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Children's Heart Group


Structural Congenital Heart Disease

What is it?

Structural congenital heart disease is any structural or functional abnormality or defect of the heart or its major blood vessels present at birth. Most types of congenital heart disease obstruct the flow of blood in the heart or the vessels near it, or cause an abnormal flow of blood through the heart. Rarer types of congenital heart disease occur when the newborn has only one ventricle, when the pulmonary artery and the aorta come out of the same ventricle, or when one side of the heart is not completely formed. Kinds of congenital heart disease include atrial septal defect, coarctation of the aorta, tetralogy of Fallot, transposition of the great vessels, tricuspid atresia, and ventricular septal defect.

Who gets it?

Congenital heart disease is a major cause of neonatal distress and is the most common cause of death in the newborn other than problems related to prematurity. More than 25,000 infants (one out of every 125 to 150) are born with heart defects each year in the United States. The likelihood of having a child with a congenital heart disease increases if the mother or father, another child, or another relative had congenital heart disease.

What causes it?

Causes of congenital heart disease include genetic and environmental factors and lifestyle habits. Viral infections, such as German measles, can produce congenital heart disease. Other factors include a mother's excessive use of alcohol or taking illegal drugs while pregnant and exposure to certain anticonvulsant and dermatologic drugs during pregnancy. There are some genetic conditions, such as Down's syndrome, which affect multiple organs and can cause congenital heart disease.

What are the symptoms?

Symptoms of congenital heart disease include shortness of breath, cyanosis (bluish discoloration of the skin), heart murmur, respiratory infections that recur excessively, stunted growth, and limbs and muscles that are underdeveloped.

How is it diagnosed?

Echocardiography and cardiac magnetic resonance imaging are often used to diagnose congenital heart disease. An echocardiograph can detects valve and other heart problems by displaying an image of the heart formed by sound waves. Fetal echocardiography is used to diagnose congenital heart disease in utero. Cardiac magnetic resonance imaging, a scanning method that uses magnetic fields and radio waves, can also help physicians evaluate congenital heart disease.

What is the treatment?

Congenital heart disease is treated with drugs and/or surgery. Diuretics may be prescribed, which aid the baby in excreting water and salts, as well as digoxin, which strengthens the contraction of the heart, slows the heartbeat, and removes fluid from tissues. Surgical procedures seek to repair the defect as much as possible and improve the flow of blood and oxygen to the body. Smaller congenital heart defects can now be repaired in a cardiac catheterization lab instead of an operating room. Catheterization procedures include balloon atrial septostomy and balloon valvuloplasty. Surgical procedures include arterial switch, Damus-Kaye-Stansel procedure, Fontan procedure, Ross procedure, shunt procedure, and venous switch or intra-atrial baffle.

Self-care tips

Congenital heart disease cannot be predicted and many types cannot be prevented. However, general measures should be taken by the mother to ensure the birth of a healthy baby, such as avoiding excess alcohol, not taking drugs, and avoiding exposure to rubella and environmental toxins. Many types of congenital heart disease that would have been fatal can now be treated successfully, and research on diagnosing heart defects while the fetus is in the womb may lead to future treatment to correct defects before the baby is born.


This information has been designed as a comprehensive and quick reference guide written by our health care reviewers.  The health information written by our authors is intended to be a supplement to the care provided by your physician.  It is not intended nor implied to be a substitute for professional medical advice.

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This page was last updated on October 31, 2006
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