Hypoplastic left heart syndrome : a review

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dc.contributor.authorGobergs, Roberts
dc.contributor.authorSalputra, Elza
dc.contributor.authorLubaua, Ingūna
dc.contributor.institutionRīga Stradiņš University
dc.date.accessioned2021-11-10T14:00:01Z
dc.date.available2021-11-10T14:00:01Z
dc.date.issued2016
dc.description.abstractBACKGROUND: Hypoplastic left heart syndrome (HLHS) is an etiologically multifactorial congenital heart disease affecting one in 5,000 newborns. Thirty years ago there were no treatment options for this pathology and the natural course of the disease led to death, usually within the first weeks of life. Recently surgical palliative techniques have been developed allowing for a five-year survival in more than half the cases. MATERIALS AND METHODS: We reviewed literature available on HLHS, specifically its anatomy, embryology and pathophysiology, and treatment. The Pubmed and ClinicalKey databases were searched using the key words hypoplastic left heart syndrome, foetal aortic valvuloplasty, foetal septoplasty, Norwood procedure, bidirectional Glenn procedure, Fontan procedure, hybrid procedure. The relevant literature was reviewed and included in the article. We reported a case from Children's Clinical University Hospital, Riga, to illustrate treatment tactics in Latvia. RESULTS: There are three possible directions for therapy in newborns with HLHS: orthotopic heart transplantation, staged surgical palliation and palliative non-surgical treatment or comfort care. Another treatment mode - foetal therapy - has arisen. Staged palliation and full Fontan circulation is a temporary solution, however, the only means for survival until heart transplantation. Fifty to 70% of patients who have gone through all three stages of palliation live to the age of five years. CONCLUSIONS: The superior mode of treatment is not yet clear and the management must be based on each individual case, the experience of each clinic, as well as the financial aspects and will of the patient's parents.en
dc.description.statusPeer reviewed
dc.format.extent13
dc.format.extent2780526
dc.identifier.citationGobergs, R, Salputra, E & Lubaua, I 2016, 'Hypoplastic left heart syndrome : a review', Acta medica Lituanica, vol. 23, no. 2, pp. 86-98. https://doi.org/10.6001/actamedica.v23i2.3325
dc.identifier.doi10.6001/actamedica.v23i2.3325
dc.identifier.issn1392-0138
dc.identifier.otherPubMedCentral: PMC5088741
dc.identifier.urihttps://dspace.rsu.lv/jspui/handle/123456789/6813
dc.language.isoeng
dc.relation.ispartofActa medica Lituanica
dc.rightsinfo:eu-repo/semantics/openAccess
dc.subjecthypoplastic left heart syndrome
dc.subjectNorwood procedure
dc.subjectFontan procedure
dc.subjecthybrid procedure
dc.subjecte, balloon valvuloplasty
dc.subject1.3. Anonymously reviewed scientific article published in a journal with an international editorial board and is available in another indexed database
dc.titleHypoplastic left heart syndrome : a reviewen
dc.type/dk/atira/pure/researchoutput/researchoutputtypes/contributiontojournal/article

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