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What Is Adult Congenital Heart Disease?
Congenital heart disease (CHD) is a defect in your heart's structure that is present at birth. A congenital heart defect can include:
- Abnormally shaped blood vessels.
- Abnormal connections between the heart and its main blood vessels.
- Defects in the walls that separate the heart chambers (commonly called a “hole in the heart”).
- Missing or malfunctioning valves.
How common is adult congenital heart disease?
Congenital heart disease affects about 40,000 (1 percent) of babies born in the U.S. each year.
What is a common adult congenital heart disease?
Common adult congenital heart diseases include:
- Blood vessel disorders, particularly of the arteries and veins that carry blood to and from the heart.
- Heart valve problems.
- Septal defects.
What are the types of adult congenital heart disease?
Our experienced physicians provide comprehensive treatment for congenital heart conditions in adults, including:
- Atrial septal defect (ASD) – ASD occurs when certain tissues in your heart do not form properly, leaving a hole between the two upper chambers of your heart called the atria.
- Atrioventricular septal defect (AVSD) – AVSD septal defect occurs when there is a hole between the chambers of your heart and the valves that control blood flow are not formed properly.
- Bicuspid aortic valve, with or without stenosis and regurgitation – Bicuspid aortic valve occurs when your aortic valve has two leaflets instead of three, which may cause problems with blood flow because your valve does not open or close properly.
- Coarctation of the aorta – Coarctation of the aorta occurs when your aorta, the large artery that connects to your heart, is narrowed and forces your heart to pump harder to circulate your blood.
- Complete atrioventricular canal defect (CAVC) – CAVC occurs when a large hole in the center of your heart causes blood from each of your heart's four chambers to mix and prevents proper circulation.
- Congenital aortic valve stenosis – Congenital aortic valve stenosis occurs when your aortic valve is unable to open completely, resulting in problems with blood flow.
- Congenital coronary anomalies – Congenital coronary anomalies occur when one or more of your coronary arteries do not form properly.
- Congenitally corrected transposition of the great arteries (ccTGA) – ccTGA occurs when your heart twists so that the ventricles in the lower half of your heart are reversed.
- Congenital pulmonary valve stenosis – Congenital pulmonary valve stenosis occurs when your pulmonary valve, which allows blood to flow out of your heart to your lungs, does not function properly.
- Double outlet right ventricle (DORV) – DORV occurs when your aorta connects to your right ventricle instead of your left ventricle, which causes your right ventricle to have two outlets (the pulmonary artery and the aorta) and your left ventricle to have no outlets.
- Ebstein's anomaly – Ebstein's anomaly occurs when the leaflets of your tricuspid valve, which connects your upper and lower right heart chambers, are not properly formed and cause problems with blood circulation.
- Eisenmenger syndrome – Eisenmenger syndrome occurs when a hole in your heart causes abnormal blood circulation within your heart and lungs.
- Hypoplastic left heart syndrome (HLHS) – HLHS occurs when the structures on the left side of your heart — including your left ventricle, mitral valve, aortic valve, and aorta do not develop properly.
- Marfan syndrome – Marfan syndrome affects your body's connective tissue and may cause problems with your heart valves or weaken your aorta.
- Partial anomalous pulmonary venous return – Partial anomalous pulmonary venous return occurs when some of your pulmonary veins, which carry blood from your lungs to your heart, flow into the wrong place.
- Patent foramen ovale (PFO) – Everyone is born with a foramen ovale, or a hole in the wall between the two upper chambers of your heart. PFO occurs when this opening does not close within a few months of birth. Many cases of PFO do not require treatment, but it may be necessary to have PFO closure surgery to treat certain complications that may develop later in life.
- Subaortic membrane – Subaortic membrane occurs when a shelf-like membrane develops under your aortic valve, causing problems with blood flow.
- Tetralogy of Fallot – Tetralogy of Fallot is a combination of four congenital heart defects that affect the structure of your heart and blood flow. These four defects are: ventricular septal defect, pulmonary stenosis, ventricular hypertrophy, and overriding aorta).
- Transposition of the great arteries – Transposition of the great arteries occurs when the positions of your pulmonary artery and your aorta are switched.
- Turner syndrome – Turner syndrome, which affects only females, may cause abnormalities in the structure of your heart and problems with your aorta.
- Univentricular heart (single ventrical) – This is a defect that occurs when one ventricle does not function properly. This causes the oxygenated and deoxygenated blood to mix.
- Ventricular septal defect (VSD) – VSD occurs when certain tissues in your heart do not form properly, leaving a hole between the bottom two chambers of your heart called the ventricles.
What causes adult congenital heart disease?
The exact cause of CHD is unknown. Possible causes include:
- Family history.
- Role of genetics and gene-interactions.
- Role of common exposures (diabetes) or teratogens (alcohol/medications during pregnancy).
What are adult congenital heart disease complications?
Complications of adult congenital heart disease?
Because heart defects are different, complications can vary. People with CHD may experience complications such as:
Many patients stop seeing their cardiac specialist during their teen or college years. It's important that a CHD doctor follow your condition — even when you feel fine — to ensure early treatment of any problems that may arise.
CHD and pregnancy
Pregnancy may increase the risk of health problems in women with CHD. It may also cause health problems for the unborn baby.
Cardiovascular risks that should be monitored during pregnancy include:
Coordinated care between an adult CHD specialist and an obstetrician can help reduce risks and result in a successful pregnancy.
Because some heart defects can be genetic, women with CHD may want to talk with their care team and a genetic counselor before becoming pregnant.
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What Are the Signs and Symptoms of Adult Congenital Heart Disease?
People with congenital heart disease (CHD) may not have symptoms. When symptoms do occur, they can include:
- Abnormal heart rate or rhythm (arrhythmia).
- Blue skin color (cyanosis).
- Fatigue after exerting yourself.
- Dizziness or fainting.
- Shortness of breath.
- Swelling of the body tissue or organs (edema).
When should I see a doctor about my adult congenital heart disease symptoms?
If you are experiencing any symptoms of CHD or other cardiac symptoms, you should schedule an appointment with your doctor. If your symptoms are severe, dial 911 or go to the nearest hospital emergency room.
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How Do You Diagnose Adult Congenital Heart Disease?
Our experts use leading-edge technology and techniques to diagnose CHD. Our evaluation process includes:
- A collaborative approach with cardiac imaging, electrophysiology, interventional cardiology, heart failure, and cardiac surgery experts working together to deliver the best possible outcome.
- A review of prior medical records about original heart defects and any cardiac procedures.
- A thorough analysis of medications.
- Testing using the latest diagnostic technology and techniques.
What is congenital heart disease that is discovered in adulthood?
Although CHD is present at birth, it may not be diagnosed until symptoms develop in adulthood. In some cases, it may be identified during a routine exam or imaging test. If you have CHD that is diagnosed in adulthood, your doctor will recommend a treatment plan.
What to expect during your visit
During your physical exam, your doctor will:
- Ask about your symptoms and when they started.
- Discuss your medical history.
- Listen to your heart with a stethoscope.
Following your exam, your doctor may order additional tests and procedures to confirm a diagnosis of CHD or identify new concerns.
Tests to diagnose adult congenital heart disease
Tests your doctor may use to diagnose CHD include:
Adult congenital heart disease prognosis
Unlike in the past, many people with CHD live into adulthood and enjoy a high quality of life.
What is the life expectancy of adults with congenital heart disease?
Life expectancy of adults with CHD depends on the type of CHD you have and whether complications develop.
Can you survive congenital heart disease?
With early diagnosis and proper treatment, many adults with CHD live long, healthy, and active lives.
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How Do You Treat Adult Congenital Heart Disease?
There are many treatment options for CHD.
Depending on your condition, doctors at the Adult Congenital Heart Disease Center may recommend:
Ongoing monitoring
If your CHD does not cause symptoms, your doctor may recommend regular check-ups to address any problems that may develop.
Medicine to treat adult congenital heart disease
For mild complications, your doctor may recommend:
- Anticongestive medications that reduce congestion in the blood vessels in the lungs.
- Anticoagulant drugs that help prevent blood clots.
- Medications to control heart rate or rhythm problems.
Surgery for adult congenital heart disease
Depending on the type of CHD you have, your doctor may recommend surgical procedures including:
- Implantable defibrillator – If you have a serious heart rhythm problem that medication cannot control, your doctor may recommend an implantable defibrillator. This device monitors your heart for abnormal heartbeats and sends a small shock to restore normal rhythm if needed.
- Implantable pacemaker – Your doctor may recommend a pacemaker to help control your heart rhythm.
- Minimally invasive surgical repair – UPMC doctors can offer minimally invasive heart surgery — such as the Melody pulmonary valve — and closure of septal defects.
- Open-heart surgery – If your congenital heart defect can’t be treated with minimally invasive techniques, your doctor may recommend open-heart surgery.
- Heart transplant – Adults with CHD may develop severe heart failure, pulmonary hypertension, or cyanosis. If you develop serious complications, your doctors may recommend a heart transplant evaluation.
How effective is treatment?
Many cases of CHD are diagnosed before birth and can be treated with surgery shortly after birth. But although surgical repairs can help the heart work more normally, they may not “cure” the problem. Sometimes, symptoms of CHD can happen again later in life and may need additional treatment.
Our adult congenital heart specialists work with our pediatric cardiologists to ensure that our patients keep receiving care so they can enjoy a high quality of life.
What should you avoid if you have congenital heart disease?
If you are diagnosed with CHD, your doctor may recommend that you make lifestyle changes, such as:
- Eating a heart-healthy diet.
- Exercising regularly.
- Maintaining a healthy weight.
You should also avoid smoking, limit alcohol, avoid recreational drugs, and control chronic conditions such as high blood pressure and high cholesterol.
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Why Choose UPMC for Adult Congenital Heart Disease Care?
As a leader in cardiovascular care in Pennsylvania, UPMC offers patients access to a team of experts skilled in managing the needs of congenital heart patients.
Our congenital heart disease specialists advise patients with CHD on health and lifestyle choices that work for them. This includes working with patients who have physical challenges or providing guidance on family planning options appropriate for CHD patients. We also offer related care by specialists in high-risk pregnancy and genetics.
If your congenital heart condition causes complications or requires further treatment, our specialists can provide advanced care using the latest minimally invasive, catheter-based, and surgical techniques.
Our goal is to provide care and patient education to lead productive life, staying on top of future health issues, coping with psychological aspect, and careful attention during family planning and reproductive issues.
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By UPMC Editorial Staff.
Last reviewed on 2024-10-01 by Patricia Hovanec, CRNP.
