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Cardiac Surgery

Treatment

Mechanical Therapies

Our surgeons are renowned for the implantation of pacemakers and defibrillators in infants and children using endocardial techniques.

In some cases the heart muscle becomes irritated from swelling or scarring, and this affects the electrical pathways that conduct heart rate and rhythm. Some children with abnormal or dangerous arrhythmias may benefit from placement of a pacemaker or an implantable cardioverter defibrillator. These devices, along with some medications, may help correct potentially life-threatening arrhythmias. Pacemakers are small, battery-operated devices-composed of a generator and a computer chip-that are placed under the skin of the chest or abdomen with electrical wires (leads) threaded through veins into the heart. The devices monitor the heartbeat and help the heart to beat in a regular rhythm. Small electrical charges travel from the pacemaker, through the wire, to an electrode placed in the heart wall. Implantation surgery usually requires sedation or anesthesia.

Once a pacemaker is inserted, it requires careful monitoring to ensure that the electrical settings are correct. Both the pacemaker's battery and the child's heart rhythm need too be checked monthly. A monitor is placed over the skin above the pacemaker's generator during routine monthly check-ups. Over time, pacemakers may need to be replaced.

Cardiac assist devices are machines that do the work of the heart by using a mechanical pump to deliver blood to the body. The improved blood flow allows patients to breathe easier and experience less fatigue, while allowing other organs to recover from the stress of heart failure. Cardiac assist devices are used when all other therapies have failed and the heart failure is severe. These devices are typically used as bridges to transplantation: they help support the child until a suitable donor organ can be found. In some cases, the device may be used as a temporary measure until the heart has recovered sufficiently to function on its own. There are a variety of cardiac assist devices available. The type of cardiac assist device chosen depends on body size and the type of support needed.

For critically ill children, our surgeons will use a left ventricular assist device (LVAD)-a mechanical pump that takes over the function of the damaged ventricle of the heart and restores normal blood flow until they can perform transplant. NewYork-Presbyterian/Columbia cardiac surgeons pioneered the LVAD in adults, which has now expanded to include an LVAD program specifically for children. Biventricular Assist Devices (BIVADs) offer support for both ventricles. There is hope that engineering improvements may eventually make LVADs a reasonable alternative to heart transplantation. In fact, recent advances in LVAD design have led to their approval as a destination therapy for adults with severe heart failure who are not candidates for heart transplantation.

ECMO is a short-term cardiac assist device used either to rest the heart until it recovers or to support the circulation for the critically ill child waiting for a donor heart. This device is similar to the cardiopulmonary bypass machine used during open heart surgery. It mechanically pumps oxygen into the blood and helps circulate blood through the body. ECMO is a short-term option for the very young, or in children whose anatomy will not permit another type of assist device, such as an LVAD. Our Hospital's physicians participated in the earliest development of ECMO, making our facility one of the first in the world to use this life-saving technology successfully in children.

Surgical Procedures

The pediatric cardiac surgery program of Morgan Stanley Children's Hospital offers a breadth or expertise and depth of experience that is incomparable, providing young patients with heart defects with treatment options never thought possible just a short time ago. Each year, our pediatric cardiac surgeons perform approximately 700 closed and open heart surgery procedures.

In closed heart surgery, a heart lung machine or bypass machine is unnecessary since no incisions are made in the heart. Our surgeons perform closed heart surgery with curative results for patent ductus arteriosus and coarctation of the aorta. Open heart surgery means the heart needs to be opened in order to repair the defect, requiring the bypass machine to be used to oxygenate and circulate the blood without using the heart or lungs. These procedures include:

• Arterial switch procedure
• Norwood procedure
• Ross procedure
• Septal myectomy

The arterial switch procedure used today to correct transposition of the great arteries was pioneered at our Hospital in the early 1980s and first performed in newborns. Our surgeons have continued to be at the forefront of a series of evolutions of the operation. Today, our Director of Pediatric Cardiac Surgery performs more arterial switches than virtually any surgeon on the eastern seaboard, with outstanding surgical outcomes compared to national averages.

Hypoplastic left heart syndrome is a complex condition in which infants are born with an undeveloped left side of the heart that cannot support circulation. A three-stage operation is required to establish circulation to that side. In the first stage, called the Norwood operation, surgeons reconstruct the heart to allow the right ventricle to pump blood to the body, and a Blalock-Taussig shunt is inserted to maintain blood flow to the lungs. When the infant outgrows the shunt, the second stage procedure, Norwood II, is required to allow more blood flow to the lungs by connecting the superior vena cava to the pulmonary artery. This reduces the volume load on the ventricle. Between the ages of 2 and 5, the final stage of the operation will be necessary. This stage involves connecting the inferior vena cava to the pulmonary artery, and placing a patch to separate the right and left atrium, and unoxygenated blood is directed into the lungs. Our Site Chief of Pediatric Cardiac Surgery has written extensively on hypoplastic left heart syndrome and its operative repair. Our results with the Norwood procedure surpass the national average, and we continue to evaluate techniques to enhance results even further.

When the aortic valve is so abnormal that it cannot be effectively repaired, a valve replacement operation may be recommended. Our surgeons perform a surgery called the Ross procedure in which the patient's own normal pulmonary valve is used to replace the damaged aortic valve. The pulmonary valve itself is then replaced with a homograft valve. The advantage of this operation is that the new aortic valve will grow with the child and the homograft (human tissue) valve, which can be large enough to allow for growth, is not subjected to high pressure and can last much longer in the position of the low pressure pulmonary valve. However, it is likely that it will eventually need to be replaced in a future operation.

Our surgeons perform septal myectomy in patients with hypertrophic cardiomyopathy when symptoms of heart failure have developed because the thickened muscle of the septum (the wall dividing the two sides of the heart) has narrowed, obstructing the blood flow from the heart. The surgery involves removing the obstructing muscle. In rare cases, the mitral valve, which connects the left atrium to the left ventricle, is replaced with an artificial valve. Septal myectomy is major, open heart surgery that requires both a hospital stay and a recovery period at home. While it is effective in controlling the severe symptoms of hypertrophic cardiomyopathy, it does not stop it from progressing, nor does it treat the abnormal rhythms associated with the condition.

Pediatric Heart Transplant Program

Our pediatric heart transplant program dates back to 1984, when the world's first successful pediatric heart transplant was performed at Columbia Presbyterian Medical Center on a 4-year-old boy with complex congenital heart disease. Since then, the program has continued to advance that proud tradition of leadership in pediatric heart transplantation and is at the forefront in the management of children after heart transplantation. Morgan Stanley Children's Hospital was the first institution in the United States to perform ABO-incompatible heart transplants (meaning that donors and recipients have different blood types) in pediatric patients. Prior to transplant surgery, physicians exchange the patient's blood, washing away any preformed antibodies that may be present. The patient also receives immunosuppressant drugs to trick the body into not making antibodies against the incompatible blood type. According to an article published in the New England Journal of Medicine, when the specific protocol is followed, survival is the same as in children who receive ABO-compatible hearts.

Our surgeons have performed over 265 heart transplants in children making NewYork-Presbyterian one of the largest and most successful pediatric heart transplant centers in North America and the world-largely due to the dedication and skill of our heart transplant team, the use of assist devices in managing heart failure, and the application of novel immunosuppression protocols. The Hospital also has a leadership role in the Pediatric Heart Transplant Study Group, which consists of 23 institutions across North America and is responsible for a significant proportion of multi-institutional research related to pediatric heart transplantation today.

When a patient no longer responds to medication, mechanical treatments or surgery for a heart condition, has persistent severe symptoms of heart failure, or suffers severe disability, a heart transplant is offered as a procedure of last resort.

Cardiomyopathy is the leading reason for heart transplantation in children. Roughly 20 percent of infants and children with symptomatic cardiomyopathy require a transplant within the first year of diagnosis. While a donor heart can cure the symptoms of heart failure and greatly improve survival, transplantation is a major operation with considerable risks.

Our Pediatric Heart Transplant Program specializes in transplanting children with end-stage congestive heart failure due to cardiomyopathy and children with complex congenital heart disease who are not candidates for palliative or corrective surgery. We have also successfully pioneered transplantations in high-risk patients who are not offered heart transplants elsewhere, including patients with severe, elevated pulmonary resistance.

With the combined expertise of the members of our pediatric cardiology team, we are able to help families learn whether they have looked at all possible treatment options before considering a heart transplant for their child. As a research center, we may be able to offer treatments unavailable elsewhere. If so, we will inform you about these alternatives and help you to determine whether or not they are appropriate for your child. If your child is a transplant candidate, we will also help you then decide if transplantation is the best option available.

Our multidisciplinary team approach has set the standard of care for children with end-stage heart failure-achieving 84 percent overall long-term survival after hospital discharge. Four pediatric cardiologists, two pediatric transplant nurses, two research nurses, an office manager, and an assistant are dedicated to serving this program and work closely with leading pediatric sub-specialists-including transplant surgeons, neurologists, psychiatrists, social workers, and physical therapists-to provide the most comprehensive care possible for complex conditions.

In addition to providing outstanding patient care, the Pediatric Heart Transplant Program stands at the forefront of pediatric heart failure research, including investigations of new strategies for post-transplant management. The director of our program, also co-founded the Pediatric Heart Transplant Study Group. Comprised of 23 institutions across North America, this group is responsible for a significant proportion of today's published research related to pediatric heart transplantation.