Cardiovascular System - Hypoplastic Left Heart
|Embryology - 23 Jan 2019 Expand to Translate|
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- 1 Introduction
- 2 Some Recent Findings
- 3 Anatomy
- 4 Ultrasound
- 5 History
- 6 Treatment
- 7 International Classification of Diseases
- 8 Cardiovascular Abnormalities
- 9 References
- 10 External Links
- 11 Glossary Links
Characterized by hypoplasia (underdevelopment or absence) of the left ventricle obstructive valvular and vascular lesion of the left side of the heart.
ICD-10 Q23.4 Hypoplastic left heart syndrome Atresia, or marked hypoplasia of aortic orifice or valve, with hypoplasia of ascending aorta and defective develop-ment of left ventricle (with mitral valve stenosis or atresia).
Some Recent Findings
|More recent papers|
This table allows an automated computer search of the external PubMed database using the listed "Search term" text link.
<pubmed limit=5>Hypoplastic Left Heart</pubmed>
The images below show the variations in ventricular morphology to be found in the hypoplastic left heart syndrome. two primary variations, one with stenosis of the mitral valve (left hand panel), and the other with mitral atresia (right hand panel).
|Small globular left ventricle, lined with fibroelastosis, as found in mitral stenosis with aortic atresia.||Slit-like left ventricle, without fibroelastosis, found when there is mitral atresia and aortic atresia.|
|Ultrasound Hypoplastic Left Heart Syndrome
GA 17 - 18 week (second trimester) = week 15 - 16
| Hypoplastic Left|
|Page | Play|
|GA 19 week|
|The four chamber heart view shows a small left atrium (LA) and tiny left ventricle (LV).||The outflow tract view shows a large right ventricular outlow tract (RVOT) and tiny aorta.|
Colour Doppler exmination shows flow from atrium into ventricle on the right side but not the left.
- Links: Ultrasound
International Classification of Diseases
The International Classification of Diseases (ICD) World Health Organization's classification used worldwide as the standard diagnostic tool for epidemiology, health management and clinical purposes. This includes the analysis of the general health situation of population groups. It is used to monitor the incidence and prevalence of diseases and other health problems. Within this classification "congenital malformations, deformations and chromosomal abnormalities" are (Q00-Q99) but excludes "inborn errors of metabolism" (E70-E90).
Congenital malformations of the circulatory system (Q20-Q28)
- ICD-10 Code: Q21.1 Atrial septal defect
Q21 Congenital malformations of cardiac septa
Heart defects and preterm birth are the most common causes of neonatal and infant death. The long-term development of the heart combined with extensive remodelling and post-natal changes in circulation lead to an abundance of abnormalities associated with this system.
A UK study literature showed that preterm infants have more than twice as many cardiovascular malformations (5.1 / 1000 term infants and 12.5 / 1000 preterm infants) as do infants born at term and that 16% of all infants with cardiovascular malformations are preterm. (0.4% of live births occur at greater than 28 weeks of gestation, 0.9% at 28 to 31 weeks, and 6% at 32 to 36 weeks. Overall, 7.3% of live-born infants are preterm)
"Baltimore-Washington Infant Study data on live-born cases and controls (1981-1989) was reanalyzed for potential environmental and genetic risk-factor associations in complete atrioventricular septal defects AVSD (n = 213), with separate comparisons to the atrial (n = 75) and the ventricular (n = 32) forms of partial AVSD. ...Maternal diabetes constituted a potentially preventable risk factor for the most severe, complete form of AVSD." 
In addition, there are in several congenital abnormalities that exist in adults (bicuspid aortic valve, mitral valve prolapse, and partial anomalous pulmonary venous connection) which may not be clinically recognized.
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Search Pubmed: Search PubMed
External Links Notice - The dynamic nature of the internet may mean that some of these listed links may no longer function. If the link no longer works search the web with the link text or name. Links to any external commercial sites are provided for information purposes only and should never be considered an endorsement. UNSW Embryology is provided as an educational resource with no clinical information or commercial affiliation.
- OMIM Hypoplastic Left Heart Syndrome 1 | Hypoplastic Left Heart Syndrome 2
- Medline Plus Hypoplastic left heart syndrome
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Cite this page: Hill, M.A. (2019, January 23) Embryology Cardiovascular System - Hypoplastic Left Heart. Retrieved from https://embryology.med.unsw.edu.au/embryology/index.php/Cardiovascular_System_-_Hypoplastic_Left_Heart
- © Dr Mark Hill 2019, UNSW Embryology ISBN: 978 0 7334 2609 4 - UNSW CRICOS Provider Code No. 00098G