• Congenital Heart Disease

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    Dr. Dennis W. Kim, Children’s Healthcare of Atlanta, describes the efforts of pediatric cardiologists and developments in the field that help children with congenital heart disease lead normal lives.

    Congenital heart disease refers to problems of the heart and major arteries that are present at birth. Those problems can relate to:

    • Heart structure, such as abnormal openings in heart walls
    • Irregular heart rhythm
    • Heart muscle deterioration

    While heart defect is the most common birth defect, affecting one in every 100 newborns, the chance of survival is high. Advances in interventional cardiology have played an important role in increased survival rates.

    Types of congenital heart disease include

  • Types of Congenital Heart Disease

    Abnormal Heart Rhythms

    To understand abnormal heart rhythms, or heartbeats, it can be helpful to first understand how your heart’s electrical system governs the heartbeat.

    Anomalous Coronary Arteries/Fistulas

    Anomalous coronary arteries are a rare occurrence when the arteries that supply the heart with blood (coronary arteries) do not come from the usual location on the large artery that arises from the heart (aorta).

    Anomalous Pulmonary Venous Return

    In total anomalous pulmonary venous return, one or more abnormal connections between the pulmonary veins and the heart causes oxygen-rich blood coming from the lungs to mix with poorly oxygenated blood (blue in color) before being pumped into the body. One result is that the child’s skin color may have a bluish cast (cyanosis).

    Aortic Stenosis/Bicuspid Aortic Valve

    In aortic valve stenosis, the valve between the lower left chamber of the heart (left ventricle) and the main artery carrying blood from the heart to the body is narrowed. Aortic stenosis can be due to: Fusion of the valve’s leaflets, the door-like structures that open to allow blood to flow out of the heart and close to prevent it from leaking backward, or A “doorway” for blood flow that is too small.

    Atrial Septal Defect (ASD)

    An atrial septal defect (ASD) is a hole in the “wall” (called the septum) that separates the heart’s two upper chambers (the atria). Of the thousands of babies born each year with a cardiovascular defect, 4 to 10 percent have septal defects in the wall between the atria or the ventricles (lower chambers), according to the American Heart Association.

    Atrioventricular Septal Defect (AVSD)

    Atrioventricular septal defect (AVSD), also referred to as endocardial cushion defects, consists of three defects in the heart: An atrial septal defect (ASD), a hole in the “wall” (septum) of the heart that separates the two upper chambers, the atria; an inlet ventricular septal defect (VSD), a hole in the septum between the heart’s two lower chambers (ventricles) and the existence of a common atrioventricular valve. In normal hearts, two valves – the mitral and tricuspid valves – help control blood flow between the upper and lower chambers of the heart. In AVSD, the two valves are combined, resulting in a common atrioventricular opening (or canal).

    Coarctation of the Aorta/Interrupted Aortic Arch

    Coarctation of the aorta is a congenital condition where there is narrowing of the aorta, the main blood vessel that carries oxygen-rich blood from the heart to the body. The narrowed segment (coarctation) is typically short, with the aorta opening up to normal size past the coarctation. However, the coarctation can cause problems with increased work on the heart and high blood pressure.

    Dilated Cardiomyopathy

    In dilated cardiomyopathy, the heart muscle weakens and the heart becomes enlarged. As the heart becomes stretched, its lower chambers (ventricles) are less able to pump blood efficiently. Eventually, the heart is not able to pump enough blood to the body and the lungs become congested - a condition called heart failure. Abnormal heart rhythms may also result when the heart becomes enlarged. 

    D-Transposition of the Great Arteries

    In transposition of the great arteries, the aorta and pulmonary artery are switched. Normally, the aorta carries blood from the heart’s left ventricle (lower left chamber) to the body, and the pulmonary artery carries blood from the heart’s right ventricle (lower right chamber) to the lungs.

    Ebstein’s Anomaly

    In Ebstein’s malformation, the tricuspid valve is abnormally developed. (The tricuspid valve is the gate between the right atrium, or upper chamber, and the right ventricle, or lower chamber, of the heart.) The abnormally developed valve may hinder blood flow into the right ventricle. It may also fail to close properly, allowing blood to leak backward (regurgitate) into the right atrium. Ebstein's anomaly may also be associated with pulmonary atresia, in which the pulmonary (lung) valve does not open or may be missing.

    Feeding a Baby Who Has Congenital Heart Disease

    The goal of feeding any infant or baby is to have steady and continued weight gain. This holds true for infants and babies with congenital heart disease (CHD),although appropriate weight gain may be more difficult.

    Hypertrophic Cardiomyopathy

    Hypertrophic cardiomyopathy (HCM) is a disease in which the muscle of the lower left chamber of the heart (the left ventricle) becomes abnormally thick and enlarged. HCM is a significant cause of both heart failure and sudden death. It is the most common cause of sudden cardiac death among athletes that are believed to be healthy.

    Hypoplastic Left Heart Syndrome

    In hypoplastic left heart syndrome (HLHS), the heart’s main pumping chamber (the left ventricle), is poorly developed. Due to its small size and limited ability to function, the left ventricle is not able to deliver enough blood to the body to sustain life. In this condition, other structures of the heart, including the mitral and aortic valves and the first portion of the aorta, are small (hypoplastic).

    L-Transposition of the Great Arteries

    L-transposition of the great arteries is a form of congenital heart disease in which blood circulation flows in the way it should, but serious problems may still exist or develop and require treatment. Read on for information about the characteristics of the defect at birth and the treatment and follow-up care that is necessary into adulthood.


    Pericarditis is a condition in which the sac-like tissue that surrounds the heart (pericardium) becomes inflamed, swollen or irritated. The amount of fluid that surrounds the heart within the pericardial sac can increase and cause a condition called a pericardial effusion. An electrocardiogram (EKG) may show signs of this, and an echocardiogram is the definitive test to assess for the build-up of fluid around the heart.

    Persistent Patent Ductus Ateriosus (PDA)

    Some babies develop a condition called persistent patent ductus arteriosus (PDA) soon after birth. It occurs when a vessel – critical to blood circulation before birth but not necessary after birth – does not close as it should.

    Pulmonary Atresia

    Pulmonary atresia exists as two main categories – pulmonary atresia with intact ventricular septum (PA/IVS) and pulmonary atresia with ventricular septal defect (PA/VSD).

    Pulmonary Stenosis

    In pulmonary stenosis, which is the second most common congenital heart disease, the heart’s pulmonary valve is thick and its opening is smaller than normal. As a result, blood cannot flow normally from the heart through the valve and to the lungs.

    Rheumatic Heart Disease (Rheumatic Fever)

    In rheumatic heart disease (also called rheumatic fever), the heart valves are damaged by substances (antibodies) that the body produces to fight streptococcal (strep) infection.

    Tetralogy of Fallot

    Four things contribute to create tetralogy of Fallot: Ventricular septal defect (VSD) – there is a large hole in the wall between the two lower pumping chambers Overriding aorta – the aorta sits over the hole between the bottom pumping chambers Narrowing in the outflow to the lungs – the pathway from the heart to the lungs (infundibulum) is smaller than normal, as are the pulmonary valve and main pulmonary artery Right ventricular hypertrophy – like any muscle, if the heart has to work harder, it becomes thicker.

    Tricuspid Atresia

    The normal pumping chambers of the heart (ventricles) must have an inflow valve to let blood in, a good-sized pumping chamber, and an outflow to allow blood to exit the chamber during contraction. In tricuspid atresia, the tricuspid valve, which lies between the heart’s upper right chamber (atrium) and lower right chamber (ventricle), does not develop.

    Truncus Arteriosus

    In the normal heart, two arteries emerge from the heart: The pulmonary artery carries blood from the right ventricle (lower right chamber) to the lungs, where it picks up oxygen and the aorta carries blood from the left ventricle to the rest of the body. In truncus arteriosus, a single blood vessel emerges from the heart, and then branches into a pulmonary artery and an aorta. This single vessel, called the truncus arteriosus, emerges from both ventricles – specifically from the hole in the wall between the two ventricles, called the ventricular septal defect (VSD).

    Vascular Ring/Sling

    The development of the heart and lung vessels is a very complex process that occurs early, mostly within the first 8 weeks in pregnancy. Complex rearrangements of the primitive vessels are made resulting in disappearance of some parts of the circulation as the embryo matures. A vascular ring is a congenital defect in which there is an abnormal formation of the aorta – the main artery that delivers blood from the heart to the body – and its associated blood vessels.

    Ventricular Septal Defect (VSD)

    A ventricular septal defect (VSD) is a hole in the wall that separates the heart’s left and right ventricles, the lower pumping chambers. The hole provides an opening for blood to cross the wall, or septum, from the left side to the right side of the heart. When this occurs, some of the blood in the left ventricle, which has just come back to the heart from picking up oxygen in the lungs, may go into the right ventricle and immediately back to the lungs.