenital heart defect where both the left atrium and the right atrium feed into the left ventricle. Usually a hypoplastic right ventricle also exists (the condition is sometimes referred to as “single ventricle”). The arteries are usually transposed (the aorta from the right ventricle, and the pulmonary artery from the left ventricle).
Infants born with DILV have excessive blood flow to the pulmonary circulation. Excessive pulmonary circulation leads to decreased systemic circulation (lack of oxygen to the body and organs). Infants develop cyanosis and/or breathlessness early.
Surgery is usually required. Surgical options include the Damus-Kaye-Stansel Procedure, the Fontan procedure, and the Norwood procedure. The goal of all of these is separating the pulmonary and the systemic circulation.
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