Michael Ackerman, M.D., a pediatric cardiologist at Mayo Clinic, Rochester, Minn., is an assistant professor of medicine in pediatrics and molecular pharmacology. Here Dr. Ackerman discusses issues involved in the treatment of congenital heart defects.
What health professionals care for children with congenital heart defects? What is your role as a pediatric cardiologist?
Children with congenital heart defects require a team of health professionals, with the pediatric cardiologist as the quarterback and coach. The pediatric cardiologist is responsible for the ongoing medical management of your child's heart problem. He or she orders any tests needed for diagnosis, determines the best time for procedures or surgery and coordinates follow-up visits. Other pediatric specialists may be necessary to ensure that all aspects of your child's health are monitored. Another key player is your child's pediatrician or family doctor, who will see your child for childhood examinations, immunizations and illnesses. Other players critical to a successful team include a heart surgeon and nurses and social workers familiar with the unique challenges facing your family.
Can all congenital heart defects be treated?
Most congenital heart defects can be treated, but not all can be cured. Some defects can be treated with medications and others require surgery. For simple defects like a hole in the heart, such as an atrial septal defect (ASD) or ventricular septal defect (VSD), surgery can be beneficial. In the past, this required opening the chest and heart. A new technique allows repair of ASDs and certain VSDs without opening the chest. In this procedure, a small tube is passed through your child's leg vein and guided into his or her heart. Then, a closure device known as a plug is delivered through the catheter to the defect. The plug is positioned and closes the hole, the catheter is removed and your child's heart defect is repaired.
Unfortunately, not all defects are treated as easily. Many congenital heart problems require ongoing follow-up and potentially more than one operation or catheter-based intervention. These procedures improve heart function, but they are not a cure.
Could you briefly describe the procedures used to correct or repair congenital heart defects?
A number of procedures can be used in attempts to repair and correct congenital heart defects. I have already described the treatment of simple defects like an ASD or VSD, which can be closed with a single open heart surgery or a catheter-based procedure usually done after the child reaches 3 years of age.
Several common congenital heart problems affect the cardiac valves. The four valves that separate the upper and lower chambers of the heart and control blood flow away from the heart can be either too narrow (stenosis) or leaky (regurgitation). Depending on the valve and the defect, surgical repair, surgical valve replacement or nonsurgical catheter-based balloon techniques are options for correcting the problem.
Complex congenital heart defects can be severe and life-threatening. Often there are multiple abnormalities, including a missing valve or major artery. In some cases one of the two pumping chambers of the heart is poorly developed and does not function. Children with such defects may need several open heart operations to redirect the blood flow and convert the heart into a single, functional pumping chamber capable of sustaining life.
Do some children outgrow their heart problem? Do some defects not show up until later in life?
Yes, some children may outgrow their congenital heart defect. For example, a child may be born with a small ASD or VSD that closes on its own during the first few years of life. However, a child will not outgrow significant holes or abnormal connections between the chambers of the heart or the large vessels leaving the heart.
Although all congenital heart problems are present at birth, the consequences of some defects may not show up until later in life. For example, high blood pressure is unusual in a child or teenager. If it is detected, further evaluation may find a significant narrowing in the main artery of the child's heart that carries blood to the rest of the body. This is known as a coarctation of the aorta. In all likelihood the coarctation was present at birth, but it was not detected until later in life. Similarly, a narrow or leaky valve may not cause significant problems until later in life.
What factors determine that surgery will be performed within days or weeks of birth versus later in childhood?
Newborn surgery is necessary if the defect could cause death or severe damage to the heart or other organs if surgery were delayed. Careful evaluation by the pediatric cardiologist, cardiac surgeon and the congenital heart team is necessary to best determine the optimal timing for surgery.
What problems can develop if a congenital heart defect is not corrected?
Depending on the specific defect, the consequences of not correcting a congenital heart defect could include early death, a gradual loss of the heart's pumping ability (congestive heart failure), a gradual decrease in the amount of oxygen in the blood (cyanosis), increased blood pressure in the lungs (pulmonary hypertension) and poor growth. After these complications develop, successful repair of the defect may not be possible.
How should parents explain a heart problem or need for heart surgery to their child?
Try to address these issues at an age-appropriate level. Children's hospitals typically have programs to acquaint children and their parents with the hospital and the procedures they need to undergo. Most hospitals also have child-life specialists who are trained to help children through the hospital experience. If your child has already undergone some diagnostic procedures, a relationship with a child-life specialist or pediatric cardiology nurse likely has been established. Other resources may be found at public libraries or on the Internet. Ask your child's doctor about the availability of additional resources and for assistance in explaining the tests or surgery to your child.
What should parents expect in the immediate hours and days after surgery?
After open heart surgery, your child is cared for in an intensive care unit (ICU) by nurses with special training in pediatric heart disease and by a team of doctors that includes the heart surgeon, pediatric cardiologist and anesthesiologist. Other health care professionals involved in postoperative care include respiratory therapists and child-life specialists. You may have the feeling that doctors, nurses and other members of the team are always at your child's bedside. Be assured that the size of the team does not mean your child is doing poorly. The number of caregivers will decrease as your child recovers from surgery.
In the first hours and days after surgery, many tubes and wires will connect your child to various pieces of equipment. A breathing machine (ventilator), which ensures adequate oxygen intake, will be connected through a tube (endotracheal tube) in your child's mouth. Multiple intravenous (IV) lines will come from the neck, arms and groin region. In addition, there will be tubes draining fluid from the chest and a tube draining the bladder (Foley catheter). As your child recovers, the first tube to be removed is usually the breathing tube, and the other tubes and IVs are disconnected when they are no longer needed.
What follow-up care is needed after successful surgery?
After successful repair of a simple defect such as a hole in the heart or an extra artery (patent ductus arteriosus), your child will see the pediatric cardiologist 2 to 4 weeks after leaving the hospital and then perhaps at 6 months and 12 months. After that, routine visits to your family physician or pediatrician are all that should be necessary. If your child is scheduled for dental work in the first 6 months after surgery, an antibiotic will be prescribed to prevent infection (endocarditis) at the site of heart repair.
Children who have more complex and serious heart defects will need regular follow-up with a pediatric � and later, an adult � cardiologist who has expertise in treating patients with congenital heart defects. Several tests of heart function, such as an electrocardiogram (ECG), chest X-ray and echocardiogram, will be done periodically to monitor your child as he or she grows. Special procedures like cardiac catheterization may be necessary for more in-depth evaluation or treatment. Your child's cardiologist and surgeon determine the frequency of check-ups and the specific tests needed.
Do some children need cardiac medications after surgery?
If the cardiac surgery is for a simple defect, medications are often not necessary following surgery. One exception would be a recommendation that your child take an antibiotic before dental work or other invasive procedures at least in the first 6 months after surgery.
When the heart surgery repairs or corrects more serious defects, medications are often necessary to help the heart perform optimally. Typical heart medications for a child with a complex defect might include medications that:
Ensure a regular heartbeat Increase the strength of the heart's contractions, such as digoxin (Lanoxin) Decrease the amount of fluid in circulation, thereby reducing the volume of blood that must be pumped, such as the diuretic Lasix Relax the arteries, thereby decreasing resistance to blood flow, such as afterload-reducing agents like captopril, enalapril and lisinopril
What is the long-term outlook for children with treated congenital heart defects?
Many children with congenital heart defects lead healthy, productive and active lives. Because the scope of congenital defects is very broad, you should discuss your concerns with your child's primary pediatric cardiologist. It is important to have regular discussions with your cardiologist. The long-term outlook may change depending on your child's heart problem and advances in treatment.