Neonates with hypoplastic left heart syndrome and their fate after staged palliation
Journal Title: Pediatric Review: International Journal of Pediatric Research - Year 2017, Vol 4, Issue 5
Abstract
Hypoplastic left heart syndrome (HLHS) is a rare congenital heart defect in which the left side of the heart is severely underdeveloped. Etiology of this condition remains unknown. After the labour, three-stage cardio-surgical treatment is necessary, however all of the procedures run a significant risk of complications. Depending on the general condition of the newborn, the first operation – Norwood procedure – may be carried out in different modifications. Complications are most common after this stage of palliation, and may include morbidities of both cardiogenic and non-cardiogenic etiology. First procedure is also characterized by the highest mortality rate. It is followed by Glenn procedure, usually performed on patients at the age of 3 to 6 months. Finally, HLHS patients undergo the Fontan operation, which is a method used commonly in cardiosurgical management of many single-ventricle lesions. A heart transplantation is an alternative method of treatment. Development of cardiac surgery significantly increased survival rate of children born with congenital heart defects, hypoplastic left heart syndrome among them. Nowadays, a high percentage of those patients may have hope to reach adulthood. Nevertheless, the surgical treatment still bears a great risk of complications and continually is considered to be mere palliation, as HLHS remains a lethal condition.
Authors and Affiliations
Baran K, Gilewska J, Gałuszka M, Surmiak P, Baumert M
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