Adult Congenital Heart Disease
About congenital heart defects
Congenital heart defects are present at birth and related to structural problems of the heart. While many defects can be treated successfully during childhood, adults often need additional consultations, procedures or medications later in life.
Also known as congenital heart failure, CHD may be first identified after a patient has been found to have a heart murmur, which is an abnormal sound of blood moving inside or near the heart as it beats. Not all heart murmurs are serious, but they can be an indication of an underlying heart condition, including congenital defects.
Common types of adult congenital heart defects
Long-term problems associated with congenital heart defects can include difficulty exercising, abnormal heart rhythms, congestive heart failure, sudden cardiac arrest and stroke.
Types of adult congenital heart defects managed and treated at Scripps or through partners include:
Ventricular septal defect (VSD)
Ventricular septal defect is a hole in the wall of the heart (called the septum) that separates the two lower chambers of the heart (called the ventricles). This causes pulmonary hypertension (high blood pressure), which can damage blood vessel walls. VSD surgery during childhood requires regular follow-up and care by a cardiologist throughout adult life. Untreated VSD diagnosed in adults may require surgery if it poses a danger to the patient.
Aortic valve stenosis (AVS)
Aortic valve stenosis is a condition in which a major heart valve does not open and close properly. This can cause unnecessary pressure inside the heart and cause damage. AVS is treated through non-surgical, catheter-based procedures (interventional cardiology) or open heart surgery.
Atrial septal defect (ASD)
Atrial septal defect is a hole in the wall of the heart (called the septum) that separates the two upper chambers of the heart (called the atria). If the opening is small, ASD may not cause symptoms or require treatment. But large openings can cause the heart to swell, put pressure on the lungs and reduce the amount of oxygen in the blood.
Most ASDs can be treated with minimally invasive, catheter-based techniques that are done with only local anesthesia. More complex ASDs may require open heart surgery.
Patent ductus arteriosus (PDA)
Patent ductus arteriosus is a hole in the aorta, the main artery through which blood flows from the heart to the body. Such holes typically close within the first few days of a baby’s life. In adults, the defect can be treated through catheter-based procedures or with heart surgery.
Coarctation of the aorta (CoA)
Coarctation of the aorta is a narrowing of the major artery (the aorta) through which blood flows to the body. Left unchecked, CoA may cause heart damage, hypertension (high blood pressure) and a weakening of the heart’s lower left chamber (ventricle), leading to heart failure. CoA can often be treated with a non-surgical, catheter-based procedure.
Tetralogy of Fallot (ToF)
ToF is a heart defect that is present when there is a hole in the wall between the heart’s lower chambers (ventricles), an aorta positioned over that hole, a blockage from the heart to the lungs and an overly thickened muscle around the lower right chamber (ventricle). Adults who have had ToF repaired earlier in life require regular follow-up and care by a cardiologist.
Less common types of adult congenital heart defects
Less common types of adult congenital heart defects managed and treated at Scripps or through partners include:
Truncus arteriosus is a condition in which the heart does not have two arteries through which blood flows to the lungs and body. Instead it has only one vessel, which results in the heart working harder, and can cause damage to the lung’s blood vessels over time. Repaired during childhood, this heart defect requires regular follow-up and care by a cardiologist who tests the heart’s performance.
I-transposition of the great arteries
I-transposition of the great arteries is a condition in which the heart’s lower section is reversed, as well as its two main arteries through which blood flows to the body. As a result, the right lower chamber (ventricle) may weaken over time because it must work harder than intended. Heart surgery or a non-surgical procedure may be needed to repair the heart if conditions worsen.
Patent foramen ovale (PFO)
Patent foramen ovale is a hole present at birth in the wall separating the heart’s upper chambers (atria) and does not close as it should without medical intervention. Most adults with PFO never know they have the congenital heart defect or require treatment for it. The condition is most commonly found during tests for heart-related problems, and in certain cases can be treated through minimally invasive procedures.
Single ventricle anomaly
Single ventricle anomaly is a condition in which one of the heart’s ventricles is underdeveloped. This can be congenital or the result of a heart procedure if one of the ventricles of the heart does not develop properly. An example is the Fontan procedure (the surgical connection of a large vein from the lower body and veins from the liver to the lung arteries).
Pulmonary valve stenosis (PS)
Pulmonary valve stenosis occurs when the valve through which blood flows from the heart to the lungs does not fully open. The defect can cause damage over time because the heart must work harder to pump blood. The valve may also leak or become what is called “regurgitant.” PS can be treated with a catheter-based procedure called a balloon valvuloplasty or with surgery.
Endocardial cushion defect
Endocardial cushion defect is a large hole in the center of the heart. A complete atrioventricular canal defect (CAVC) may be repaired surgically when a child is young, but requires an artificial valve implant later in adulthood. Endocardial cushion defect is among the more complex adult congenital heart defects.
Transposition of the great vessels
This condition is when the two main arteries leaving the heart are reversed or transposed. Different types of heart surgery are commonly required to redirect blood flow or totally repair the defect. Adults who have had the defect surgically addressed in their youth can sometimes experience symptoms of congestive heart failure or abnormal heart rhythms later in life due to scar tissue.
Marfan syndrome is a disorder of connective tissue affecting the heart and its related structures, particularly the aorta. This condition requires active surveillance, medical management and counseling — and often requires surgical repair of the affected heart structure.