Blue baby syndrome
Blue baby syndrome, also known as tetralogy of Fallot, is a congenital heart condition characterized by four specific defects that impede the flow of oxygenated blood to the body. This condition leads to a bluish discoloration of the skin and mucous membranes, known as cyanosis, which is evident at birth. The primary causes of this syndrome are congenital heart defects that may stem from maternal infections or certain medications taken during pregnancy, especially during the critical period of fetal heart development.
The four defects involved include narrowing of the pulmonary trunk leading to reduced blood flow to the lungs, a ventricular septal defect that allows mixing of oxygenated and deoxygenated blood, misalignment of the aorta, and thickening of the right ventricular muscle. Children with this condition often experience breathlessness following exertion. Fortunately, blue baby syndrome occurs in approximately one in every 1,500 births, and most of the associated defects are treatable through surgical intervention. Early surgical correction is crucial to prevent complications and ensure healthy growth for affected children.
Blue baby syndrome
ALSO KNOWN AS: Tetralogy of Fallot
ANATOMY OR SYSTEM AFFECTED: Circulatory system, heart
DEFINITION: A congenital heart disease consisting of four distinct defects that result in poorly oxygenated blood being delivered to the tissues, thereby causing a bluish discoloration of the skin and mucous membranes upon birth.
CAUSES: Congenital heart defects resulting in poorly oxygenated blood delivered to body tissue
SYMPTOMS: Bluish discoloration of skin and mucous membranes upon birth, breathlessness after exertion
DURATION: Temporary, once corrected
TREATMENTS: Surgery
Causes and Symptoms
Most congenital heart defects are thought to arise from maternal infections or the intake of certain drugs during pregnancy. The developing fetal heart is particularly susceptible to these effects during the second month of pregnancy, when major developmental changes are taking place.
Four distinct defects, called the tetralogy of Fallot, occur in infants suffering from blue baby syndrome. The first is a narrowing (stenosis) of the that takes blood to the lungs, where it is oxygenated. Narrowing of the pulmonary trunk is accompanied by a narrowing of the pulmonary semilunar valve, which resides in the pulmonary trunk. These two defects decrease blood flow to the lungs, resulting in a decreased of the blood. Second, the wall that separates the left and right of the heart fails to form completely, thereby allowing the poorly oxygenated blood in the right ventricle to mix with the well-oxygenated blood in the left ventricle and decreasing oxygen delivery to tissues. Third, the aorta, which typically opens from the left ventricle and carries oxygenated blood to the tissues, is misaligned such that it opens from both the left and right ventricle. Fourth, the right ventricular muscle is thickened, as this muscle must work harder to push blood through the narrowed pulmonary semilunar valve and pulmonary trunk.
The first three defects result in poorly oxygenated blood being delivered to tissues, causing the skin and mucous membranes to appear bluish within minutes after birth, a condition called cyanosis. Children with uncorrected blue baby syndrome suffer from breathlessness after any form of exertion.
Treatment and Therapy
The tetralogy of Fallot is relatively rare, occurring in about one in every 1,500 births. Most defects can be corrected surgically. Successful correction of these defects early in the child’s life prevents delayed growth and other complications of poor oxygen delivery.
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