Diagnosis and Treatment
How We Diagnose Cardiomyopathy in Children
At NewYork-Presbyterian, our pediatric cardiologists conduct a series of tests to diagnose cardiomyopathy. Depending on your child's specific condition, they may need some or all of the following tests:
- Echocardiogram including Doppler ultrasound
- Non-invasive electrophysiology studies, such as electrocardiogram (ECG or EKG), Holter monitoring, and treadmill exercise test to detect abnormal heart rhythms Cardiac catheterization to evaluate the timing and the risk of a heart transplant or to perform a biopsy of the heart muscle; this may include angiography to see the blood vessels and chambers of the heart
- Chest X-ray, CT scan, and/or MRI to show detailed images of the heart
- Genetic testing to detect gene mutations that are associated with cardiomyopathy
Treatments We Offer
Comprehensive Child-Centered Advanced Care
If your child has been diagnosed with cardiomyopathy, our expert pediatric cardiologists will tailor your child’s treatment to the type and severity of the cardiomyopathy, the prognosis of the underlying disease, family preferences and your child’s overall health. Treatment is individualized and tailored to your child’s unique needs. Our goal is to manage and monitor your child’s heart condition from diagnosis until adulthood, so that any disease progression can be treated quickly and effectively.
Medications can be very useful in treating cardiomyopathies and heart failure, especially in the early stages. Medications are also sometimes used in conjunction with surgery to correct heart abnormalities that can cause heart failure. Some of the medications currently used to treat heart failure include:
- Angiotensin-converting enzyme (ACE) inhibitors, angiotensin receptor blockers and combination angiotensin receptor blocker/neprolysin inhibitors dilate the blood vessels in the body to make it easier for the heart to pump blood forward.
- Beta blockers slow down the heart rate and lower blood pressure, which allows the heart to pump more efficiently.
- Digoxin helps the heart muscle contract better and controls the rate and rhythm of the heart.
- Phosphodiesterase-5 inhibitors such as sildenafil and tadalafil dilate the body’s blood vessels with particular focus on the blood vessels in the lungs, and also help with relaxation of the ventricles.
- Aldosterone antagonists reduce the scar tissue of the heart muscle and helps the heart muscle recover.
- Diuretics help prevent the buildup of fluid or remove fluid from the body and can help ease breathing by reducing fluid in the lungs.
- Intravenous inotropes (increases the heart’s ability to pump blood) and vasopressors (increases the blood pressure when it is too low) are used in cases of heart failure refractory to oral medication therapies.
Pacemakers and Defibrillators
Pacemakers and defibrillators are tiny devices that are implanted under your child’s skin, where they help monitor and control the heart’s rhythm. Your child may need a pacemaker or defibrillator if he or she has an certain type of abnormal heart rhythm (arrhythmia) that cannot be controlled with medication.
Procedures That Treat Arrhythmias
These procedures, also called electrophysiology procedures, use long thin tubes called catheters, which are inserted through a tiny incision in the body and guided into the heart. They measure the electrical activity in the heart’s chambers to determine the origin of an abnormal rhythm, and sometimes can correct it. Radiofrequency ablation is a type of minimally invasive treatment in which a small, needle-like probe is inserted into the scarred tissue of the heart muscle, sending out radiofrequency waves that burns away the scar tissue causing the abnormal rhythm. It can be effective for some children with cardiomyopathy that is secondary to a long-standing arrhythmia.
Ventricular Assist Devices (VADs)
Children experiencing advanced heart failure and are getting worse symptomatically despite the best oral and intravenous medications may be candidates for a ventricular assist device (VAD). A VAD is a specialized heart pump that maintains blood flow in people with serious heart failure. We currently use various devices to help left, right or biventricular heart failure in patients from infants to young adults. Our pediatric heart surgeons are at the forefront of developing and designing VADs for infants and small children as a bridge to transplantation. We also specialize in the use of durable VADs as a permanent option for patients unable to receive a heart transplant and the use of temporary devices as a bridge to recovery.
- As leaders in research into the use of mechanical circulatory support as a bridge to transplantation, we were among the first in the United States to implant a Berlin Heart EXCOR heart pump into a newborn and a Jarvik 2015 ventricular assist device in a toddler as a successful bridge to heart transplant.
- As a pioneer in the use of alternatives to transplant in pediatrics, we have successfully used the Heartmate 3 LVAD as destination therapy in several patients, including ones with Duchenne Muscular dystrophy.
- We recently became one of the first pediatric centers in the United States approved to use the Syncardia Total Artificial Heart
- We have extensive experience and expertise in the use of a multitude of permanent and temporary support devices including:
- Berlin Heart EXCOR VAD
- CentriMag and PediMag (Levitronix) continuous flow VADs
- Heartmate 3 VAD
- Jarvik 2015 VAD
- Impella temporary assist devices
National Award-Winning Extracorporeal Membrane Oxygenation (ECMO) Program
ECMO is a short-term device to support the heart and lungs in very young patients, or in children whose anatomy will not permit a ventricular assist device. Patients may also be placed on ECMO in an emergency life-saving situation. Our physicians participated in the earliest development of ECMO, making NewYork-Presbyterian one of the first in the world to successfully use this life-saving technology in children.
NewYork-Presbyterian/Columbia has been repeatedly designated a Platinum Level Award of Excellence in Life Support by the Extracorporeal Life Support Organization (ELSO), since the first year the Platinum designation was awarded.
Cardiomyopathy is the leading reason for heart transplantation in children. About one in five infants and children with a cardiomyopathy who experience heart failure symptoms will need a transplant within the first year of diagnosis. With over 35 years of experience, NewYork-Presbyterian Morgan Stanley Children's Hospital has one of the largest pediatric heart transplant programs in the United States. We consistently rank among the nation's top five centers for pediatric heart transplant.
Participation in Clinical Trials
Our pediatric cardiologists are actively engaged in research to improve the well-being of children with heart disease. A key benefit of our comprehensive program is the opportunity to offer your child the chance to participate in any appropriate clinical trials. The clinical coordinator and cardiologist will discuss these options with you. Participation in research is purely voluntary, and deciding not to participate in a study does not affect the care a child receives from our team.
The Cardiogenetics Program of NewYork-Presbyterian Morgan Stanley Children's Hospital provides genetic evaluation and ongoing comprehensive care for children and other family members with known or suspected heart conditions with a genetic cause. Examples include inherited arrhythmia syndromes (such as long QT syndrome), cardiomyopathy, Marfan syndrome, pulmonary hypertension, and congenital heart disease.