Rare Diseases News Briefs for Week of July 29, 2001
Two articles on hypoplastic left heart syndrome (HLHS): Prenatal diagnosis of HLHS helps reduce seizures and coma; the drug allopurinol helps infants with HLHS after heart surgery.Prenatal Diagnosis Of Heart Defect May Reduce Seizures And Coma In Newborns
Children's Hospital of Philadelphia
June 22, 2001
Philadelphia, Pa. - Fetal echocardiography is increasingly used to detect congenital heart defects before birth, although whether prenatal diagnosis benefits children with heart disease has been questioned. A new study by The Children's Hospital of Philadelphia suggests that newborns with a severe, life-threatening defect called hypoplastic left heart syndrome (HLHS) are less likely to have early neurologic problems, such as seizures or comas, if HLHS is diagnosed before birth.
The researchers found that prenatal detection of the heart defect allowed better treatment, such as delivering the baby in an advanced, high-risk nursery, and promptly providing the drug prostaglandin to maintain blood flow until the first stage of reconstructive surgery can be performed. Compared to newborns with HLHS diagnosed after birth, infants diagnosed prenatally were half as likely to experience seizure or coma prior to or within 6 weeks of their surgery. The research was reported in the June 2001 issue of the journal Pediatrics.
Drug Protects Infants With High-Risk Heart Defect From Neurologic And Cardiac Injury After Heart SurgeryChildren's Hospital of Philadelphia
July 5, 2001
Philadelphia, Pa. - A drug used to treat gout in adults also helps protect infants from neurological and cardiac injury after heart surgery for a high-risk birth defect, according to researchers at The Children's Hospital of Philadelphia. The drug, allopurinol, benefited infants with hypoplastic left heart syndrome (HLHS), in which a severely underdeveloped left ventricle is unable to pump enough blood to the body.
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