Parents’ perceptions during the transition to home for their child with a congenital heart defect: How can we support families of children with hypoplastic left heart syndrome?

2017 ◽  
Vol 22 (3) ◽  
pp. e12185 ◽  
Author(s):  
Sarita March
Keyword(s):  
Hypoplastic Left Heart Syndrome ◽  
Congenital Heart Defect ◽  
Congenital Heart ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Heart Defect ◽  
Left Heart Syndrome

Hypoplastic Left Heart Syndrome: Natural History in a Geographically Defined Population

PEDIATRICS ◽  
1990 ◽  
Vol 85 (6) ◽  
pp. 977-983
Author(s):  
Cynthia D. Morris ◽  
Jacquelyn Outcalt ◽  
Victor D. Menashe
Keyword(s):  
Natural History ◽  
Hypoplastic Left Heart Syndrome ◽  
Congenital Heart ◽  
Heart Defects ◽  
Norwood Procedure ◽  
Secular Change ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
History Of ◽  
Left Heart Syndrome

Advances in surgical treatment of hypoplastic left heart syndrome with the Norwood procedure and cardiac transplantation have made essential the understanding of the natural history of hypoplastic left heart syndrome. In a geographically defined population, we ascertained the prevalence of hypoplastic left heart syndrome in children born in Oregon from 1971 through 1986. Clinical and anatomic data were extracted from the charts of the 98 affected children and the survival rate was calculated. Hypoplastic left heart syndrome occurred in 0.162 per 1000 live births in Oregon during this period. No syndrome complex was prevalent and 84% were free of other congenital malformations. However, there was an increased occurrence of congenital heart defects in first-degree relatives of probands with hypoplastic left heart syndrome. Of the affected children 15 ± 4% died on the first day of life, 70 ± 5% died within the first week, and 91 ± 3% died within 30 days. No secular change in survival occurred during the study. Palliation with the Norwood procedure was performed in 20 children. Although survival was significantly improved with this surgery (P = .01), the effect was observed principally through 30 days of life and only one of these children remains alive. Hypoplastic left heart syndrome is a lethal congenital heart defect in children and poses management and ethical dilemmas.

scholarly journals Spinal Anesthesia for Urologic Surgery in an Infant with Hemi-Fontan Physiology: A Case Report

2019 ◽  
Vol 4 (4) ◽  
Keyword(s):  
Case Report ◽  
Right Ventricle ◽  
Spinal Anesthesia ◽  
Congenital Heart Defect ◽  
Congenital Heart ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Fontan Physiology ◽  
Surgical Conditions ◽  
Left Heart Syndrome

Hypoplastic left heart syndrome is a congenital heart defect characterized by hypoplasia of left-sided heart structures and a single functional right ventricle. The resultant physiology poses significant challenges for the anesthesiologist when general anesthesia is necessary for these children. This case report describes an infant with hemi-Fontan physiology undergoing circumcision in which awake spinal anesthesia was used with favorable hemodynamic, respiratory, and surgical conditions. Written consent for publication was obtained from the patient’s parents.

scholarly journals Surgical palliation for an infant with hypoplastic left heart syndrome and congenital lobar emphysema: a case report

2021 ◽  
Vol 3 (1) ◽  
pp. 01-04
Author(s):  
Raj Sahulee
Keyword(s):  
Congenital Heart Disease ◽  
Hypoplastic Left Heart Syndrome ◽  
Congenital Heart ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Congenital Lobar Emphysema ◽  
First Case ◽  
Surgical Palliation ◽  
Lobar Emphysema ◽  
Left Heart Syndrome

Hypoplastic left heart syndrome is a form of congenital heart disease that is associated with significant lifelong morbidity and mortality. Although rarely reported together, the prognosis for patients with hypoplastic left heart syndrome and congenital anomalies of the airway or lung, such as congenital diaphragmatic hernia, has been shown to be very poor. The outcomes for patients with hypoplastic left heart syndrome and congenital lobar emphysema specifically is currently unknown. Reported herein is the first case of a child with a rare combination of hypoplastic left heart syndrome and congenital lobar emphysema, and a discussion of his presentation, diagnosis, management and outcome to date.

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