A heart transplant gives a patient with congenital heart disease the opportunity to have a normal heart with normal blood circulation. If the transplant goes well, heart function and blood flow will be better than ever.
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What is a heart transplant?
A heart transplant replaces the patient's heart with a donor heart. Doctors remove the patient's heart by transecting the aorta, the main pulmonary artery and the superior and inferior vena cavae, and dividing the left atrium, leaving the back wall of the left atrium with the pulmonary vein openings in place. The surgeon connects the donor heart by sewing together the recipient and donor vena cavae, aorta, pulmonary artery and left atrium. In patients with congenital heart disease, the surgeon may simultaneous transplant the lungs and the heart.
Why is it needed?
You may require a heart transplant for several reasons. The most common reason is that one or both ventricles have aren't functioning properly and severe heart failure is present. Ventricular failure can happen in many forms of congenital heart disease, but is more common in congenital defects with a single ventricle or if long-standing valve obstruction or leakage has led to irreversible heart failure. Patients who as children had the Fontan procedure, which helps complex congenital heart defects, may need a heart transplant because the blood flow through the venous system is slow and the veins are congested, which can lead to swelling, fluid accumulation, and protein loss.
How does it affect the heart?
The donor heart is matched to the recipient by blood type and body size. As the heart transplant recipient, you must take medications to prevent his or her immune system from rejecting the new heart. These drugs are called immunosuppressive medication. Your medical team will balance the amount of immunosuppressive medication you need to prevent rejection of your new heart with the risk of side effects, which include infection or cancer.
What can I expect if my child is a heart transplant recipient?
When a child receives a heart transplant, the transplanted heart grows to adult size as the patient grows. Your child will need to take immunosuppressive medications and other medicine for the rest of his or her life to control the sides effects of the transplant. During young adulthood, your child's medical care will be transitioned from a pediatric to an adult heart transplant cardiologist.
What will I need in the future?
After your heart transplant, your medical team will monitor you closely for heart rejection, which can happen in the heart muscle cells or in the heart's arteries. They will also watch for side effects of the immunosuppressive medications, which include diabetes, infection, kidney disease, cancer or high blood pressure. If any of these problems arise, your doctor will change the medication type or dose. You and you're your doctor may also decide to change your immunosuppressive medications as new drugs become available.
You will require regular checkups after your transplant by a transplant cardiologist. At these visits, your cardiologist will do blood tests to check the levels of your immunosuppressive drugs and look for side effects. He or she may also order electrocardiogram, echocardiogram and Holter monitoring to help monitor your heart rhythm and function, or an endomyocardial biopsy, which is a diagnostic procedure that surveys the sufficiency of your immunosuppressive therapy. Your doctor will evaluate your coronary arteries yearly or every other year to monitor for signs of narrowed coronary arteries in your transplanted heart. You should also have routine medical checkups to maintain overall health.
Heart transplant recipients have no specific activity restrictions. Discuss activity ideas with your transplant cardiologist.
Endocarditis is an infection of the inner layer of the heart. While some people who have congenital heart disease must take antibiotics prior to some medical and dental procedures to prevent endocarditis, most heart transplant recipients don't need them unless they also have significant heart valve disease.
Women who've had a transplant could have complications during pregnancy. Depending on the type, immunosuppressive medications may negatively affect the fetus. You may also have a greater risk of rejection once the baby is born. If you are considering pregnancy, discuss the pros and cons with your transplant cardiologist and obstetrician.
See the Pregnancy section for more information.
Will I need more surgery?
A transplant heart can help you lead a more active, fulfilling life, but there may be times when additional surgery is required. For instance, if the rhythm of your transplant heart becomes slow, you may need to have a pacemaker. Rarely, the tricuspid valve can become damaged by the endomyocardial biopsy procedure; if that happens it will need to be repaired or replaced. Patients with congenital heart disease who have had a coarctation repair or problems with narrow or small pulmonary arteries may need surgery or interventional catheterization after the transplant to increase the size of these areas. Sometimes, a transplanted heart may fail because of rejection, damage to the heart cells or coronary arteries of the heart, which leads to heart failure. If this happens, doctors can sometimes transplant another heart.