Adult Congenital Heart Disease (ACHD)

Although congenital heart disease is present at birth, symptoms may not appear until adulthood.

Congenital heart defects may present in adults with the following symptoms:

• Shortness of breath at rest or with exercise
• A bluish tint to the skin and fingernails (cyanosis)
• Fatigue or tiredness
• Palpitations and fast heartbeats
• Leg or abdominal swelling
• High blood pressure

Some congenital heart defects, or their associated repairs, can predispose a person to other medical problems involving his or her liver, kidneys, lungs or even blood. This means regular checkups will remain an important component of managing your congenital heart defect throughout your life.

To diagnose a congenital heart defect in adulthood, your doctor will perform a physical exam and may evaluate your heart using one or more of the following diagnostic tests:

• Chest X-ray
• Echocardiogram – a type of ultrasound that takes picture of your heart
• Electrocardiogram (ECG or EKG) – a test of the electrical activity of your heart
• Cardiac catheterization – a catheter-based procedure that can help identify defects in your heart
• Cardiac MRI – a noninvasive method of imaging your heart using radio waves, magnets and a computer
• Cardiac CT – an imaging method that uses X-rays to take many detailed pictures of your heart and its blood vessels

Adults with congenital heart disease require specialized, lifelong care that frequently involves a combination of medicine and lifestyle changes — with some people also requiring catheter-based procedures, implantable heart devices or even open-heart surgery.

Your doctors will work together and with you to determine the best course of treatment for your specific heart defect and unique lifestyle needs, which may include one or more of the following:

Medications for Adult Congenital Heart Disease

Management of adult congenital heart disease often requires taking medication. Several types of medicine may be used to help relieve your symptoms, including:

• Diuretics, to decrease excess fluid in your body 
• Vasodilators, to widen blood vessels and promote blood flow 
• Anti-arrhythmics, to help prevent irregular heartbeats 
• Inotropes, to strengthen your heartbeat

In addition, antibiotics and blood thinners may be recommended before any surgeries you may have in order to prevent endocarditis and lower your risk of blood clots, respectively.

Lifestyle Modification for Adult Congenital Heart Disease

Your doctor may recommend adopting, adjusting or maintaining heart-healthy lifestyle behaviors, such as:

• Exercising regularly
• Maintaining a healthy diet
• Stopping or avoiding smoking
  
  

Cardiac Catheterization for Adult Congenital Heart Disease

In some cases, your cardiologist may recommend evaluating or repairing your heart defect(s), and the surrounding arteries, using cardiac catheterization.

During this procedure, a catheter (a thin, flexible tube) is inserted into a blood vessel and navigated through your blood vessels and into your heart. Your cardiologist then threads very tiny tools through the catheter to repair your heart defect.

At Houston Methodist, our cardiologists are experts in the minimally invasive techniques used to treat adults with congenital heart disease, including:

• Percutaneous septal defect closure to treat common congenital heart defects
• The revolutionary transcatheter pulmonary valve to implant a new prosthetic valve without need for open heart surgery

Open-Heart Surgery for Adult Congenital Heart Disease

In recent years, cardiac catheterization has reduced the need for open surgery to treat adult congenital heart disease.

However, some defects still require open-heart surgery to restore normal function to the heart. Fortunately, improvements in both technology and techniques have made heart surgery safer and more effective than ever before.

Houston Methodist’s cardiovascular surgeons rank among the most respected in their fields for a variety of open-heart surgeries.

Implantable Devices for Adult Congenital Heart Disease

Some types of congenital heart defects can lead to arrhythmias or heart failure, which may need to be treated with implantable heart devices that restore heart function to a normal level.

Recent advances have made these devices smaller and easier to manage than ever before, allowing the people who need them to lead normal, active lives.

Common implantable devices include:

• Pacemaker – a small device implanted in the chest to control heart rhythm
• Defibrillator – a small, implanted device that treats arrhythmias
• Ventricular assist device (VAD) – a mechanical pump to support heart function and blood flow

Heart and Multi-Organ Transplant for Adult Congenital Heart Disease

Unrepaired or undiagnosed congenital heart defects can sometimes lead to long-term heart damage, ultimately causing heart failure. In these cases, a heart transplant may be required.

The Adult Congenital Heart Program at Houston Methodist is one of the few programs in the world with a specialized team dedicated to the evaluation and management of adults with congenital heart disease who may need heart and multi-organ transplant. In collaboration with doctors from the J.C. Walter Transplant Center, we use advanced therapies, including organ transplant, to restore your quality of life to the best it can be.

Replacing a failing heart with a healthy heart via transplant surgery is a complex procedure, but many people go on to lead full and active lives after transplant. Because of complex congenital heart disease, some patients may also experience lung, liver or kidney failure and require transplant of another organ in addition to heart.

At Houston Methodist, our experts have set the standard and lead the field for heart and multi-organ transplant, with more than 760 heart transplants performed in our transplant center.

Many adults are able to live normal, healthy lives after receiving treatment for a congenital heart condition.

However, even following successful surgical repair, some adults are at risk for developing complications. These complications may require additional surgery or other procedures — sometimes years after the initial treatment. These complications include:

• Abnormal heart rhythms (arrhythmias), such as atrial fibrillation (AFib) 
• Heart failure 
• Heart infections (endocarditis) 
• Heart valve issues 
• Pulmonary hypertension 
• Stroke

Because of this, adults with repaired congenital heart disease should regularly follow up with their cardiologist. They should also be seen at least once a year by a heart expert who specializes in treating adults with congenital heart disease.

This is especially important if you’re a women with congenital heart disease who wants to become pregnant, as you will need a complete evaluation. If any other measures need to be taken, your heart care team is committed to providing the best available treatment for you.