Congenital heart surgery; Patent ductus arteriosus ligation; Hypoplastic left heart repair; Tetralogy of Fallot repair; Coarctation of the aorta repair; Heart surgery for children; Atrial septal defect repair; Ventricular septal defect repair; Truncus arteriosus repair; Total anomalous pulmonary artery correction; Transposition of great vessels repair; Tricuspid atresia repair; VSD repair; ASD repair; PDA ligation

Heart defects come in all types, from minor to major. Defects can occur inside the heart or in the large blood vessels outside the heart. The heart defect may need immediate surgery or may be able to safely wait for months or years. In most cases, the timing of the surgery will depend on how sick the baby is.

The heart defect may be repaired in a single surgical procedure or may require a series of procedures. Surgery may involve opening the heart to repair defects or repairing defects of the blood vessels.

An incision may be made through the breastbone (sternum) and between the lungs (mediastinum) while the child is under general anesthesia. For some heart defect repairs, the incision is made on the side of the chest, between the ribs (thoracotomy), instead of through the breastbone (sternotomy).

It is sometimes necessary to use tubes to re-route the blood through a special pump (heart-lung bypass machine) that adds oxygen to the blood and keeps it warm and moving through the rest of the body while the repair is being done. This machine does the work of the heart and lungs during the operation.

Heart surgery for children requires a specialized team of health care providers:

• Pediatric heart (cardiovascular) surgeons
• Pediatric cardiologists and echocardiographers
• Pediatric anesthesiologists
• Pediatric heart-lung (cardiopulmonary) bypass pump technologists
• Pediatric surgical nurses and technicians
• Pediatric intensive care physicians and nurses

Heart surgery requires intensive and extensive monitoring, treatment and coordination by the entire team. Heart surgery for children may take as many as 12 hours in the operating room.

After heart surgery, the child will be moved to the intensive care unit (ICU) to be constantly and closely monitored and treated for several days. During this time the child will have the following:

• A tube in the airway (endotracheal tube) and a respirator to help with breathing. The child will be kept sleeping (sedated) while on the respirator.
• One or more small tubes in a vein (IV line) to give fluids and medications.
• A small tube in an artery (arterial line) to measure the blood pressure.
• One or two chest tubes to drain air, blood, and fluid from the chest cavity.
• A tube through the nose into the stomach (nasogastric tube) to empty the stomach and give medications and feedings for several days.
• A tube in the bladder to drain and measure the urine for several days.
• Many electrical lines and tubes used to monitor the patient.

The child may also have pacemaker wires in the chest in case a pacemaker is needed to regulate heartbeat and rhythm.

SPECIFIC HEART DEFECT SURGERIES INCLUDE:

Patent ductus arteriosus (PDA) ligation

• Before birth, there is a natural blood vessel 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. A PDA occurs when this opening fails to close.
• PDA is often treated initially with a medication called indomethacin. If the ductus arteriosus 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 arteriosus is either ligated (tied off) or divided and cut. A minimally invasive technique is available. In this procedure, the surgeon inserts a few small tubes into the chest to close the PDA.

Coarctation of the aorta repair

• 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 generally is made on the left side of the chest, between the ribs.
• A few different techniques can be 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.
• Another approach is called 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.
• In the third and most common approach, the surgeon cuts the narrowed region and uses a Gor-tex patch to enlarge the area.
• In the fourth approach, the surgeon places a tube between the areas before and after the narrowing in the aorta, allowing blood to bypass the narrowed segment.

Atrial septal defect (ASD) repair

• 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 can 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 performed only in select centers and can only be done on certain types of ASDs.
• 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) repair

• 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.
• Depending on the location of the lesion, small defects usually close on their own -- 90% by age 8 -- and do not require surgery. Larger defects, small defects in some part of the ventricular septum, or those causing heart failure or endocarditis require surgical repair with a synthetic patch.
• Some VSDs may be closed using cardiac catheterization.

Tetralogy of Fallot repair

• 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 and main pulmonary artery to improve circulation to the lungs.

Transposition of the great vessels repair

• 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 and coronary arteries are connected to the left ventricle, where it belongs.

Truncus arteriosus repair

• Truncus arteriosus is a rare condition that occurs when the aorta, coronary arteries, and the pulmonary artery arise from one common trunk. This very complex defect requires a complex surgical repair.
• Repair is generally done in the first few days or weeks of life. The pulmonary arteries are separated from the aortic trunk and any defects are patched. There is usually an associated VSD, which is patched. A conduit is then placed between the right ventricle and the pulmonary arteries.

Tricuspid atresia repair

• 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 missing. Blood must cross an ASD, VSD, or a PDA to get to the lungs. This condition severely restricts blood flow 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 allow blood from the body to flow into the lungs, and blood from the lungs to be pumped to the rest of the body through the left ventricle.

Total anomalous pulmonary venous return (TAPVR) correction

• 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 and divided.

Hypoplastic left heart repair

• This is a very severe heart defect that results from a severely underdeveloped left heart and causes death in most affected babies if not treated. Operations to treat this defect are performed at specialized centers with excellent success rates.
• The best reported results are with a series of three heart operations. The first operation is done in the first week of life. This is a complicated surgery in which a single vessel is formed from the pulmonary artery and aorta to create a blood supply to the lungs and the body.
• At about 4-6 months of age, the second operation is performed. A third operation is required a year later.
• 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.

The type and timing of surgical repair depends on the child's condition and the type and severity of heart defects.

In general, symptoms that indicate that surgery is needed include the following:

• Blue or gray skin, lips, and nail beds (cyanosis), meaning there is not enough oxygen in the blood (hypoxia)
• Difficulty breathing because the lungs are "wet," congested, or fluid-filled (congestive heart failure)
• Problems with heart rate or rhythm (arrhythmias)
• Excessive workload on heart that interferes with breathing, feeding, or sleeping

Risks for any anesthesia include the following:

• Reactions to medications
• Problems breathing

Risks for any surgery include the following:

• Bleeding
• Infection

Additional risks of heart surgery include the following:

• Blood clots (thrombi)
• Air bubbles (air emboli)
• Pneumonia
• Heartbeat problems (arrhythmias)
• Heart attack
• Stroke

The outcome of heart surgery depends on the child's condition, the type of defect, and the type of surgery. Many children recover completely and lead normal, active lives.

Most children need to stay in the intensive care unit (ICU) for 2 - 4 days and then stay in the hospital for 5 - 7 more days. By the time the child is transferred out of the ICU, most of the tubes and wires have been removed and the child is encouraged to resume many of his daily activities.

At the time of discharge, parents are instructed on activity, how to care for the incision, and how to give medications their child may need.

The child needs at least several more weeks at home to recover.

Zipes DP, Libby P, Bonow RO, Braunwald E, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine, 8th ed. St. Louis, Mo; WB Saunders; 2007.