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Cardiac Surgery

Cardiomyopathy, Heart Failure, and Transplantation Program

Linda J. Addonizio, MD

Marc Richmond, MD

We would like to welcome you and your family to the Comprehensive Pediatric Cardiomyopathy, Heart Failure, and Transplantation Program of Morgan Stanley Children's Hospital. Our program offers state-of-the-art pediatric cardiovascular care, beginning with a comprehensive evaluation that allows us to understand your child's heart problem fully. We take great pride in providing individualized management for our patients and their families. We recognize that family-oriented, comprehensive care and follow-up are critical to achieving a successful outcome. We understand that parents and caregivers are key members of the child's healthcare team, and we work closely with you and with your child's referring physicians to implement the best plan of care.

Our treatment plan takes advantage of the most advanced medical therapies, as well as cutting-edge interventions in cardiology and open-heart surgery procedures, including cardiac transplantation. Our 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, our Pediatric Heart Transplant Program has continued to advance that proud tradition of leadership in pediatric heart transplantation. Our surgeons have performed over 265 heart transplants in children making NewYork-Presbyterian one of the largest pediatric heart transplant centers in North America and the world.

The goal of our program is to return children to their childhood. We provide our young patients and their families with the support and treatments necessary to lead as normal a life as possible. Please ask questions of anyone, at any time.

The Center for Pediatric Cardiomyopathy, Heart Failure and Transplantation gratefully acknowledges the Children's Cardiomyopathy Foundation (CCF) for its key role in the establishment of this multi-disciplinary comprehensive care program. With the support provided by CCF, the members of the center have been able to realize their goal of improving the lives of children with this rare disease.

The Children's Cardiomyopathy Foundation (CCF) is a national nonprofit organization focused on pediatric cardiomyopathy. CCF was founded in 2002 by a New Jersey parent whose two young children were affected by the disease. In recognition of the need for more research and better support, CCF was established with the purpose of stimulating and funding research on pediatric cardiomyopathy, educating and assisting physicians and patients, and increasing awareness and advocacy related to the needs of affected children and their families.

Today, CCF reaches out to more than 1,800 physicians and 350 families worldwide. As the only public interest group focused on pediatric cardiomyopathy, CCF remains committed to accelerating the search for a cure while improving diagnosis, treatment, and quality of life for children with cardiomyopathy. In terms of research, CCF provides seed funding for basic and clinical studies, supports the establishment of comprehensive care programs, and sponsors scientific think tanks in association with the National Institutes of Health-funded Pediatric Cardiomyopathy Registry. CCF also takes a leadership role in the development of a biologic specimen repository and in the planning of scientific workshops on pediatric cardiomyopathy.

To increase the visibility of the disease, CCF actively works with professional medical societies and healthcare companies as well as the National Heart, Lung, and Blood Institute, the American Heart Association, and the National Organization for Rare Disorders. For families, CCF provides easy-to-read patient materials, family matching, referrals to physicians and clinical studies, a newsletter, a list serve, and a website with comprehensive medical and care information. By providing information, resources, and support, CCF hopes that parents will be better equipped to deal with their child's diagnosis and empowered to seek the best medical care.

Our Expertise

A Dedicated Team

The Center for Pediatric Cardiomyopathy, Heart Failure and Transplantation calls on the expertise of a wide range of pediatric cardiologists, pediatric cardiac surgeons and health professionals with expertise in pediatric cardiology and heart transplant. Your child will be cared for by a multidisciplinary team of which parents are key members. Only you can provide important information on how your child was prior to and after becoming ill. You can also help other members of the team shape your child's personalized treatment program based on your child's physical, emotional, and practical needs, and make final decisions regarding your child's care. In addition to parents, the centers team includes:

• Pediatric cardiologists
• Pediatric cardiac surgeons
• Medical consultants, including pediatric electrophysiologists, neurologists, and endocrinologists
• Nurse specialists in cardiology and transplant
• Research nurses
• Genetic counselor
• Pediatric cardiology social workers
• Child psychiatrists
• Child life specialists
• Physical therapists
• Pharmacist

Conditions We Treat

The Center for Pediatric Cardiomyopathy, Heart Failure and Transplantation provides state-of-the-art care for children with complex congenital heart disease and severe cardiac disorders, including:

Cardiomyopathy
Cardiomyopathy is any disease of the heart muscle in which the heart loses its ability to pump blood effectively, preventing the heart muscle from pumping enough blood to meet the bodys needs. In some instances, heart rhythm is disturbed, leading to irregular heartbeats, or arrhythmias. There may be multiple causes of cardiomyopathy, including viral infections. Sometimes, the exact cause of the muscle disease is never found. Cardiomyopathy, which tends to be progressive and sometimes worsens fairly quickly, is a leading cause for heart transplantation.

Dilated cardiomyopathy is the most common form of cardiomyopathy. The heart muscle becomes enlarged and stretched (dilated), causing the heart to become weak and pump inefficiently. This can result in irregular heart rhythms, risk of blood clots, and congestive heart failure.

Hypertrophic cardiomyopathy involves abnormal enlargement of the muscle mass of the left ventricle of the heart or the wall between the two ventricles becomes enlarged. These abnormalities obstruct blood flow from the left ventricle or cause a leaky valve, allowing blood to move backwards from the left ventricle into the left atrium, instead of forward to the body.

Restrictive cardiomyopathy is a rare condition in which the heart muscle becomes stiff and cannot fully expand to allow enough blood to enter its lower chambers. Because the ventricles cannot expand fully, blood that would normally enter the heart backs up in the circulatory system leading to heart failure.

Diagnostic Services

Many different tests may be used to evaluate your child's heart condition and present you with appropriate treatment options. Depending on your child's specific condition, he or she may need some or all of the following tests:

Non-Invasive Procedures

Chest X-rays use a small amount of radiation to take a picture of the heart and lungs. They show the size and shape of the heart, the major blood vessels in the chest, whether there is any fluid present, and any abnormalities in the lungs.

CT Scans (CAT or computed tomography) are special X-rays that create two-dimensional, cross-sectional images of the body. These images help reveal how well the heart is working and may reveal additional information about the structure and function of the heart and blood vessels.

Doppler ultrasound is sometimes taken in conjunction with an echocardiogram, and provides color-coded images of blood-flow within the heart. It can also help to reveal an obstruction or leaky valve.

Echocardiogram is a very informative non-invasive test for determining the degree of dysfunction in the heart muscle and for assessing the progression of disease. An echocardiogram uses high-frequency sound waves to produce moving images of the beating heart on a video screen. These cross-sectional views of the heart allow the cardiologist to measure the muscle thickness, size, pumping ability, degree of obstruction, chamber size, and valve movement. Pressures inside the heart chambers and major vessels can also be indirectly determined.

Electrocardiogram (ECG or EKG) measures the hearts electrical activity via electrodes placed on the child's limbs and chest wall. The ECG records the hearts rate and rhythm, and can indirectly provide information about the size of the child's heart.

Exercise stress test monitors a patients heart while the patient is exercising, usually while pedaling a stationary bike. The child's blood pressure, heart rhythm, and breathing are measured during different degrees of exertion.

Holter monitor is an external device, similar to an ECG, worn by the patient for 24-72 hours while the device continuously records the heart rhythm. This report is used to identify any irregular heart rhythms.

Magnetic Resonance Imaging (MRI) uses waves from a large magnet to generate a three-dimensional image that allows doctors to evaluate heart and blood vessel anatomy, function, and flow dynamics.

MUGA scan (Multiple Gated Acquisition or radionuclide ventriculogram) is similar to an echocardiogram, but offers a more accurate measurement of how well the heart functions as a pump. It measures how much blood the heart pumps, or ejects, with each contraction and how quickly that blood is ejected.

Pulmonary function test measures lung function. A child breathes into a tube attached to a measuring device that shows how well the lungs work and how well oxygen travels through the body.

Myocardial Perfusion Test allows doctors to see how the blood flows through the heart muscle. A radioactive substance that only travels to the heart muscle cells receiving blood is injected to identify areas of the heart that are at risk for a heart attack. The test is usually performed in two stages, one during exercise on a treadmill and one at rest. This allows doctors to see how blood flows through the heart while working hard (on the treadmill) and when resting. Aside from the injection, this is a non-invasive test. The imaging can take up to 25 minutes. However, because it takes time for the substance to circulate, the entire test may take upwards of five hours.

Ventilation perfusion scan measures the flow of air and blood in the lungs. A radioactive substance is used to track the flow of blood through the lungs.

Catheterization Procedures

Cardiac catheterization involves inserting a thin, flexible, plastic tube through tiny incisions in the skin into a blood vessel (often in the groin area) and threading the catheter into the heart or the coronary arteries. Catheterization can be used for a range of diagnostic and therapeutic procedures, including measuring the blood pressure within the heart, the amount of oxygen in the blood, and the pumping ability of the heart muscle. Catheterization can help the physician decide the timing and the risk of a heart transplant, or determine if any viable treatment alternatives exist.

Angiography examines the blood vessels and chambers of the heart. Dye that is visible on an X-ray is injected through a catheter. The flow of the dye is followed on a video monitor.

Endomyocardial biopsy (or heart muscle biopsy) is helpful in monitoring diseases of the heart caused by infection, inflammatory processes, and structural abnormalities of the heart muscle. A catheter is used to remove a small piece of heart muscle tissue for laboratory analysis. The biopsied tissue can also be used to assess and monitor rejections in heart transplant recipients.

In some instances, a skeletal muscle biopsy (a tiny piece of muscle taken from an arm or leg) may be required. Skeletal muscle biopsies are used to test for specific neuromuscular diseases associated with cardiomyopathy, such as Duchene muscular dystrophy. This procedure does not require catheterization.

Electrophysiology study (EP study) tests for abnormal electrical conduction pathways, susceptibility to arrhythmias, the effectiveness of anti-arrhythmic drugs, and the need for an implantable cardioverter defibrillator. The study uses fine wires inserted through veins in the groin area and threaded to the hearta similar approach to cardiac catheterization. Electrical stimuli are applied to these wires to induce a very fast heart rhythm, which is then used to map the hearts electrical system.

Research

Molecular Diagnostic Laboratory

The Molecular Diagnostic Laboratory performs research to identify the underlying genetic basis for inherited susceptibilities to cardiomyopathy, as well as investigates genetic and environmental determinants for response to therapy, prognosis, and risk of arrhythmias. Several projects are ongoing and include:

• Identify new genetic etiologies for cardiomyopathy in children.
• Characterize the type and frequency of mutations in gene associated with cardiomyopathy in children of different ages.
• Identify genetic factors that modify the severity and progression of ventricular dysfunction and arrhythmias in children with cardiomypathy.

Pediatric Heart Transplant Study Registry

The primary purpose of this study is to identify risk factors for certain outcomes (mortality, retransplant, coronary artery disease, rejection, and infection) in pediatric heart transplant patients in order to advance the state of knowledge regarding pediatric heart transplant. All patients less than 18 years old who are listed for cardiac transplant with UNOS through our institution are enrolled in this registry.

Pediatric Cardiomyopathy Registry

The Pediatric Cardiomyopathy Registry is an ongoing registry funded by the National Institutes of Health and the National Heart, Lung, and Blood Institute. Its purpose is to describe the epidemiologic features and clinical course of selected cardiomyopathies in patients aged 18 years or younger and to promote the development of etiology-specific treatments.

Our Team

Medical Director, Children's Transplantation,
Morgan Stanley Children's Hospital
Medical Director, Cardiomyopathy, Heart Failure and Transplantation Program

Linda J. Addonizio, MD