The pioneering Congenital Heart Disease Program at North Shore-LIJ Health System provides adults with advanced, multidisciplinary care and treatment for adult congenital heart disease. Our expert team of cardiologists, cardiac surgeons, diagnosticians, technicians and health care professionals provide state-of-the-art minimally invasive congenital heart surgery, open heart surgery, medications and ongoing monitoring, testing and follow-up.
Using the latest medical technology advances to ensure the best outcomes, we provide treatment for a wide spectrum of congenital heart disease conditions, ranging from relatively simply to highly complex, including:
Anomalous Pulmonary Venous Return (Partial and Total) Treatment
Depending on how many pulmonary veins are affected, congenital heart disease treatment and surgery are necessary to correct this congenital heart defect prior to discharge from the hospital. Anomalous return of two or three pulmonary veins often requires cardiac catheterization or cardiac magnetic resonance imaging (MRI) to confirm the anatomy prior to surgical intervention. Single anomalous pulmonary veins are likely to be completely asymptomatic, often diagnosed incidentally at older ages. Usually, no intervention is required.
Aortic Stenosis Treatment
Treatment for alleviating aortic stenosis (an obstruction of blood flow across the aortic valve) includes both interventional cardiac catheterization and surgery. Relief of obstruction can be achieved with an aortic balloon vavuloplasty. Occasionally, the aortic valve can be repaired, but most often the aortic valve needs to be replaced surgically in long term follow-up.
Aortopulmonary Window Treatment
Congenital heart surgeryis required to correct an aortopulmonary window (a defect between the walls of the aorta and pulmonary artery).
Our electrophysiology services provide comprehensive evaluations and treatment strategies for all forms of heart rhythm abnormalities. Often, at the discretion of the electrophysiologist, arrythmia can be permanently treated with ablation, a procedure that uses freezing energy (cryoablation) or heating energy (radiofrequency ablation). Other types of arrhythmias can be managed medically using pharmacologic rate and/or rhythm control.
Some patients with congenital heart disease require pacemaker and/or defibrillator implantation. Interrogation of the implanted devices is a routine part of these patients’ evaluation and can be performed in our arrhythmia clinic.
Atrial Septal Defects Treatment
Small atrial septal defects (holes in the wall between the right and the left atrium) will often close spontaneously and require no intervention. Larger atrial septal defects (ASDs) may require closure, either in the cardiac catheterizationlaboratory or with congenital heart surgery.A septal occluder device is inserted into the damaged area through a catheter. The device is then opened to effectively close the hole. The device remains in the heart permanently.
Atrioventricular Canal Defects Treatment
Atrioventricular canal defects (a large hole in the center of the heart) require surgical repair , usually by 3-6 months of age. Prior to surgical intervention, medical theraply may be employed to treat congestive heart failure symptoms and encourage weight gain. Lifelong surveillance is required due to related valve problems and possible development of left ventricular outflow obstruction.
Bacterial Endocarditis Treatment
Bacterial endocarditis is a serious infection in the heart and/or heart valves and requires a long course of intravenous antibiotics over approximately six weeks. In some cases, surgery is required to resect the damaged tissue or replace a damged valve.
Prevention of bacterial endocarditis is indicated in a subset of congenital heart defects. Your cardiologist will tell you if and when you require prophylactic antibiotics prior to other medical care including dental visits or invasive urological procedures.
Bicuspid Aortic Valve Treatment
Bicuspid aortic valves refer to aortic valves that have only two leaflets, instead of the normal three leaflets. The condition may require lifelong surveillance. In some cases, the bicuspid aortic valve needs to be replacedwith a bioprosthetic or mechanical valve.
Coarctation of the Aorta Treatment
Coarctation of the aorta refers to an obstruction in the aorta, the main artery carrying oxygenated blood to the body. Medication may be necessary to stabilize the infant prior to surgical correction. Following congenital heart disease treatment and surgery, lifelong surveillance is required. Complications may include aortic dissection or aortic aneurysm at the suture site. Patients often continue to have high blood pressure, requiring medication following repair. Surveillance with advanced imaging modalities in the adult patient is performed as a routine.
Double Outlet Right Ventricle Treatment
Double outlet right ventricle is a complex form of congenital heart disease in which the pulmonary artery and the aorta--the heart's two great vessels--arise from the right ventricle.
The type and timing of surgical repairfor this condition depends on three main factors:
- The size and location of the ventricular septal defect
- The amount, if any, of pulmonary stenosis
- The anatomic relationship of the great vessels
Ebstein’s Anomaly Treatment
A rare congenital heart defect, Ebstein’s anomaly may require surgery, but is most commonly treated conservatively with medical therapy. A small subset of patients with Ebstein's anomaly may have abnormal heart rhythms that require intervention.
Hypoplastic Left Heart Syndrome Treatment
A serious form of congenital heart disease, hypoplastic left heart syndrome treatment involves a staged surgical approach requiring multiple operations. Often, cardiac catheterization required prior to each operation to assess the child’s circulation.
Stage 1: Norwood procedure – establishment of stable pulmonary blood flow and re-creation of a new aorta.
Stage 2: Bi-directional cavopulmonary anastomosis – removes excess volume on the single systemic ventricle while establishing the first of two operations to permanently allow blue blood to go to the lungs. This is done by disconnecting the superior vena cava from the right atrium and connecting it to the pulmonary artery. The blood flows to the lungs via a passive process, dependent on low pulmonary artery pressures.
Stage 3: Fontan (operation). Completion of the Fontan represents a complete separation of the systemic venous blood from the heart. The inferior vena cava is separated from the right atrium and connected to the pulmonary artery. Occasionally, a small hole called a fenestration is placed between the conduit and the right atrium to aid in the post-operative recovery process. Later, this fenestration can be closed in the cardiac catheterization laboratory with a device (same one employed in atrial septal defect closure).
Patent Ductus Arteriosus Treatment
Patent ductus arteriosus treatment (an open structure in fetal life connecting the pulmonary artery and the aorta that should have closed spontaneously) can be performed in the cardiac catheterization laboratory or in the operating room. This decision is often made based on the size and age of the child.
Pulmonary Hypertension Treatment
Treatment for pulmonary hypertension (elevated pressure in the pulmonary artery) is provided based on the underlying cause of the elevated blood pressure in the lungs. Frequent cardiac catheterizationis often necessary to assist in titrating medications.
Pulmonary Stenosis Treatment
Treatment for pulmonary stenosis (an obstruction in the valve between the right ventricle and the pulmonary artery) is often followed serially without intervention. Occasionally, the obstruction requires an intervention, usually in the cardiac catheterization laboratory. A balloon is inserted through a catheter and placed across the stenotic valve. The balloon is then inflated, relieving the obstruction and dilating the pulmonary valve orifice.
Single Ventricle Defect Treatment
The most serious, complex types of congenital heart disease, virtually all single ventricle defects (the presence of only one functional pumping chamber) require at least one surgery. A staged surgical palliation is performed (Fontan pathway). Lifelong surveillance is required for assessment of the Fontan circuit as well as surveillance of the single systemic ventricle. Exercise stress testing, advanced imaging with cardiac MRIand occasional cardiac catheterizationis required.
Tetralogy of Fallot Treatment
A complex form of congenital heart disease involving four main heart defects, Tetralogy of Fallot usually requires at least one congenital heart surgery prior to one year of life. Lifelong surveillance is required due to the increased incidence of arrhythmia, exercise intolerance and reduced right ventricular function. Periodic assessment is warranted to assess the health of the right ventricle. Later on in life, most patients will require a pulmonary valve replacement. While usually performed surgically, newer technologies are becoming available to offer pulmonary valve placement in the cardiac catheterization laboratory.
Transposition of the Great Arteries Treatment
Transposition of the great arteries requires urgent intervention to sustain life, usually in the form of a balloon atrial septostomy. This is done via a bedside procedure, where a balloon attached to a catheter is used to create a hole between the upper chambers of the heart. Surgical correction is performed a few days later. Following congenital heart disease treatment and surgery, most children with transposition of the great arteries have a normal childhood and grow normally. Lifelong surveillance is required due to the possibility of leaky heart valves, arrhythmia, coronary arterial stenosis and other heart problems.
Tricuspid Atresia Treatment
In cases of tricuspid atresia (the congenital absence of the tricuspid valve), staged Fontan palliation is performed. Lifelong surveillance is required for assessment of the Fontan circuit as well as surveillance of the single systemic ventricle. Exercise stress testing, advanced imaging with cardiac MRIand occasional cardiac catheterizationis required.
Truncus Arteriosus Treatment
The cardiac surgery for truncus arteriosus involves:
- closure of the ventricular septal defect
- separation of the pulmonary arteries from the truncus arteriosus, and
- creation of a new conduit from the right ventricle to the pulmonary arteries
Long term follow-up is required due to the possible development of leaky valves, obstruction to right ventricular outflow and pulmonary hypertension.
Vascular Ring Treatment
A result of abnormal formation of the aortic arch and its surrounding blood vessels, vascular rings are normally treated with congenital heart surgery. Dividing the vascular ring and releasing the compression on the esophagus and the trachea relieves the symptoms. Lingering issues may be present related to underdevelopment of the trachea due to the compression.
Ventricular Septal Defect Treatment
Ventricular septal defect (VSDs) is a hole in the wall, or septum, between the two pumping chambers of the heart. Some small VSDs will close spontaneously without intervention. Others will be adequately managed with medical therapy until they become smaller on their own. Larger ventricular septal defects will require surgical treatment. Left untreated, large VSDs can cause pulmonary hypertension.