Cyanotic heart disease

Injury	Disease	Nutrition	Poison

Symptoms	Surgery	Test	Special Topic

Heart, section through the middle

Cardiac catheterization

Heart, front view

Clubbing

Cyanotic heart disease

Cyanotic heart disease

Definition:

Cyanotic heart disease is a defect or group of defects in the structure or function of the heart or the great vessels, present at birth, consisting of abnormal blood flow from the right to the left part of the circulatory system (either at the level of the atria, the ventricles, or the great vessels).

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This abnormal communication (called right-to-left shunt) results in poor oxygenation of the body and therefore cyanosis (bluish coloration of the body).

Alternative Names:
Right-to-left cardiac shunt; Right-to-left circulatory shunt
Causes, incidence, and risk factors:

Cyanotic heart diseases are congenital heart defects (heart diseases present at birth) that result in low oxygen levels in the blood and cause the child's skin to look blue (cyanosis).

This bluish coloration is most often detected on the lips, fingers, and toes or during exercise. Some heart defects cause major problems immediately after birth, and some cause few if any problems until adulthood.

Not all congenital heart diseases are cyanotic. Congenital heart defects that cause cyanosis include the following:

Tetralogy of Fallot
Transposition of the great vessels
Ebstein's anomaly
Tricuspid atresia (a deformity of the tricuspid heart valve)
Total anomalous pulmonary venous return
Pulmonic stenosis
Truncus arteriosus
Hypoplastic left heart syndrome

Most congenital heart disease occurs as a birth defect limited only to the heart, but some conditions occur as part of genetic and chromosomal syndromes that affect many organs.

Some syndromes that include congenital heart disease (not all cyanotic) are Down syndrome, trisomy 13, Turner's syndrome, Marfan syndrome, Noonan syndrome, and Ellis-van Creveld syndrome.

Some congenital heart diseases are caused by drugs, chemical exposure, or infections during pregnancy. Fetal rubella, infection by cytomegallovirus (CMV), maternal alcohol use (fetal alcohol syndrome), lithium carbonate use (for manic-depressive disorder), and retinoic acid use (for acne) are examples of infections and drugs that can cause congenital heart disease.