Adult Congenital
Heart Center

Conditions That Cause Congenital Heart Disease

If you’re living with a congenital heart disease (CHD) you’re not alone. About 1 million adults in the U.S. are living with a congenital heart disease (CHD), according to the Adult Congenital Heart Association (ACHA). Many live full and active lives. Most need ongoing specialized care and monitoring throughout life.

You’ll find all the care you need at the Emory Adult Congenital Heart Center. It’s one of only a few programs of its kind in the U.S. — and the only one in Georgia — specializing in the diagnosis and treatment of adults with moderate and complex CHDs. Our multidisciplinary team offers diagnosis, advanced treatment, and coordinated, comprehensive care at one of the nation’s most respected academic medical centers. Your heart is in good hands.

Many congenital heart defects or conditions can cause congenital heart disease. Some of the most common include:

Atrial Septal Defects (ASDs)

An ASD is a hole in the wall that separates the two upper chambers of the heart. There are many different kinds of ASDs. Some ASDs only need follow-up observation, but many require surgical repair. In some cases, ASDs can be repaired with a minimally invasive procedure using catheters to place small closure devices within the heart.

Bicuspid Aortic Valve Disease

The aortic valve typically consists of three leaflets (tiny flaps of tissue that open and close). In bicuspid valve disease, only two leaflets are functional. This can lead to aortic valve stenosis (stiffening) and regurgitation (reverse blood flow). Many cases require valve replacement or replacement of part of the aorta.

Coartacation Of the Aorta

This is obstruction or narrowing of the aorta, usually beyond the left subclavian artery (the artery that supplies blood to the left arm). This condition can lead to high blood pressure and is often related to a bicuspid aortic valve. Even if your condition was repaired in childhood, you need ongoing follow-up to check for abnormal growth or re-narrowing of the aorta, or heart valve disease that requires surgery.

Ebstein’s Abnormality

This term refers to any of a number of defects of the tricuspid valve, which separates the upper right and lower right chambers of the heart. Ebstein’s anomaly can result in severe regurgitation (reverse blood flow), heart rhythm disorders and atrial septal defects. Some people with Ebstein’s anomaly require heart valve replacement, while others may only develop a mild heart rhythm disorder. Even if the defect has been repaired, lifelong monitoring and care is critical.

Single Ventricle Defects

A single ventricle defect means that only one chamber of the heart is large or strong enough to pump effectively. Children with single ventricle defects are often treated with the Fontan procedure, which allows oxygen-poor blood to bypass the heart and flow directly to the lungs. If you had the Fontan procedure as a child, you’ll need ongoing evaluation and care to check for heart rhythm disorders, reverse blood flow and other complications.

Tetralogy Of Fallot

One of the most common congenital heart defects in children, this complex condition results in low levels of oxygen in the blood. The low oxygen level can cause the skin to appear blue (cyanosis). Tetralogy of Fallot is often repaired in early childhood. In adults, it can lead to severe regurgitation (reverse blood flow) in the pulmonary valve, heart rhythm disorders and other complications. It’s important to have regular evaluations by an experienced team.

Transposition Of The Great Arteries

In this condition, the positions of the aorta and pulmonary artery are reversed, causing oxygen-rich blood to flow to the lungs and oxygen-poor blood to flow to the body. Transposition of the great arteries requires corrective surgery in the first weeks of life. Your needs as an adult depend on the type of repair you had in childhood. You’ll need regular screening for to make sure your blood is flowing correctly, to check for irregular heart rhythms and to assess left and right heart function.

Ventricular Septal Defects (VSDs)

A VSD is a hole in the wall that separates the two lower chambers of the heart. Some kinds of VSDs must be repaired in childhood. If you have a VSD that has not been repaired, you might be at risk for complications, such as low oxygen levels in the blood. You need regular screening by a team that can identify any problems and take steps to correct or manage them.

Whatever the cause and nature of your CHD, our team will create a care plan specific to your needs — now and into the future.

Congenital Heart Diseases and Defects: Complete List

For your reference, here is a full list of congenital heart diseases, including simple, moderately severe and complex. Simple CHDs usually don’t lead to other heart conditions. They require less care and follow-up than moderately severe or complex conditions.

Moderately severe and complex congenital heart diseases are more serious defects. These conditions generally require surgery and long-term management. If you have one of these conditions, you need an experienced team at a center dedicated to congenital heart defects. That’s what you’ll find at the Emory Adult Congenital Heart Center.

Simple Congenital Heart Disease

  • Isolated congenital aortic valve disease
  • Isolated congenital mitral valve disease (except parachute valve, cleft leaflet)
  • Isolated patent foramen ovale or small atrial septal defect
  • Isolated small ventricular septal defect (no associated lesions)
  • Mild pulmonic stenosis
  • Previously ligated or occluded ductus arteriosus
  • Repaired secundum or sinus venosus
  • Repaired atrial septal defect without residua
  • Repaired ventricular septal defect without residua
  • Moderately Severe Congenital Heart Disease includes:
  • Aorto-left ventricular fistulae
  • Anomalous pulmonary venous drainage (partial or total)
  • Atrioventricular canal defects (partial or complete)
  • Coarctation of the aorta
  • Ebstein’s anomaly’
  • Infundibular right ventricular outflow obstruction of significance
  • Ostium primum atrial septal defect
  • Patent ductus arteriosus (not closed)
  • Pulmonary valve regurgitation (moderate to severe)
  • Pulmonic valve stenosis (moderate to severe)
  • Sinus of Valsalva fistula/aneurysm
  • Sinus venosus atrial septal defect
  • Subvalvar or supravalvar aortic stenosis
  • Tetralogy of Fallot

Ventricular Septal Defects

  • Absent valve or valves
  • Aortic regurgitation
  • Coarctation of the aorta
  • Mitral disease
  • Right ventricular outflow tract obstruction
  • Straddling tricuspid/mitral valve
  • Subaortic stenosis
  • HOCM = hypertrophic obstructive cardiomyopathy

Complex Congenital Heart Disease

  • Conduits, valved or nonvalved
  • Cyanotic congenital heart (all forms)
  • Double-outlet ventricle
  • Eisenmenger syndrome
  • Fontan procedure
  • Mitral atresia
  • Single ventricle (also called double inlet or outlet, common or primitive)
  • Pulmonary atresia (all forms)
  • Pulmonary vascular obstructive diseases
  • Transposition of the great arteries
  • Congenitally corrected transposition of the great arteries
  • Tricuspid atresia
  • Truncus arteriosus/hemitruncus
  • Other abnormalities of atrioventricular or ventriculoarterial connection, such as crisscross heart, isomerism and heterotaxy syndromes
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