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    Congenital Heart Disease

    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.

  • Congenital Heart Disease

    Aortic Valve Stenosis

    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:

    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 Canal Defect (AVC)

    Atrioventricular canal defect (AVC) consists of three defects in the heart:

    Coarctation of the Aorta (CoA)

    Coarctation of the aorta occurs when the arch of the aorta – the part of the vessel that curves to connect the aorta’s ascending and descending sections – is narrowed. The aorta is the main vessel carrying blood from the heart to the rest of the body.

    Ebstein’s Malformation or 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.

    Hypoplastic Left Heart Syndrome (HLH)

    In hypoplastic left heart syndrome (HLH), the left ventricle, the heart’s main pumping chamber, 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. Also in this condition, other structures of the heart, including the mitral and aortic valves and the first portion of the aorta, are small (hypoplastic.)

    Patent Ductus Ateriosus (PDA)

    Some babies develop a condition called patent ductus arteriosus (PDA) soon after birth. It occurs when a vessel – critical to blood circulation before birth but having no function after birth – does not close as it should. Before birth, the vessel, called the ductus arteriosus, connects the two major arteries from the heart: the pulmonary artery, which carries blood from the heart to the lungs, and the aorta, which carries blood from the heart to the body. Before birth, the PDA diverts blood away from the lungs, which are collapsed, and carries it to the aorta to be distributed to the body.

    Pulmonary Atresia (PA)

    Children with pulmonary atresia are born without a functioning pulmonary valve – the valve located between the heart’s right ventricle (lower right chamber) and the pulmonary artery to the lungs. Without a functioning pulmonary valve, blood cannot flow properly from the heart’s right ventricle. Since blood cannot exit the right side of the heart, it instead passes through a hole (an atrial septal defect or patent foramen ovale) in the wall that separates the heart’s two upper chambers. The wall is called the atrial septum.

    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 through the valve and to the lungs.

    Tetralogy of Fallot

    In Tetralogy of Fallot, the pathway through which the blood leaves the right lower chamber (ventricle) is small, as is the pulmonary valve, making it hard for blood to pass through to the lungs. In addition, there is a large hole in the wall between the heart’s right and left ventricles (ventricle septal defect, or VSD). And, the aortic arch, the vessel that connects the ascending and descending sections of the aorta may be abnormally oriented toward the right, rather than the left, as it normally is. This is called “right aortic arch.”

    Total Anomalous Pulmonary Venous Return

    In total 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.

    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; the pulmonary artery carries blood from the heart’s right ventricle (lower right chamber) to the lungs.

    Tricuspid Atresia

    In tricuspid atresia, the tricuspid valve, which lies between the heart’s upper right chamber (atrium) and lower right chamber (ventricle), is abnormally developed. As a result, it prevents blood that is returning to the right atrium from the body from flowing into the right ventricle – the chamber that normally pumps blood to the lungs where it picks up oxygen.

    Truncus Arteriosus

    In truncus arteriosus, a single vessel emerges from the heart, 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).

    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.