How the heart works
The heart is divided into four hollow chambers, two on the right and two on the left. In performing its basic job — pumping blood throughout the body — the heart uses its left and right sides for different tasks.
The right side of the heart moves blood to the lungs through vessels called pulmonary arteries. In the lungs, blood picks up oxygen then returns to the heart's left side through the pulmonary veins. The left side of the heart then pumps the blood through the aorta and out to the rest of the body.
How heart defects develop
During the first six weeks of pregnancy, the heart begins taking shape and starts beating. The major blood vessels that run to and from the heart also begin to form during this critical time during gestation.
It's at this point in your baby's development that heart defects may begin to develop. Researchers aren't sure exactly what causes most of these defects, but they think genetics, certain medical conditions, some medications and environmental factors, such as smoking, may play a role.
Types of heart defects
There are many different types of congenital heart defects, falling mainly into these categories:
Oct. 25, 2015
Holes in the heart. Holes can form in the walls between heart chambers or between major blood vessels leaving the heart. These holes allow oxygen-rich and oxygen-poor blood to mix. If the holes are large and a lot of blood is mixed, the blood that ends up being circulated through your child's body is not carrying as much oxygen as normal.
Not having enough oxygen can cause your child's skin or fingernails to appear blue. Your baby may also develop signs of congestive heart failure, such as shortness of breath, irritability and leg swelling, because both oxygen-rich and oxygen-poor blood are flooding the lungs.
A ventricular septal defect is a hole in the wall between the right and left chambers on the lower half of the heart (ventricles). An atrial septal defect occurs when there's a hole between the upper heart chambers (atria).
Patent ductus arteriosus (PAY-tunt DUK-tus ahr-teer-e-O-sus) is a condition that causes an opening between the pulmonary artery (containing deoxygenated blood) and the aorta (containing oxygenated blood). A complete atrioventricular canal defect is a condition that causes a hole in the center of the heart.
Obstructed blood flow. When blood vessels or heart valves are narrow because of a heart defect, the heart must work harder to pump blood through them. Among the most common of this type of defect is pulmonary stenosis (stuh-NO-sis). This condition occurs when the valve that allows blood to pass from the right ventricle to the lungs via the pulmonary artery is too narrow to function properly.
Another type of obstructive defect is aortic stenosis. In this condition, the aortic valve, which allows blood to pass from the left ventricle out to the body via the aorta, is too narrow. Narrowed valves force the heart muscle to work harder, eventually leading to thickening and enlarging of the heart.
Abnormal blood vessels. Several congenital heart defects happen when blood vessels going to and from the heart don't form correctly, or they're not positioned the way they're supposed to be.
A defect called transposition of the great arteries is a condition caused by abnormal blood vessels. It occurs when the pulmonary artery and the aorta are on the wrong sides of the heart.
A condition called coarctation of the aorta happens when the main blood vessel supplying blood to the body is too narrow. Coarctation of the aorta causes high blood pressure.
Total anomalous pulmonary venous connection is a defect that occurs when blood vessels from the lungs attach to wrong area of the heart.
Heart valve abnormalities. If the heart valves can't open and close correctly, blood can't flow smoothly.
One example of this type of defect is called Ebstein's anomaly. In Ebstein's anomaly, the tricuspid valve — which is located between the right atrium and the right ventricle — is malformed and often leaks.
Another example is pulmonary atresia, in which the pulmonary valve is missing, causing abnormal blood flow to the lungs.
- An underdeveloped heart. Sometimes, a major portion of the heart fails to develop properly. For example, in hypoplastic left heart syndrome, the left side of the heart hasn't developed enough to effectively pump enough blood to the body.
- A combination of defects. Some infants are born with several heart defects. Tetralogy of Fallot (teh-TRAL-eh-jee of fuh-LOW) is a combination of four defects: a hole in the wall between the heart's ventricles, a narrowed passage between the right ventricle and pulmonary artery, a shift in the connection of the aorta to the heart, and thickened muscle in the right ventricle.
- Congenital heart defects. National Heart, Lung, and Blood Institute. http://www.nhlbi.nih.gov/health/health-topics/topics/chd/. Accessed Jan. 24, 2014.
- Bonow RO, et al. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 9th ed. Philadelphia, Pa.: Saunders Elsevier; 2012. http://www.clinicalkey.com. Accessed Jan. 24, 2014.
- Overview of congenital cardiovascular anomalies. The Merck Manuals: The Merck Manual for Health Care Professionals. http://www.merckmanuals.com/professional/pediatrics/congenital_cardiovascular_anomalies/overview_of_congenital_cardiovascular_anomalies.html?qt=Overview%20of%20Congenital%20Cardiovascular%20Anomalies&alt=sh. Accessed Dec. 22, 2013.
- Yuan S, et al. Congenital heart disease: Emerging themes linking genetics and development. Current Opinion in Genetics & Development. 2013;23:352.
- About congenital heart defects. American Heart Association. http://www.heart.org/HEARTORG/Conditions/CongenitalHeartDefects/AboutCongenitalHeartDefects/About-Congenital-Heart-Defects_UCM_001217_Article.jsp. Accessed Jan. 24, 2014.
- Facts about hypoplastic left heart syndrome. Centers for Disease Control and Prevention. http://www.cdc.gov/ncbddd/heartdefects/hlhs.html. Accessed Feb. 1, 2014.
- Longo DL, et al. Harrison's Online. 18th ed. New York, N.Y.: The McGraw-Hill Companies; 2012. http://www.accessmedicine.com/resourceTOC.aspx?resourceID=4. Accessed Feb. 1, 2014.
- Congenital heart defects. March of Dimes. http://www.marchofdimes.com/baby/congenital-heart-defects.aspx. Accessed Jan. 24, 2014.
- Congenital heart defects surgery. American Heart Association. http://www.heart.org/HEARTORG/Conditions/CongenitalHeartDefects/CareTreatmentforCongenitalHeartDefects/Congenital-Heart-Defects-Surgery_UCM_307729_Article.jsp. Accessed Jan. 24, 2014.
- Hochberg L, et al. Folic acid for prevention of neural tube defects. http://www.uptodate.com/home. Accessed Feb. 1, 2014.