Hypoplastic left heart syndrome
Clausen, Henning LU
(2015)
In Paediatrics and Child Health (United Kingdom)
25(1).
p.18-22
- Abstract
Hypoplastic left heart syndrome (HLHS) represents a severe spectrum of congenital cardiac malformations, which lead to systemic blood supply being dependent on patency of the arterial duct after birth. Antenatal diagnosis, advances in postnatal care and modifications of surgical palliation strategies have led to improved outcomes with the majority of patients born in the current era being expected to live into adulthood. This review discusses the surgical treatment strategies for HLHS and the wider implications these may have on the wellbeing of patients and families. The 'Fontan' circulation constitutes a common endpoint for HLHS patients who often experience physiological circulatory compromise rather than pump failure of the... (More)
Hypoplastic left heart syndrome (HLHS) represents a severe spectrum of congenital cardiac malformations, which lead to systemic blood supply being dependent on patency of the arterial duct after birth. Antenatal diagnosis, advances in postnatal care and modifications of surgical palliation strategies have led to improved outcomes with the majority of patients born in the current era being expected to live into adulthood. This review discusses the surgical treatment strategies for HLHS and the wider implications these may have on the wellbeing of patients and families. The 'Fontan' circulation constitutes a common endpoint for HLHS patients who often experience physiological circulatory compromise rather than pump failure of the univentricular heart. Heart transplantation has been performed in HLHS, but donor shortage in countries such as the United Kingdom make this a viable option only for a few patients. Protein losing enteropathy and plastic bronchitis may be signs of physiological failure in 'Fontan' patients and require specialist input. Family-centred care should enable paediatricians to build relationship outside specialised cardiac centres and optimise healthcare experiences and outcomes through multidisciplinary care systems for these vulnerable patients.
(Less)
- author
- Clausen, Henning
LU
- publishing date
- 2015-01-01
- type
- Contribution to journal
- publication status
- published
- subject
- keywords
- Aortic atresia, Congenital heart disease, Norwood procedure, Univentricular circulation
- in
- Paediatrics and Child Health (United Kingdom)
- volume
- 25
- issue
- 1
- pages
- 18 - 22
- publisher
- Churchill Livingstone
- external identifiers
-
- scopus:84919482011
- ISSN
- 1751-7222
- DOI
- 10.1016/j.paed.2014.07.006
- language
- English
- LU publication?
- no
- additional info
- Publisher Copyright: © 2014 Elsevier Ltd.
- id
- 1e44e219-2f44-4fb2-8450-788acee3413f
- date added to LUP
- 2025-08-08 18:29:16
- date last changed
- 2025-08-11 08:17:08
@article{1e44e219-2f44-4fb2-8450-788acee3413f,
abstract = {{<p>Hypoplastic left heart syndrome (HLHS) represents a severe spectrum of congenital cardiac malformations, which lead to systemic blood supply being dependent on patency of the arterial duct after birth. Antenatal diagnosis, advances in postnatal care and modifications of surgical palliation strategies have led to improved outcomes with the majority of patients born in the current era being expected to live into adulthood. This review discusses the surgical treatment strategies for HLHS and the wider implications these may have on the wellbeing of patients and families. The 'Fontan' circulation constitutes a common endpoint for HLHS patients who often experience physiological circulatory compromise rather than pump failure of the univentricular heart. Heart transplantation has been performed in HLHS, but donor shortage in countries such as the United Kingdom make this a viable option only for a few patients. Protein losing enteropathy and plastic bronchitis may be signs of physiological failure in 'Fontan' patients and require specialist input. Family-centred care should enable paediatricians to build relationship outside specialised cardiac centres and optimise healthcare experiences and outcomes through multidisciplinary care systems for these vulnerable patients.</p>}},
author = {{Clausen, Henning}},
issn = {{1751-7222}},
keywords = {{Aortic atresia; Congenital heart disease; Norwood procedure; Univentricular circulation}},
language = {{eng}},
month = {{01}},
number = {{1}},
pages = {{18--22}},
publisher = {{Churchill Livingstone}},
series = {{Paediatrics and Child Health (United Kingdom)}},
title = {{Hypoplastic left heart syndrome}},
url = {{http://dx.doi.org/10.1016/j.paed.2014.07.006}},
doi = {{10.1016/j.paed.2014.07.006}},
volume = {{25}},
year = {{2015}},
}