Functionally univentricular hearts and Fontan circulation

2011 ◽  
Keyword(s):  
Hypoplastic Left Heart Syndrome ◽  
Congenital Heart ◽  
Complex Form ◽  
Fontan Circulation ◽  
Univentricular Heart ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Ventricular Cavity ◽  
Staged Approach ◽  
Left Heart Syndrome

The functionally univentricular heart 172Staged approach to achieve definitive palliation 174Fontan circulation 176Hypoplastic left heart syndrome (HLHS) 186Rare and highly complex form of cyanotic congenital heart disease.The term describes a variety of cardiac malformations in which: • There is functionally a single ventricular cavity....

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 Noninvasive Imaging in Interventional Cardiology: Hypoplastic Left Heart Syndrome

2021 ◽  
Vol 8 ◽  
Author(s):  
Hannah Bellsham-Revell
Keyword(s):  
Hypoplastic Left Heart Syndrome ◽  
Pulmonary Arteries ◽  
Interventional Cardiology ◽  
Systemic Circulation ◽  
Fontan Circulation ◽  
Active Management ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Systemic Right Ventricle ◽  
Left Heart Syndrome

Hypoplastic left heart syndrome (HLHS) is a spectrum of left heart underdevelopment leaving the left side unable to support the systemic circulation. If active management is pursued, then the condition is managed with staged palliation to the Fontan circulation, leaving a systemic right ventricle. Through all surgical stages, and even after completion of Fontan, there are multiple areas that may require intervention, most frequently the branch pulmonary arteries which are essential to a successful Fontan circulation. Echocardiography is the mainstay of assessment, but there is an increasing use of magnetic resonance imaging (MRI) and computed tomography (CT) particularly in relation to extracardiac structures which can be more challenging with echocardiography. Both MRI and CT require set-up, experience and training, and usually sedation or anesthetic in smaller children, but can provide excellent imaging to guide interventions. Cardiac MRI is also able to quantify right ventricular (RV) function which can be challenging on echocardiography. This article describes the modalities available and their use in assessing patients with HLHS prior to catheter interventions.

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