Scripps cardiologists help patients manage congenital heart defects through diagnosis, coordination of care and referrals to partner providers.
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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.
Adult congenital heart defects might also 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.
Many patients with congenital heart defects have unique and complex situations and have often had multiple surgeries or cardiovascular interventions.
Scripps provides sophisticated medical management for patients with congenital heart defects by working with nationally recognized physicians and medical partners who are among the most experienced in treating the particular condition a patient has.
Because of the complexity of each type of heart defect, it’s important to have the right team of cardiologists and surgeons from each heart specialty, including:
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:
Aortic valve stenosis (AS) 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. AS is treated through non-surgical catheter-based procedures (interventional cardiology) or open-heart surgery.
Atrial septal defect (ASD), which is a hole in the wall of the heart (called the septum) that separates the two upper chambers of the heart (called the atria). ASD is treated with interventional cardiology or open-heart surgery. Scripps physicians have a depth of experience in ASD procedures and surgeries.
Ventricular septal defect (VSD), which is a congenital heart defect in which there is a hole in the wall that separates the heart’s lower chambers (ventricles) causing pulmonary hypertension (high blood pressure) that 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. Scripps interventional cardiologists are experts in the treatment of VSD with minimally invasive catheter-based procedures when appropriate.
Patent Ductus Arteriosus (PDA), which 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), which 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.
Patent foramen ovale (PFO), in which a hole present at birth in the wall separating the heart’s upper chambers (atria) 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 offered at Scripps.
Transposition of the great vessels, which is the reversal of two main arteries through which blood flows from the heart. 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.
I-Transposition of the great arteries, which 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 has to work harder than intended. Heart surgery or a non-surgical procedure may be needed to repair the heart if conditions worsen.
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 and is also known as Atrioventricular (AV) canal defect or Atrioventricular septal defect or AVSD.
Pulmonary Valve Stenosis (PS), which 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.
Tetralogy of Fallot (ToF), which is a heart defect that combines 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 who use tests that may include electrocardiograms, wireless heart monitors and heart imaging such as a cardiac MRI. Medication to manage an abnormal heart rhythm may also be needed.
Single ventricle anomaly is a condition in which one of the heart’s ventricles is underdeveloped. This can be congenital or the result of another heart procedure such as the Fontan procedure (the surgical connection of a large vein from the lower body and veins from the liver to the lung arteries) or a similar procedure when one of the ventricles of the heart does not develop properly such as in cases of tricuspid atresia or pulmonary atresia.
Truncus arteriosus, which 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. Although always repaired during childhood, this heart defect requires regular follow-up and care by a cardiologist who uses testing to check the heart’s performance.
Marfan syndrome, which 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.