The outlook for babies born with a heart defect has improved dramatically over the past 50 years. Today, nearly 1 million babies born with a significant heart defect will survive into adulthood to enjoy a fulfilling life. For those with minor defects, the heart may adapt and compensate for the abnormality in its structure. However, for the majority of patients these adaptations are not sustainable and treatment eventually is required at some point.
Treatments are generally categorized as either "corrective" or "palliative." Palliative treatments improve the functioning of the heart and aren't geared toward actually correcting the defect. Palliative treatments often revolve around noninvasive procedures like the long-term use of medications but may also entail catheterization or surgery. Corrective treatments seek to repair the defect through invasive procedures like heart catheterization or surgery.
Deciding when to undergo an invasive procedure involves balancing competing risks. As a general rule, physicians try to delay invasive treatment for as long as possible because all invasive procedures put the patient at some level of risk. Furthermore, many repairs are not permanent, so delaying treatment may eliminate the need for additional procedures over the course of a lifetime. On the other hand, if treatment is delayed too long, irreversible damage can occur. Overall, about half of the children requiring a surgical repair undergo surgery before age 2. And many of these individuals may undergo three or more operations or catheterizations over the course of their lives.
Generally the best place to seek out treatment is a medical center that specializes in congenital heart disease, usually called congenital heart centers or clinics. They tend to offer the most up-to-date treatments and be staffed by doctors who are highly experienced at performing those procedures. Before choosing a cardiologist or cardiac surgeon for a procedure, it is important to make sure that the doctor is experienced at treating the defect. The best way to evaluate surgeons' skill level is to ask how many times they've performed the procedure overall, and how many times they've done so within the last year. Good doctors will not be offended by these questions and should readily share answers. A second opinion can be very helpful as well, especially in situations in which the defect and proposed treatments are complex.
Still, no treatment or correction is a cure for congenital heart disease. No matter the treatment goals, lifelong management of the disease is required.
This section has more information on:
- Monitoring and observation
- Heart catheterization
- Heart surgery and transplant
- Treatment options, by type of heart defect
Monitoring and observation is often the first line of treatment for many people whose heart defect(s) are not severe and do not cause significant symptoms. For example, a person with a bicuspid aortic valve may be monitored for several years until symptoms develop and additional treatment is recommended. Monitoring involves regular checkups and evaluation of the defect by a cardiologist who specializes in congenital heart disease. Eventually most congenital heart defects will need treatment beyond monitoring, so it is critical for patients to continue seeing their cardiologist on a regular basis even if, for the time being, they are not receiving other treatments.
Medications are prescribed to improve the functioning of the heart, control symptoms, maximize heart function, maintain stamina, and help prevent future complications such as hypertension or endocarditis (an infection of the heart). Medications commonly prescribed to people with congenital heart defects include:
- Angiotensin-converting enzyme (ACE) inhibitors block the enzyme in the body that causes blood vessels to tighten. The net result is that the blood vessels relax, decreasing the resistance to blood flow or blood pressure. This reduces the workload on the heart.
- Antiarrhythmics alter the way in which electrical currents are transmitted through the heart muscle. The heart pumps most efficiently when it beats in a regular rhythm; antiarrhythmics are used to regulate the heart rhythm.
- Antibiotics are used to prevent endocarditis, a potentially fatal infection in the heart for people with congenital heart disease. Read more about endocarditis in the section on managing congenital heart disease.
- Anticoagulants, commonly referred to as blood thinners, reduce the blood's ability to form clots by either reducing proteins involved in coagulation or changing the way the clotting proteins work. Some congenital defects, especially those involving a heart valve, can increase the likelihood of an individual developing a blood clot; therefore these patients receive anticoagulants to manage this risk. All patients with a mechanical heart valve need to take anticoagulants as well.
- Beta-adrenergic blocking agents, or beta-blockers, slow the heartbeat, lower blood pressure, and decrease the squeezing strength of the heart muscles by reducing the affect of adrenaline on the heart. In effect, these drugs reduce the heart's demand for oxygen and decrease the heart's workload.
- Calcium channel blockers keep calcium from entering the heart muscle and blood vessels. This has the effect of slowing the heart rate, reducing the squeezing strength of the heart muscle, decreasing blood pressure, dilating coronary arteries, and helping normalize fast or irregular heartbeats.
- Digitalis, or its derivative, digoxin, is an inotropic agent that improves the squeezing strength of the heart and helps regulate the rate and rhythm of the heartbeat. These medications are used to improve blood circulation and help reduce swelling of hands and ankles.
- Diuretics reduce the amount of water and salt in the body, thereby reducing swelling in the hands and ankles as well as the workload on the heart.
- Vasodilators act directly on the muscles of the blood vessels, causing them to relax. This reduces blood pressure and the workload on the heart.
More detailed information on dosages and contraindications for each of these types of drugs can be found on the National Institute of Health Medline website.
Cardiac catheterization, once solely a diagnostic tool, can effectively treat some forms of congenital heart disease including atrial septal defects, coarctation of the aorta, and pulmonary valve stenosis. These defects can now be treated with this minimally invasive procedure under a local anesthetic. Cardiac catheterization eliminates the need to open up the chest, dramatically shortening recovery times for patients.
Catheterization techniques can be corrective or palliative. Corrective procedures include using a catheter to close patent ductus arteriosus, or patch a septal defect. Palliative procedures include using balloon dilation to widen a constricted area that may be associated with valve stenosis or regurgitation, or coarctation of the aorta. More information on heart catheterization can be found under Heart Catheterization in the Testing section.
Cardiac operations can be either corrective or palliative. Examples of corrective procedures include repairing or replacing heart valves, relocating the major blood vessels of the heart to their correct position, or patching a septal defect. Palliative procedures are generally performed to improve circulation. For instance, a surgeon may reroute, or shunt, an artery that normally supplies blood to the arm to the lungs instead, as in the Blalock-Taussig procedure. This procedure allows the blood to pick up more oxygen before circulating to the rest of the body. Another example of a palliative surgery is placing a constricting band on the pulmonary artery to decrease blood flow to the lungs.
In most heart operations, the surgeon makes an incision in the center of the chest to operate on the heart. Less-invasive incisions can also be made on the side of the chest when appropriate. A cardiopulmonary bypass machine is usually needed to take over the functions of the heart and lungs during the procedure on the heart. Once the procedure is completed and the surgical team is satisfied that the heart is beating strongly, the cardiopulmonary bypass machine is disconnected and the chest incisions are sutured closed. Heart operations usually last two to four hours, but complex procedures may last longer. After heart surgery, all patients are moved to a surgical intensive care unit (ICU) to begin the recovery process.
Recovering from heart surgery usually takes six to eight weeks before patients can return to their daily routine. The first five to seven days are spent in the hospital, starting in the intensive care unit. In the ICU, the patient's condition is constantly monitored using several surgically inserted tubes and drains to record fluid uptake and loss as well as assist with breathing. Sleeping in the ICU can be difficult for patients; visitors may find this environment distracting. It's not uncommon for patients to appear pale or swollen, or even become temporarily confused during this stage of recovery. Once the patient's condition has improved, most of the tubes and drains will be removed and the patient will be moved out of the ICU to complete the hospital stay in a bed equipped for continuous cardiac monitoring.
Before discharge from the hospital, members of the healthcare team will discuss the at-home recovery plan with the patient. Written instructions for diet, pain management, incision care, physical therapy, medications, and followup appointments will be provided. Activity restrictions including driving, heavy lifting, and showering will also be covered. Pain management is an important aspect of recovery, allowing patients to perform simple functions such as coughing, breathing deeply, and walking that are important steps to recovery.
Emotional responses to heart surgery vary widely due to the stress of operation and medication. These events may trigger feelings of denial, distress, fear, anger, and even depression. For most people, these feelings are temporary; however, if they persist, discuss them with a member of the healthcare team.
Heart transplants are occasionally needed for babies born with multiple defects that are too complex to repair or for older children and adults whose hearts have experienced irreparable damage. Although the procedure itself is straightforward for an experienced surgeon, recovering from a heart transplant involves significant medical monitoring and followup care. Heart transplants require a lifelong commitment from the patient to daily medications, regular medical visits, and a healthy lifestyle.
This section contains information on treatments for 13 congenital heart defects:
- Aortic valve stenosis
- Atrial septal defects (ASD)
- Coarctation of the aorta
- Congenitally corrected transposition of the great arteries
- Ebstein's anomaly
- Eisenmenger's syndrome
- Hypertrophic cardiomyopathy
- Hypoplastic left heart syndrome
- Patent ductus arteriosus
- Pulmonary valve atresia
- Pulmonary valve stenosis
- Tetralogy of Fallot
- Valve regurgitation: Aortic, mitral, pulmonary, or tricuspid
- Ventricular septal defects (VSD)
Stenotic aortic valves need to be repaired or replaced before irreversible damage to the heart occurs. The timing of repair or replacement is critical because once the symptoms of heart failure develop--chest pain, fainting, shortness of breath--survival is limited to two or three years if the condition is left untreated.
Typically the aortic valve is replaced after the symptoms develop, which may occur in infancy or adulthood. In rare instances, aortic valve replacement is recommended in patients who have not yet developed symptoms but have very tight valves. In any case, the aortic valve can be surgically repaired or replaced in almost all patients, from children to otherwise healthy people in their 80s.
Infants who develop symptoms of aortic stenosis need treatment early on in life and often follow a two-stage treatment plan starting with balloon valvuloplasty. Balloon valvuloplasty is a heart catheterization procedure where a small tube equipped with an inflatable device is inserted inside the heart where it is dilated to mechanically widen the valve. This procedure is usually performed to delay surgical replacement of the valve until the infant has time to reach childhood, when a normal-sized valve can be used for replacement. When necessary, surgeons can replace valves in infants, but it's preferable to wait until the infant reaches childhood.
When stenosis occurs in the older child or adult, it frequently is associated with a bicuspid aortic valve. For these patients, replacement may not be necessary until the stenosis worsens or leakage develops. Medications may be used to delay replacement and help alleviate some of the symptoms of stenosis. Digitalis may be prescribed to augment the contraction of the ventricle. Diuretics, vasodilators, or antiarrhythmics may be prescribed to reduce the workload on the heart, and/or regulate the heart rhythm. In this situation, the condition of the valve and function of the heart are carefully monitored with regular checkups to ensure that the valve is replaced at the appropriate time before the left ventricle is irreversibly damaged.
Valve replacement requires heart surgery. The surgeon removes the faulty valve and replaces it with an artificial one--either a mechanical prosthetic valve or a bioprosthetic valve. Approximately 95 percent of all valve replacements are performed on mitral or aortic valves. Occasionally, the surgeon will need to use a complex procedure when repairing or replacing the valve to enlarge the aorta and/or part of the left ventricle leading to the aortic valve to accommodate an adequate-sized valve. The key to selecting the right prosthetic valve involves weighing the advantages and disadvantages of certain valves with the risk of anticoagulation therapy.
Mechanical prosthetic valves, made of synthetic materials, are more durable than tissue valves but require a blood-thinning drug, like warfarin, to be taken for the rest of an individual's life. People who take these drugs must have their blood tested regularly to ensure that their blood has not gotten too "thick" or "thin." This constant monitoring can be especially challenging in children. In addition, blood thinners are not recommended for people who play contact sports and pose risks for a developing fetus. Women who wish to have a pregnancy or young adults who wish to participate in contact sports should discuss in detail the choice of valves with the congenital cardiologist and surgeon.
Bioprosthetic valves do not require blood thinners; however, they are not are not as durable as mechanical valves and may require replacement within 10 years. In general, patients younger than 70 receive mechanical valves; those over 65 receive bioprostheses. For some younger people, a bioprosthetic valve may be used with the understanding that an additional surgery will be necessary in the future to replace it with a mechanical valve.
Individuals who have a small atrial septal defect, a hole in the atrial septum, without symptoms or heart rhythm disturbances may require only periodic monitoring to ensure that the right side of the heart is not enlarging or that pulmonary hypertension does not develop. A typical evaluation would include a thorough physical exam, electrocardiogram, chest X-ray, echocardiogram, and an exercise test. A Holter monitor or event recorder may be necessary to check for irregular heart rhythms. More details about these tests can be found in the Testing section.
Larger ASDs require catheterization or surgery to close the opening between the atria. Repairing the ASD before elevated blood pressures develop in the artery of the lungs, a condition known as pulmonary hypertension, is critical for these patients. Once pulmonary hypertension develops, it usually is not reversible and closing the defect may actually exacerbate the patient's condition.
Historically, the opening has been closed with a heart operation, preferably before age 6. Surgical closure is very effective, having been performed for over 35 years, and is considered very safe; however, minimally invasive techniques have been developed that are just as effective with shorter recovery times. Today, most ASDs are closed by cardiologists using a heart catheterization technique. During this procedure, the cardiologist inserts a patch that resembles a small umbrella into the damaged area of the atrial septum. The cardiologist enters the heart using a catheter that enters the body through a blood vessel in the arm or leg. More information on heart catheterization is under Heart Catheterization in the Testing section.
People who do not have the ASD repaired usually die of heart failure before age 50. In contrast, long-term survival rates after a successful repair of this defect is similar to that of a healthy person of the same age who never had the defect. Continued medical monitoring including regular echocardiograms is required to ensure that the repair remains effective.
For a minority of patients who have no symptoms and a relatively mild obstruction, regular observation and monitoring by a cardiologist with expertise in congenital heart disease may be all the treatment that is necessary for coarctation of the aorta, an abnormally narrow aorta. A typical evaluation would include a thorough physical exam, electrocardiogram, chest X-ray, echocardiogram, and an exercise test. An MRI or CT scan may be necessary to get a more detailed look at the narrowed portion of the aorta. A Holter monitor or event recorder may also be used to check for irregular heart rhythms. More detail about these tests can be found in the Testing section.
Most people however, need treatment to survive beyond their mid-30s. Heart surgery has been the standard treatment for coarctation of the aorta for over 35 years; however, heart catheterization techniques are now available as well. Selecting a procedure depends on many factors, including the patient's overall health, the severity of the symptoms, as well as the size, location, and degree of obstruction. Catheterization is used for selected patients whose anatomy is favorable and who wish to avoid an operation. For some, heart surgery remains the preferred treatment option.
Catheterization, using balloon angioplasty, is a treatment alternative for some people. Once the catheter is in place, an inflatable balloon device is inserted into the narrowed segment of the aorta. The device is inflated to mechanically widen the aorta and improve blood circulation. In some instances, a small metal tube called a stent may be placed in the narrowed area after the dilation to help maintain the blood flow pathway. It is not unusual for a surgical repair performed in childhood to need to be redone in adulthood with coarctation of the aorta.
Surgeons usually use one of three general procedures to repair a coarctation depending upon the individual patient's anatomy in relation to the section of the aorta involved in the defect. An end-to-end anastomosis involves removing the narrowed section of the aorta and then reconnecting the two ends. Other repairs involve opening the aorta and placing a Dacron patch to widen the narrowed area: This is called a patch augmentation procedure. Alternatively, a tube graft can be used to replace the narrowed portion of the aorta with a Dacron tube. Occasionally, the surgeon may bypass the narrowed section completely by connecting a tube from the ascending aorta above the aortic valve to the descending aorta beyond the coarctation. This is known as an ascending-to-descending aortic bypass graft.
Although medications cannot widen the aorta, they are commonly prescribed to treat hypertension associated with coarctation of the aorta. Residual hypertension is very common even after an excellent surgical repair. ACE inhibitors, vasodilators, beta-blockers, or calcium channel blockers are frequently used. More detail can be found in the section on Medication.
For people without heart rhythm disturbances or any other symptoms relating to congenitally corrected transposition of the great arteries arteries, regular observation and monitoring by a congenital cardiologist may be all that is needed. A typical evaluation would include a thorough physical exam, electrocardiogram, chest X-ray, echocardiogram, and an exercise test. A Holter monitor or event recorder may also be used to check for irregular heart rhythms. More detail about these tests can be found in the Testing section.
Treatment for congenitally corrected transposition of the great arteries is highly individualized. Abnormally slow heart rhythms, which are common in these patients, are treated by implanting a pacemaker. Medications such as digitalis and antiarrhythmics may be prescribed to improve the pumping capabilities of the right ventricle and reduce the effects of a leaky valve when necessary. In some patients, surgery may be needed to replace valves where regurgitation develops. This should be performed before the ventricle becomes weak and before patients develop significant symptoms. More information is available in the section on Treatment for Valve Regurgitation Valve Regurgitation.
Depending upon individual circumstances, treatment and prognosis can vary widely with Ebstein's anomaly, an abnormal tricuspid valve. Some people with Ebstein's anomaly live into their 60s and 70s with no treatment despite significant abnormalities in their tricuspid valves; others die in infancy. For people without symptoms, monitoring and observation including a thorough physical exam, electrocardiogram, chest X-ray, echocardiogram, and an exercise test at regular intervals is necessary. A Holter monitor or event recorder might also be used to check for irregular heart rhythms. More detail about these tests can be found in the Testing section.
If symptoms develop, treatment is recommended to avoid future complications, in particular an enlarged heart, as well as to help control symptoms. Medication is usually the first line of treatment and may include diuretics to decrease swelling, minimize shortness of breath, and improve activity levels.
Abnormal heart rhythms are common in people with Ebstein's anomaly and may require electrophysiology studies to map out abnormal electrical conduction pathways. Most often these problems can be controlled by antiarrhythmic medication, heart surgery, catheter-based intervention, or a combination of treatments.
Surgery may be necessary to repair or replace a faulty tricuspid valve that is regurgitating blood or close the atrial septal defect, if present. More information is available in the section on Treatment for Valve Regurgitation or the section on Atrial Septal Defects. Surgery is usually postponed until the heart becomes enlarged, but should be performed before the right ventricle becomes severely enlarged and poorly functioning and before patients develop severe symptoms of shortness of breath. Heart rhythm problems should be evaluated prior to surgery, to identify abnormal electrical pathways that may be affected by the procedure.
Treatment of Eisenmenger's syndrome focuses on controlling the symptoms and preventing complications that may occur from the combination of pulmonary hypertension with the abnormal blood circulation patterns in the heart. In pulmonary hypertension, elevated blood pressures develop only in the blood vessels of the lungs. Once Eisenmenger's syndrome develops, corrective surgery is not recommended and may even be detrimental. Repairing the hole in the heart would likely result in dangerously high blood pressure in the heart, due to the elevated pressures in the lungs. These high pressures in the heart can cause it to enlarge and eventually fail.
The primary treatment options for patients with Eisenmenger's syndrome are medications used to control rhythm disturbances, optimize heart-muscle contraction, and reduce fluid buildup in the legs, abdomen and lungs. Occasionally, pulmonary vasodilators, medications that dilate the arteries in the lungs, have been successful at reducing the symptoms of Eisenmenger's syndrome. However, many of these medications can also affect blood pressure, fluid and electrolyte values, and heart rate. For a patient with Eisenmenger's syndrome, rapid changes in blood pressure, fluid volume, or pulse rate can exacerbate the condition and result in dangerously low levels of oxygen in the blood. Any medication that could lower blood pressure needs to be carefully reviewed and monitored by a congenital cardiologist.
Heart and lung transplantation or lung transplant with repair of the heart defect are options for some people with Eisenmenger's syndrome. Heart and lung transplants are rare--fewer than 50 are performed in the United States each year. As donor availability increases and surgical technology and drug research continue to advance, long-term results will also improve.
Complications from Eisenmenger's syndrome can be deadly. Even a simple operation requiring a general anesthetic (such as an appendectomy) is potentially life-threatening. Surgery should be performed at a medical center where the congenital cardiologist, surgeon, cardiac anesthesiologist, and staff have experience with Eisenmenger's syndrome. In addition, patients with Eisenmenger's syndrome need to discuss the use of vitamin supplements with their physician. Iron changes the chemistry of blood and should be used only if the blood iron level is low. Over-the-counter multivitamins often contain supplemental iron and should not be used without approval of the congenital cardiologist.
People with hypertrophic cardiomyopathy have an abnormally thick heart muscle that interferes with the ability of the heart to pump blood. Family members should also be evaluated for this disease since hypertrophic cardiomyopathy can be inherited. The Heart Center includes a complete module on hypertrophic cardiomyopathy, with much detail on treatments for the condition.
Treatment generally is not undertaken until the symptoms of shortness of breath, dizziness, chest pain, or palpitations develop. The first line of treatment is usually medication. Beta-blockers, calcium channel blockers, or antiarrhythmics can be used to relieve symptoms of hypertrophic cardiomyopathy.
When medications no longer relieve the symptoms of hypertrophic cardiomyopathy, a surgical procedure known as a myectomy may be warranted. Myectomy, the surgical removal of muscle tissue blocking blood flow out of the heart, is currently considered the safest and most successful treatment for hypertrophic cardiomyopathy. Myectomy is a specialized procedure with the best results produced by experienced surgeons.
Other treatment options include septal ablation or implantation of a cardiac pacemaker. Septal ablation, a heart catheterization technique that incapacitates and shrinks the localized area of obstructing muscle of the heart, can also be an effective treatment. This treatment is considered experimental by the American Heart Association and lacks long-term data on the survival of patients. Implantable pacemakers, which generally are not as successful as myectomy or septal ablation, are an option for people who cannot or will not undergo surgery or catheterization.
Approximately 1 percent of patients with hypertrophic cardiomyopathy die suddenly with little or no warning; sudden death may be the first symptom a person experiences. All people with hypertrophic cardiomyopathy should be evaluated to determine their risk of sudden death. Cardioverter defibrillators are usually implanted in individuals at increased risk for sudden death.
Hypoplastic left heart syndrome is a serious congenital heart defect in which the left ventricle is severely underdeveloped. Treatment starts immediately upon diagnosis with administration of the medicine prostaglandin to prevent the patent ductus arteriosus from closing. A ventilator may be necessary to assist with breathing, as well as intravenous fluids and additional medications to strengthen the heartbeat. These measures are undertaken to stabilize the baby's condition while decisions regarding treatment can be made. Management of hypoplastic left heart syndrome largely depends on the family's wishes and the severity of the condition.
For some babies, a three-stage surgical procedure is an option. The operation reconstructs the child's heart so that the single pumping chamber can meet the patient's lifetime needs. Completion of these operations allows separation of the blood without oxygen from the oxygenated blood.
Stage 1: Norwood Procedure
Performed as soon as possible after the diagnosis, the Norwood procedure enables the heart to pump blood to both the lungs and the body. This procedure involves reconstructing the aorta, the main blood vessel supplying blood to the body, and inserting a tube that connects the aorta to the pulmonary artery, the blood vessel that supplies blood to the lungs. The baby will still look blue (cyanotic) after this first-stage operation. Two heart catheterizations are typically performed in the next two years, usually just before each of the next two surgical stages.
Stage 2: Bidirectional Glenn Procedure or hemi-Fontan
The bidirectional Glenn procedure reduces the work of the heart by routing blood from the upper body directly to the lungs. This operation is usually performed at 6 months of age and increases the oxygenation of the blood.
Stage 3: Fontan Procedure
The third and final operation is usually performed between 18 to 48 months. This procedure allows the blood coming back from the lower body to go directly to the lungs, eliminating mixing of oxygenated and nonoxygenated blood in the child's heart. After this operation, the patient's general health and appearance will improve and the child will no longer look blue.
The function of the reconstructed heart and blood vessels needs long-term monitoring and followup care, which may include ongoing treatment with cardiac medications. Regular monitoring should include evaluating the functions of the child's heart with echocardiography as the child grows and develops.
Alternatively, an infant with this defect can be put on a list to receive a heart transplant. The infant is then kept on prostaglandin until a donor heart becomes available.
The strategy for treating patent ductus arteriosus (PDA) depends largely upon the size of the opening. A small PDA often closes spontaneously as the child matures. PDAs that persist may increase the risk of developing endocarditis, cause excess blood flow to the lungs, and lead to heart failure. If the duct remains open and the heart does not respond to medical treatment, closure of the duct using either a transcatheter procedure or surgery may be necessary. Once the duct has closed, no further problems are typically encountered.
Medical management may include administering indomethacin, a nonsteroidal anti-inflammatory drug, to newborns and infants to help constrict the muscle in the wall of the patent ductus arteriosus and close it. Digoxin may be used to strengthen the heart muscle and improve the efficiency of the heart and lungs. Diuretics also may be prescribed to help the body remove excess fluids.If medication is not successful or closure of the defect is deemed necessary, a transcatheter may be used to plug the opening and stop blood flow through the PDA. A transcatheter is a long, thin tube that is inserted into the body, usually in the groin area, and is guided through an artery to the defect, much like heart catheterization. A tiny device on the tip of the transcatheter, called an intravascular coil or occluder, is used to place plugs or coils to block the patent ductus arteriosus. This method is often used to treat small PDAs that do not close spontaneously.
Alternatively, surgeons can use ligation, a technique that may be used to close the patent ductus arteriosus by tying or clipping it together without opening the heart. The goal of PDA ligation is to prevent the lungs from becoming diseased from too much blood flow and prevent the development of heart failure. The procedure is performed with the infant under general anesthesia. An incision is made on the left side of the chest near the armpit, through which the PDA is exposed where it attaches to the aorta. The tie or clip is placed on the PDA and the flow across the PDA stops immediately. If the infant has no symptoms, the operation can be postponed until 6 months to 3 years of age.
Pulmonary valve atresia frequently occurs in conjunction with other congenital heart defects that largely determine the course of action. The most common association is with a ventricular septal defect.
More information about pulmonary valve atresia without a ventrical septal defect is on the next page.
When accompanied with a septal defect, pulmonary valve atresia may be diagnosed in infancy or childhood, or may not be diagnosed until adolescence or even adulthood. Treatment for these patients aims at normalizing blood circulation in the heart by closing the septal defect and connecting the right ventricle to the pulmonary artery. This involves open-heart surgery to insert a conduit with a new valve that channels blood from the right ventricle into the pulmonary artery for oxygenation in the lungs.
If the pulmonary arteries are too small, an additional operation will be needed prior to inserting the conduit. In this procedure, the surgeon uses a shunt to create a connection between the aorta, or a branch of the aorta, and the pulmonary artery. The shunt is a palliative measure that increases blood flow to the lungs, facilitating growth of the pulmonary arteries until the more permanent conduit can be surgically placed.
This section also contains information about pulmonary valve atresia without a ventricular septum defect.
Infants born with pulmonary valve atresia and an intact ventricular septum need immediate medical attention in a medical center with experienced pediatric cardiologists and pediatric cardiovascular surgeons. Upon diagnosis, the infant will be admitted to an intensive care unit to stabilize his or her condition while subsequent treatment decisions are made. In the intensive care unit, oxygen will be administered using a ventilator to assist breathing. Medications will be administered to help the heart and lungs function as efficiently as possible and to keep the ductus arteriosus ductus arteriosus open in order to maintain blood flow to the lungs.
Heart catheterization may be performed early on to evaluate any other heart defects as well as to evaluate an initial treatment. Heart catheterization usually will be performed to establish whether the foramen ovale foramen ovale or ductus arteriosus is still open, and to determine the amount and relative proportions of oxygen-rich and oxygen-poor blood circulating in the heart.
As an initial treatment, balloon atrial septostomy may be performed to increase the amount of oxygenated blood in the heart by creating an opening between the right and left atria (upper chambers of the heart). In this procedure, a special catheter with a balloon in the tip is guided through the foramen ovale and into the left atrium, where the balloon is inflated to open a connection between the atria.
Open-heart surgery is often required within the first week of life. The surgeon will either open the pulmonary valve, allowing blood flow to the lungs, or insert a conduit that includes a new valve to channel blood flow from the right ventricle to the lungs. Alternatively, the surgeon may replace the baby's closed valve with a prosthetic valve.
Following surgery, the baby's circulation may be normal. Patients with donor valves may need another heart valve replacement surgery within the next 10 years as the donor valve slowly wears out. In this situation, the surgeon will create an artificial connection (shunt) to divert blood from the aorta, which carries blood to the body, to the pulmonary artery, which delivers blood to the lungs for more oxygenation.
Additional surgery may be needed when the child is older, particularly in cases where the right ventricle is underdeveloped. The type of procedure depends upon the size of the right ventricle and the pulmonary artery. If they are normal in size and the right ventricle is able to pump blood, the goal of the open-heart procedure is to channel the blood flow through the heart in a normal pattern. If the right ventricle is small and unable to pump, doctors may perform an operation called the Fontan procedure, in which the right atrium is connected directly to the pulmonary artery to improve oxygenation of the blood.
A child who has pulmonary atresia requires intensive monitoring both before and after surgery. The child will also need lifelong monitoring by a congenital heart specialist, with regular blood tests, X-rays, and urine analysis.
Treatment options for ventricular septal defects depend on the size of the opening in the ventricle. Almost 50 percent of VSDs are small and close spontaneously shortly after birth, requiring no further treatment. Larger defects need to be closed, often before the age of 1. Ventricular septal defects often occur in conjunction with other heart defects, therefore treatment is highly individualized.
Isolated VSDs may be closed using a cardiac catheterization procedure. Commonly a small patch resembling a parachute is placed over the opening using a catheter; however, there are a variety of other patches that can be used as well. The cardiologist uses a catheter that enters the body through a leg or arm to place the patch. More detail about heart catheterization can be found in the testing section.
Not all VSDs are suitable for catheterization; in these cases heart surgery is necessary to close the VSD. This may be due to the location or size of the VSD or because the VSD occurs with other congenital defects. The procedure is highly individualized depending upon the patient's situation.
Congenital heart valve regurgitation, in which blood is able to flow back through the valve, is caused by an abnormally formed valve that does not close properly. In the case of the pulmonary and tricuspid valves, if the defect is mild to moderate and the patient has no symptoms, regular medical checkups to carefully monitor the heart valve and pump function may suffice. More severe regurgitation may cause the right ventricle to enlarge, requiring repair or replacement of the valve (see below). Mitral and aortic valve regurgitation can lead to irreversible heart damage and therefore are surgically treated. In many patients, regurgitation is associated with other congenital heart defects that may largely determine the course of treatment for an individual. While no medication can correct valve regurgitation, certain drugs can minimize the symptoms by easing the heart's workload and regulating the heart's rhythm. Depending on the nature of the heart valve defect, any of the following medications may be prescribed:
- Digitalis to strengthen the heart muscle contractions.
- Diuretics to reduce swelling and ease the heart's workload.
- Anticoagulants to prevent blood clots.
- Beta-blockers to control the heart rate and lower blood pressure.
- Calcium channel blockers to lower blood pressure and reduce the heart's workload.
- ACE inhibitors to lower blood pressure and decrease the heart's workload.
Read more in the section on medications.
If the valve defect is significant and medications do not control the symptoms, surgery to repair or replace the valve may be necessary. In general, most surgeons agree that heart valves should be repaired when possible and replaced only when necessary. Valve regurgitation can be resolved in a variety of ways depending upon the circumstances of the structural defect.
Valvuloplasty, or surgically modifying the original valve, can effectively eliminate regurgitation in some people. Surgeons can shorten or replace the cords that support the valves; when the cords and muscles are the right length, the valve leaflet edges meet and eliminate the leak. Alternatively, the ring of tissue that supports the valve leaflets can be made smaller. By decreasing the size of the opening, the leaflets of the valve are able to close tightly, preventing regurgitation. Sometimes, surgeons will implant an annuloplasty ring to downsize an abnormally enlarged valve opening. Surgeons can also repair the valve by removing excess tissue to help the valve close snugly and prevent back flow. Valve replacement requires open-heart surgery. The surgeon removes the faulty valve and replaces it with an artificial one, also known as a prosthetic valve--either a mechanical prosthetic valve or a biological prosthetic valve. Approximately 95 percent of all valve replacements are performed on mitral or aortic valves. The key to selecting the right prosthetic valve involves weighing the advantages and disadvantages of certain valves with the risk of anticoagulation therapy.
Mechanical prosthetic valves, made of synthetic materials, are more durable and reliable than tissue valves. However, blood tends to stick to mechanical valves, causing blood clots; therefore, patients receiving mechanical valves must take a blood-thinning drug (anticoagulant), such as warfarin, for the rest of their lives. People who take anticoagulants must have their blood tested regularly to ensure that their blood has not gotten too "thick" or "thin." This constant monitoring can be especially challenging in children. In addition, blood thinners are not recommended for people who play contact sports and pose risks for a developing fetus. Women who wish to become pregnant or young adults participating in contact sports should discuss in detail the choice of valves with their congenital cardiologist and cardiovascular surgeon.
Biological prosthetic valves are made from animal tissue or human tissue taken from a donated heart and do not require blood thinners. However, they are not are not as durable as mechanical valves and may require replacement within 10 years. In general, patients younger than 70 receive mechanical valves; those over 65 receive biological prosthetic valves. For some younger people, a bioprosthetic valve may be used with the understanding that an additional surgery will be necessary in the future to replace it with a mechanical valve at a future date.
Most people born with tetralogy of Fallot will need heart surgery during childhood, either to relieve symptoms or to repair the abnormalities associated with this condition, actually a set of defects including a ventricular septal defect, thickened right ventricular wall, an obstruction to blood flow beneath the pulmonary valve, and an aorta that is shifted to the right. However, some individuals reach adulthood without having had surgery. In either case, regular evaluation by a cardiologist experienced in caring for congenital heart disease is needed. A typical evaluation would include a thorough physical exam, electrocardiogram, chest X-ray, echocardiogram and an exercise test. A Holter monitor or event recorder may also be used to check for irregular heart rhythms. More detail can be found in the testing section.
Two main surgical options for patients with tetralogy of Fallot include:
- Palliation, usually done in early childhood, involves surgically placing a small tube to connect a branch of the aorta to the pulmonary artery. This procedure channels more blood to the lungs to improve blood oxygenation. The Blalock-Taussig procedure is an example of this kind of surgery in which the subclavian artery, a branch of the aorta that normally carries blood to the arms, is surgically connected to the pulmonary artery instead to allow the blood to pick up more oxygen. This operation is usually performed on an infant to postpone corrective surgery until the heart and lungs are more developed.
- Reparative or "corrective" surgery has become a highly effective long-term treatment for tetralogy of Fallot. This surgery redirects the blood flow inside the heart using a variety of measures. The ventricular septal defect is closed, usually with a Dacron patch. The right-sided obstruction to the lungs is relieved by surgically removing the muscle that is obstructing flow. Often the pulmonary artery is enlarged as well. If the pulmonary valve is also thickened or abnormal, it will be repaired or replaced to further relieve the obstruction. People who have undergone reparative operations are still at risk for developing problems as they age and should be evaluated regularly by a specialist in congenital heart disease. Sometimes further surgery is necessary, often to replace the pulmonary valve, which may have been distorted at the time of the first operation.
Medications are commonly prescribed for people with tetralogy of Fallot, for both surgically corrected as well as uncorrected defects. Inotropes, like digitalis or digoxin, may be used to enhance heart function, and antiarrhythmics may be used to control heart rhythms. Read more in the section on medication.
For individuals who have pulmonary valve stenosis with little or no symptoms, regular medical checkups to monitor the narrowed valve and the patient's overall health are usually recommended. If symptoms develop, medications may be used to control pain, reduce the workload on the heart, and regulate the heart's rhythm, and in some cases may slow the progression of stenosis and delay the need for surgery. Common medications used to treat pulmonary valve stenosis include:
- Digitalis to improve heart function
- Diuretics to reduce swelling and ease the heart's workload
- Anticoagulant medicines to prevent blood clots
- Beta-blockers to control the heart rate and lower blood pressure
- Calcium channel blockers to lower blood pressure
Read more in the section on medications.
As conditions change, surgery or catheterization may be necessary. Pulmonary valve stenosis, when it occurs in isolation of any other abnormalities, is usually treated by a cardiologist using balloon dilation to mechanically widen the valve. Balloon dilation is a heart catheterization technique. More detail about heart catheterization can be found in the testing section.
However, if the valve stenosis is severe or involves other abnormalities in the heart, surgery may be necessary to repair the defect. Heart surgeons generally agree that, whenever possible, a heart valve should be repaired instead of replaced. The advantage of heart valve repair is that patients retain their own normally functioning tissue, which is resistant to infection and does not require blood-thinning medication. In some cases, the valve is too damaged for repair and must be replaced with a prosthetic valve.
Last reviewed on 2/11/2009
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