Cardiology

Pediatric Cardiac Catheterization Laboratories

The Center's pediatric catheterization laboratories perform more than 650 procedures a year. Patients with complex congenital heart disease are evaluated to determine if they are candidates for open heart surgery. Their hemodynamics and anatomy are detailed during the procedure. In addition to carrying out diagnostic procedures, studies are performed to evaluate pre-and post-cardiac and lung transplantation patients, patients with pulmonary hypertension, and patients with cardiac arrhythmias.

One of the major innovations added to the pediatric catheterization laboratories is the development of the Interventional Cardiology Program under expert direction by nationally recognized and experienced leaders in interventional pediatric cardiology. Many congenital heart lesions can now be treated in the cardiac catheterization laboratory with only one day of hospitalization, obviating the need for open heart repair. Pediatric interventional cardiac catheterization offers balloon valvuloplasty of the aortic and pulmonary valves, angioplasty of aortic coarctations, balloon valvuloplasty of distal pulmonary artery stenosis, stenting, coil closure of patent ductus arteriosus and atrial septal defect, and other advanced procedures for definitive treatment of many congenital heart defects.

The Pediatric Cardiac Arrhythmia Center is under the direction of trained pediatric cardiac electrophysiologists skilled in the evaluation and management of children with complex cardiac arrhythmias. The arrhythmia team conducts evaluations in both outpatient and inpatient settings. Patients with suspected cardiac arrhythmias or syncope undergo non-invasive diagnostic procedures, which include electrocardiograms, 24-hour holter monitors, event recorders, stress testing, and tilt table studies. Should the patient have an arrhythmia that requires further therapy, this team has an unsurpassed reputation in the medical community for performing electrophysiologic studies and radiofrequency ablation in children.

These procedures are implemented in the pediatric catheterization laboratories with an often expected result of cure of the cardiac arrhythmia, thus eliminating the need for chronic anti-arrhythmic therapy. In addition, the team manages patients who require cardiac pacemakers and automatic implantable defibrillators for the treatment of both heart block and potentially lethal ventricular arrhythmias.

Under the expert guidance of the Center's pediatric echocardiographic team, detailed evaluation of the anatomy of congenital heart lesions can now be effectively determined without invasive diagnostic procedures. The field of echocardiography has expanded and the technology is such that many patients no longer require cardiac catheterization as a diagnostic tool. Echocardiographers can now evaluate the anatomy and estimate the hemodynamics of most congenital heart lesions in pediatric patients so that they undergo open heart surgery with the echocardiogram as the definitive pre-operative assessment.

The Pediatric Cardiovascular Center uses transesophogeal echocardiogram intraoperatively to evaluate surgical results. In addition, echocardiography is used by our pediatric critical care staff to evaluate cardiac function and anatomy in the pediatric intensive care units.

Some women are at increased risk of giving birth to a baby with Congenital Heart Disease (CHD). If an abnormal fetal heart is suspected in a patient, she should be referred to a pediatric cardiologist with specialized training in fetal echocardiography. The Pediatric Cardiovascular Center offers all the specialists who may be required after the diagnosis of fetal heart disease. These include geneticists, experts in extracardiac ultrasound, fetal cardiologists, perinatologists, and pediatricians in all specialties, especially cardiology and pediatric cardiac surgery. The care and management of each patient can therefore be planned and individualized to provide the best possible outcome.

Fetal echocardiography can detect even the most serious lesions in the fetal heart from as early as 12 weeks gestation. More typically, evaluation of the fetal heart takes place between 14 and 18 weeks gestation. A single visit to one of our specialists will achieve a very detailed evaluation of a potentially high-risk patient who may be from one of the following groups:

• Those with a family history of CHD in one of the parents or in a previous child;
  

• Those who are insulin-dependent diabetics;
  

• Those who may have been exposed to cardiotoxic drugs in early pregnancy, especially anti-epileptic medications;
  

• Those whose ultrasound has revealed a malformation in any other part of the fetal anatomy.
  

The pediatric specialists at Children's Hospital of New York, the oldest and largest university-affiliated children's hospital in New York City, ensure that even the youngest patients in need of transplantation benefit from the program's integration with many specialized services.

Cardiac Transplantation
The Cardiac Transplant Program at Children's Hospital of New York was among the first to perform cardiac transplants in children and is now the largest pediatric cardiac transplantation program in the country. Since the program's inception in 1984, more than 120 heart transplants have been performed in children, some as neonates.

Through their tremendous efforts, our cardiac transplant team has had a huge impact on the lives of children who previously had no hope of survival. These include patients with cardiomyopathy and also those with congenital heart disease for whom conventional cardiac surgery no longer offered viable options. Now, many of these children can lead relatively full and functional lives.

Our transplant team consists of pediatric cardiologists, pediatric cardiac surgeons, nurses, and ancillary personnel including psychiatrists, social workers, and physical therapists, who do extensive pre-transplant evaluations in children with chronic heart failure who are under consideration for a possible heart transplant. Afterwards, long term care is provided.

Pulmonary Transplantation
The Pulmonary and Cardio-Pulmonary Transplant Program at Children's Hospital calls upon the talents of a broad range of health care specialists including pediatric surgeons, pulmonologists, immunologists, endocrinologists, nurse coordinators, intensivists, rehabilitation medicine specialists, physical therapists, psychiatrists, social workers, and financial counselors.

For children suffering from cystic fibrosis, pulmonary hypertension, or other debilitating respiratory and combined cardiac and respiratory disorders, transplantation offers a return to improved breathing, as well as the prospect of an improved quality of life.

Our multidisciplinary team evaluates each child who may be a candidate for lung or heart-lung transplantation and performs the comprehensive assessment needed to provide individualized treatment for each child and family.

Patients who require both lung and heart transplantation benefit from a joint team effort of the cardiac and pulmonary groups to coordinate care of these very sick and complex children.

The Columbia Cornell Pediatric Cardiovascular Center is one of the major facilities in the tri-state area that offers extended heart/lung life support via Extracorporeal Membrane Oxygenation (ECMO). In selected children, this therapy substitutes for the heart and lung function until the child's organs recover. ECMO may also play a role in bridging children to transplantation while awaiting a suitable donor. Physicians at the hospital participated in the earliest development of ECMO, making our facility one of the first in the world to use this life-saving modality successfully in children.

The Center's Preventive Cardiology Program offers comprehensive services for both hypercholesterolemia and hypertension. The program is administered and staffed by experts in pediatric cardiology, pediatric gastroenterology, and nutrition, all of whom oversee the care of patients with abnormalities in lipid metabolism. This includes patients with both hypercholesteremia and hyperlipidemia. They also evaluate patients with unexplained systemic hypertension.

The program primarily operates on an outpatient basis to advise families on the evaluation and management of children with these problems. However, children with severe lipid disorders, particularly with elevated cholesterol, may be admitted to the hospital for plasmaphoresis to control their cholesterol levels.

Established in 1987, the Children's Pulmonary Hypertension Center at Children's Hospital of New York is the only pediatric center in the world to focus on the evaluation and management of children with primary and secondary pulmonary artery hypertension. Children from the U.S. and other nations who are referred to the center undergo a thorough evaluation to assess the cause of the child's pulmonary hypertension. Treatment is subsequently initiated and individualized based on a therapeutic algorithm for each child.

Prior to the era of vasodilator therapy, which began in the late 1970's, most children with primary pulmonary hypertension died within one year of diagnosis. Now, with IV Prostacyclin and other agents, survival and quality of life are improved in children who acutely respond to vasodilator drug testing.

As our understanding of the pathogenesis of pulmonary artery hypertension evolves, newer strategies for its treatment are being developed and implemented. Standard therapy includes calcium channel blockers by oral administration or prostacyclin by continuous intravenous infusion. The decision to implement a particular treatment is determined by a patient's response during a comprehensive evaluation. Oxygen, digitalis, and diuretics are also used if needed. Lung transplantation is an option if medical therapy is ineffective.

It has been shown that with optimal medical and/or surgical treatment, patients are often able to return to a virtually normal lifestyle including attending school and participating in many physical activities.

We are currently involved in ongoing research to develop more efficacious and easier methods of therapeutic delivery to improve our patients' quality of life.

To serve the ever-increasing number of adults with congenital heart disease, New York Presbyterian has developed an Adult Congenital Heart Program, the first and only one of its kind in New York State. The program's team of dedicated cardiologists and surgeons work in conjunction with referring physicians to assist them in managing young adults who present with primary or post-repair congenital heart disease. Cardiac services and treatment are offered on both an inpatient and outpatient basis.

A physician who refers a patient to the Adult Congenital Heart Program becomes part of a pre-existing Columbia-Cornell team comprised of adult and pediatric cardiologists and cardiac surgeons, all of whom have extensive experience in the successful management of patients with congenital heart disease. A consulting physician works closely with this team on all diagnoses and treatment recommendations. The cardiologists document follow-up visits and reports are provided to referring physicians for their reference and continuum of patient care. This ensures the patient receives continuity of care by all involved health-care providers.

Each member of the Adult Congenital Heart Program is a member of the International Society for Adult Congenital Cardiac Disease (ISACCD). Each has access to the world renowned cardiac services and program suffered by the Columbia-Cornell Heart Institute, including the heart transplantation program, the Arrhythmia Control Center, the Syncope Center, LVAD technology, and cardiac rehabilitation. Obstetrics and gynecology (for high-risk labor and delivery), nutrition and psychiatry services are available, when necessary.

Our program is one of few that combine pulmonary and cardiac assessment in the same setting. Cardiopulmonary exercise testing, the tool of exercise physiology, is used to investigate the interactions of the heart, lungs, and muscles under conditions of external stress. Often limitations in one or more of the systems can be elicited during exercise. This information is important as it assists the patients and the physician in recommending the level of exercise in pre-and post-operative patients. It is extremely helpful in the management of patients with suspected cardiac rhythmic disturbances, and to assess the etiology of chest pain.

Frequently, these studies are used to reassure the parents that their child is completely normal and may participate in regular activity.

These tests are mostly non-invasive and often found enjoyable by the children. In the pediatric exercise laboratories, we use either the cycle ergometer or a treadmill. Most children and adolescents are familiar with one or the other of these machines, enhancing patients' cooperation.