Natural and modified history of complete atrioventricular septal defect - a 17 year study

Article Abstract:

The atrioventricular septum is a wall of tissue that separates the atria, the upper chambers of the heart, from the lower heart chambers called the ventricles. A birth defect in the atrioventricular septum may lead to obstruction in the blood vessels of the lungs. One study suggested that the survival of patients with an atrioventricular septal defect at six months, two years, and five years was 50, 15, and 4 percent, respectively. However, the quality of life of these patients is limited. Long-term survival may be improved by surgery to repair the defect. Fibrosis, the formation of fiber-like debris, may occur in the blood vessels, and surgery should be carried out at an early stage of the disease. However, many children may not be eligible for corrective surgery because they have obstructed blood vessels in their lungs. Disease of the blood vessels may develop more rapidly in patients with Down's syndrome, who account for a major proportion of patients with atrioventricular septal defect. The development and outcome of this malformation were assessed in 103 cases of atrioventricular septal defect. Down's syndrome was diagnosed in 76 children, and 67 children underwent complete surgical repair of the defect. Between 1971 and 1982, corrective surgery was performed at an average of 23 months of age and was associated with a death rate of almost 89 percent. Between 1983 and 1988, the defect was corrected at approximately 13 months of age and was associated with a death rate of 43 percent. Of 22 patients who survived complete corrective surgery, only eight patients were alive 10 years later and all had developed obstructive disease of the blood vessels in the lungs. These findings show the importance of early complete repair of atrioventricular septal defect. (Consumer Summary produced by Reliance Medical Information, Inc.)

Abnormalities, Complications and side effects, Down syndrome, Heart septum

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Who's in CHARGE? Multidisciplinary management of patients with CHARGE association

Article Abstract:

CHARGE association is an acronym that stands for colobomata (an eye abnormality), heart defect, choanal atresia (a defect blocking the passageway between the nose and the throat), developmental retardation, genital hypoplasia (small genitals), and ear abnormalities. Kidney abnormalities, facial palsy, cleft defects, and abnormalities of the esophagus can also be present. The treatment, survival, and outcome of 50 children with CHARGE association is reported. An average of two to three surgical procedures were required in each patient. Postoperative death was high, particularly in those patients who received more than three general anesthetics. Most of the children died in the first year of life (13 children). The survival of children with CHARGE was poor if one of the following features was present: a heart defect causing cyanosis (blue skin color from a lack of oxygen), choanal atresia, or an abnormal communication between the trachea and the esophagus (tracheo-esophageal fistula). The main cause of death was the breathing in of secretions caused by the lack of coordination between the throat and the larynx. Since CHARGE association is a multisystem disease, a multidisciplinary treatment team lead by a pediatric and cardiac surgeon is required. (Consumer Summary produced by Reliance Medical Information, Inc.)

Causes of, Nasal fossa

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Poor Sensitivity of Routine Fetal Anomaly Ultrasound Screening for Antenatal Detection of Atrioventricular Septal Defect

Article Abstract:

The antenatal diagnosis of atrioventricular septal defect is poor especially for infants with trisomy 21.

United Kingdom, Product information, Evaluation, Testing, Congenital heart disease, Congenital heart defects, Echocardiography, Prenatal ultrasonography, Ultrasonics in obstetrics

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