Neonates with hypoplastic left heart syndrome and their fate after staged palliation

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

Keywords

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  • EP ID EP281842
  • DOI 10.17511/ijpr.2017.05.02
  • Views 119
  • Downloads 0

How To Cite

Baran K, Gilewska J, Gałuszka M, Surmiak P, Baumert M (2017). Neonates with hypoplastic left heart syndrome and their fate after staged palliation. Pediatric Review: International Journal of Pediatric Research, 4(5), 297-303. https://europub.co.uk/articles/-A-281842