Cardiovascular System - Hypoplastic Left Heart
|Embryology - 3 Jul 2020 Expand to Translate|
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LA89.3 Hypoplastic Left Heart Syndrome
|ICD-11 LA89 Functionally univentricular heart|
|LA89.3 Hypoplastic left heart syndrome
Characterized by hypoplasia (underdevelopment or absence) of the left ventricle obstructive valvular and vascular lesion of the left side of the heart.
Classified within ICD-11 as LA89 Functionally univentricular heart: LA89.0 Double inlet atrioventricular connection | LA89.1 Tricuspid atresia | LA89.2 Mitral atresia | LA89.3 Hypoplastic left heart syndrome
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.
Search term: Hypoplastic Left Heart
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
The Fontan procedure or Fontan–Kreutzer procedure is a surgical procedure used in children with univentricular hearts.
Search PubMed: Fontan procedure
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.
- Cloete E, Sadler L, Bloomfield FH, Crengle S, Percival T & Gentles TL. (2019). Congenital left heart obstruction: ethnic variation in incidence and infant survival. Arch. Dis. Child. , , . PMID: 30824490 DOI.
- Winlaw DS. (2018). Hypoplastic left heart: the next chapter in our unsolved problem. Heart , 104, 1476-1477. PMID: 29549091 DOI.
- Abu-Rustum RS, Ziade MF, Abu-Rustum SE & Daou LS. (2016). Are There Head Volume Alterations at 11 to 14 Weeks in Fetuses with Congenital Heart Defects? A First Trimester Case Series. AJP Rep , 6, e232-8. PMID: 27308099 DOI.
- Tanner K, Sabrine N & Wren C. (2005). Cardiovascular malformations among preterm infants. Pediatrics , 116, e833-8. PMID: 16322141 DOI.
- Loffredo CA, Hirata J, Wilson PD, Ferencz C & Lurie IW. (2001). Atrioventricular septal defects: possible etiologic differences between complete and partial defects. Teratology , 63, 87-93. PMID: 11241431 <87::AID-TERA1014>3.0.CO;2-5 DOI.
Ciocca L, Digilio MC, Lombardo A, D'Elia G, Baban A, Capolino R, Petrocchi S, Russo S, Sirleto P, Roberti MC, Marino B, Angioni A & Dallapiccola B. (2015). Hypoplastic left heart syndrome and 21q22.3 deletion. Am. J. Med. Genet. A , 167A, 579-86. PMID: 25663264 DOI.
Smith JB & Vernon-Levett P. (1993). Care of infants with hypoplastic left heart syndrome. AACN Clin Issues Crit Care Nurs , 4, 329-39. PMID: 8489880
Norwood WI. (1989). Hypoplastic left heart syndrome. Cardiol Clin , 7, 377-85. PMID: 2659180
Search Pubmed: Search PubMed
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- 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. (2020, July 3) 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 2020, UNSW Embryology ISBN: 978 0 7334 2609 4 - UNSW CRICOS Provider Code No. 00098G