Congenital Disease
I. Incidence and Classification
II. Ventricular Septal Defect
III. Patent Ductus Arteriosus
IV. Aortic Stenosis
V. Bicuspid Aortic Valve
VI. Coarctation of the Aorta
VII. Other Congenital Anomalies
VIII. Congenital Cyanotic Heart Disease
IX. Pregnancy and Congenital Heart Disease
GLOSSARY
autograft a tissue graft transferred from one part of the patients
the body to another part. commissures a site of union of corresponding parts, especially
the sites of junction between the adjacent cusps of the heart
valves. cyanosis purplish-blue discoloration of the lips, tongue, mucous
membranes, ear lobes, extremities, fingers, and toes. endocarditis infection on deformed or damaged valves in the
heart, or at the site of a hole in the heart (ventricular septal
defect). heterograft a graft of tissue taken from a donor of one species
and grafted into a recipient of another species, also called a
xenograft. homograft a graft of tissue taken from a donor of the same
species as the recipient. hypoxemia severe lack of oxygen in the blood. ischemia temporary lack of blood and oxygen to an area of cells,
for example, the heart muscle, usually due to severe obstruc¬tion of the artery supplying blood to this area of cells. murmur a blowing sound heard with a stethoscope usually
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- I. INCIDENCE AND CLASSIFICATION
The incidence of moderate and severe forms of congeni¬tal heart disease (CHD) is about 6 per 1000 live births, but it is 19 per 1000 live births if the potentially serious bicuspid aortic valve is included. If very small muscular ventricular septal defects (VSDs) are included, all forms increase to 75 per 1000 live births. Although several classifications are used, the oldest and most meaningful is the division into cyanotic and noncyanotic CHD. - II. VENTRICULAR SEPTAL DEFECT
Ventricular septal defects are the most common form of congenital heart disease. If all newborns are examined about 3% would reveal tiny muscular VSDs. Spontaneous closure occurs by age 3 in greater than 50% of patients born with VSDs. Still, VSDs after age 2 represent the most common lesion if bicuspid aortic valves are not included in the equation. Occasionally children, however, may not experience spontaneous closure up until age 10. - III. PATENT DUCTUS ARTERIOSUS
Patent ductus arteriosus is a common lesion occurring in approximately 10% of infants, about the same incidence as ASD. In the fetus a widely opened, patent vessel connects the pulmonary trunk to the descending aorta. This patent vessel is called a patent ductus arteriosus, (see Fig. 2). Thus, the output of deoxygenated blood from the right ventricle bypasses the unexpanded lungs of the fetus. Blood is propelled form the right ventricle into the pulmonary trunk and through the patent ductus and enters the descending aorta where it is carried to the fetal organ of oxygenation, the placenta abnormality. In the preterm infant the patent ductus may cause minor problems, but if other serious congen¬ital malformations coexist the shunting of blood through the ductus provides a unique mechanism that can be lifesaving. - IV. AORTIC STENOSIS
The normal aortic valve has three leaflets. With congenital aortic stenosis the valve is often bicuspid (two leaflets). The aortic valve leaflets are thickened and fusion occurs at the commissures, which results in obstruction to the valve so that blood flow from the left ventricle into the aorta is restricted, (see figures in the chapter Anatomy of the Heart and Circulation for normal aortic valve and heart structures). Severe or moderate degrees of aortic stenosis can be life threatening and surgical correction is necessary. Percutaneous balloon aortic valvuloplasty may delay surgical correction of the deformity in some individuals. Bacterial endocarditis must be prevented with antibiotic coverage. - VI. COARCTATION OF THE AORTA
Coarctation of the aorta is a congenital narrowing of the aorta as the artery winds its way from the top of the heart. The condition may be discovered when the child is an infant if the coarctation is severe or if an astute physician correctly interprets the signs. About 80% of children with coarctation have a mild coarctation or develop an extensive corollary system of vessels that carry blood past the coarctation. These children may have no symptoms until they reach adolescence; some children have no symptoms until they reach the age of 15–30. - VII. OTHER CONGENITAL ANOMALIES
A. Coronary Artery Anomalies in Adults - VIII. CONGENITAL CYANOTIC HEART DISEASE
Congenital heart disease is a general term that refers to defects of the heart that occur during the development of the fetus. They are present at birth but may be discovered much later. The exact causes of congenital heart disease, which occurs in about 0.8% of live births, is unknown. Approximately 10% of all congenital cardiac defects can be accounted for by chromosomal aberrations or genetic mutations or transmission. Down syndrome is the most common chromosome aberration and occurs in about 1 in every 700 births; the risk rises steeply if the mother is over age 35 and is as high as 4% for women over age 44. In this condition, an atrial septal defect (ASD), a hole in the septum dividing the top chambers of the heart is common. - BIBLIOGRAPHY
Fawzy, M. E., Awad, M., Hassan, W. et al. Long-term outcome (up to