Today, almost 70% of babies with hypoplastic left heart syndrome (HLHS) will survive into adulthood, although significant long-term morbidity and mortality still exists. Prenatal diagnosis of HLHS is increasingly common, allowing improved counseling, and the potential for fetal intervention if indicated. Exciting progress continues to be made in the area of fetal diagnosis and intervention, specifically catheter intervention for intact atrial septum or severe aortic stenosis. Pediatric cardiologists should be keenly aware of the flaws of staged palliation for the treatment of HLHS, and need to keep abreast of the emerging data regarding fetal diagnosis and intervention.
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