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Congenital Heart Disease |
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Conditions and Treatments
Congenital heart disorders are those that are present at birth. These conditions may be inherited, may occur from a parent's exposure to a harmful substance or disease or may develop for no apparent reason. Some congenital defects are diagnosed before a child is born, others go unnoticed until a child is older and many may not produce symptoms until adulthood or even old age.
One of the ways doctors classify congenital heart defects is by the main symptoms they produce, including:
Cyanosis
Cyanosis is the medical term for turning blue, when the skin is slightly blue, gray or purple because of low hemoglobin, the iron-containing pigment in red blood cells. This occurs when blood isn't getting oxygen from the lungs or the oxygenated blood isn't being distributed to the rest of the body. Congenital heart defects that can result in cyanosis include:
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Anomalous Pulmonary Venous Connections — All the pulmonary veins from the lungs drain blood into the right atrium instead of the left.
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Double Inlet Left Ventricle — Both atria, or the two upper chambers of the heart, connect to only one ventricle and more than half of the overriding valve lies over the connected ventricle.
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Double Outlet Right Ventricle (DORV) — Both the aorta and the pulmonary artery arise from the right ventricle, often accompanied by an abnormal opening between the two ventricles called a ventricular septal defect (VSD).
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Ebstein Anomaly — When the tricuspid valve is displaced into the right ventricle and usually is malformed. Severe displacements and malformations may result in blood flowing backward.
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Pulmonary Atresia — This occurs when the pulmonary valve has failed to develop and blood can't flow to the lungs.
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Single Ventricle — In this condition, only one ventricle exists, instead of two.
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Tetralogy of Fallot — A combination of four heart defects:
- An abnormal opening that allows blood to go from the right to left ventricle without going through the lungs, called a ventricular septal defect (VSD)
- Narrowing below the pulmonary valve that partially blocks blood flow to the lungs, called pulmonary artery stenosis
- Overly muscular right ventricle, also called right ventricular hypertrophy
- The aorta is directly over the abnormal opening between the ventricles, also called an overriding aorta
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Transposition of Great Arteries — The two major arteries leaving the heart are connected to the wrong ventricles. Oxygenated blood from the lungs is pumped back into the lungs and unoxygenated blood from the body is pumped back into the body.
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Tricuspid Atresia — An absent or misshaped tricuspid valve that controls blood flow from the right atrium to the right ventricle.
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Truncus Arteriosus — When only one artery connects to the heart instead of two — the aorta, which takes oxygenated blood to the body from the heart, and the pulmonary artery, which takes blood from the heart to the lungs. There is nearly always a ventricular septal defect (VSD), an abnormal opening between the two ventricles. Correction involves closing the VSD and removing the pulmonary arteries from the aorta so they can be connected to the right ventricle.
Respiratory Distress
Respiratory distress or rapid breathing can be the result of:
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Anomalous Pulmonary Venous Connection — When all the pulmonary veins drain into the right atrium instead of the left atrium.
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Arteriovenous Fistulas — Abnormal passages between arteries and veins. Congenital fistulas usually involve blood vessels from the legs.
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Endocardial Cushion Defect — Also called atrioventricular septal defect, this condition is a combination of defects resulting in extra blood going through the lungs, causing symptoms of congestive heart failure. The defects are:
- An atrial septal defect or hole between the upper chambers of the heart
- A ventricular septal defect or opening in the wall between the heart's lower chambers
- An abnormal tricuspid or mitral valve
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Hypertrophic Cardiomypathy — Excessive thickening of the heart muscle. Cardiomyopathy refers to any heart abnormality with no apparent cause.
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Mitral Stenosis — A narrowing or blockage of the mitral valve that most commonly occurs in people who have had rheumatic fever. This narrowing interferes with blood flow from the left atrium to the right ventricle.
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Patent Ductus Arteriosus — The ductus arteriosis, the passageway that connects the aorta and pulmonary artery in the fetus, fails to close after birth.
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Pulmonary Valve Stenosis — A narrowing of the pulmonary valve that regulates the flow of blood from the right ventricle to the lungs. This may force the heart to pump harder and lead to an enlarged heart.
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Ventricular Septal Defect — An opening in the wall between the two lower chambers of the heart, resulting in abnormal blood flow to the lungs.
Blood Flow Problems
Blood flow problems that can produce lethargy, poor feeding, reduced urination and clamminess include:
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Aortic Coarctation — A narrowing of the aorta, the artery that sends oxygenated blood from the heart to the rest of the body.
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Aortic Stenosis — When the aortic valve between the left ventricle and aorta is deformed and too narrow to allow healthy blood flow.
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Atrial Septal Defect — A hole n between the two top chambers of the heart.
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Cor Triatriatum — A rare condition in which the left atrium is divided into two chambers by a membrane with one or more openings in it.
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Hypertrophic Cardiomypathy — An excessive thickening of the heart muscle. Cardiomyopathy refers to any heart abnormality with no apparent cause.
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Hypoplastic Left Heart Syndrome — When the left side of the heart is underdeveloped. Babies with this condition may appear normal at birth but will die within the first days or months of life without treatment, which consists of a heart transplant or a series of operations.
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Mitral Stenosis — A narrowing or blockage of the mitral valve that most commonly occurs in people who have had rheumatic fever. This narrowing interferes with blood flow from the left atrium to the right ventricle.
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Pulmonary Artery Stenosis — A congenital heart defect that causes a narrowing of the pulmonary artery, the large blood vessel that takes blood from the right ventricle of the heart to the lungs.
Transposition of Great Arteries — The two major arteries leaving the heart are connected to the wrong ventricles. The result is that oxygenated blood from the lungs is pumped back into the lungs and unoxygenated blood from the body is pumped back into the body.
Reviewed by health care specialists at UCSF Children's Hospital.
Last updated May 8, 2007
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