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2016 ; 23
(2
): 86-98
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Hypoplastic left heart syndrome: a review
#MMPMID28356795
Gobergs R
; Salputra E
; Lubaua I
Acta Med Litu
2016[]; 23
(2
): 86-98
PMID28356795
show ga
BACKGROUND: 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.
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