Cardiovascular System - Hypoplastic Left Heart

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Introduction

Hypoplastic Left Heart cartoon
Hypoplastic Left Heart

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).


Heart Abnormal: Tutorial Abnormalities | atrial septal defects | double outlet right ventricle | hypoplastic left heart | patent ductus arteriosus‎ | transposition of the great vessels | Tetralogy of Fallot | ventricular septal defects | coarctation of the aorta | Category ASD | Category PDA | Category ToF | Category VSD | ICD10 - Cardiovascular | ICD11


Cardiovascular Links: cardiovascular | Heart Tutorial | Lecture - Early Vascular | Lecture - Heart | Movies | 2016 Cardiac Review | heart | coronary circulation | heart valve | heart rate | Circulation | blood | blood vessel | blood vessel histology | heart histology | Lymphatic | ductus venosus | spleen | Stage 22 | cardiovascular abnormalities | OMIM | 2012 ECHO Meeting | Category:Cardiovascular
Historic Embryology - Cardiovascular 
1902 Vena cava inferior | 1905 Brain Blood Vessels | 1909 Cervical Veins | 1909 Dorsal aorta and umbilical veins | 1912 Heart | 1912 Human Heart | 1914 Earliest Blood-Vessels | 1915 Congenital Cardiac Disease | 1915 Dura Venous Sinuses | 1916 Blood cell origin | 1916 Pars Membranacea Septi | 1919 Lower Limb Arteries | 1921 Human Brain Vascular | 1921 Spleen | 1922 Aortic-Arch System | 1922 Pig Forelimb Arteries | 1922 Chicken Pulmonary | 1923 Head Subcutaneous Plexus | 1923 Ductus Venosus | 1925 Venous Development | 1927 Stage 11 Heart | 1928 Heart Blood Flow | 1935 Aorta | 1935 Venous valves | 1938 Pars Membranacea Septi | 1938 Foramen Ovale | 1939 Atrio-Ventricular Valves | 1940 Vena cava inferior | 1940 Early Hematopoiesis | 1941 Blood Formation | 1942 Truncus and Conus Partitioning | Ziegler Heart Models | 1951 Heart Movie | 1954 Week 9 Heart | 1957 Cranial venous system | 1959 Brain Arterial Anastomoses | Historic Embryology Papers | 2012 ECHO Meeting | 2016 Cardiac Review | Historic Disclaimer

Some Recent Findings

  • Are There Head Volume Alterations at 11 to 14 Weeks in Fetuses with Congenital Heart Defects? A First Trimester Case Series[1] "This study aims to assess head volume (HV) alterations at 11 to 14 weeks in fetuses with congenital heart defects (CHD). Methods A retrospective case-control study on 100 normal and 26 CHD fetuses was conducted. ...Despite the small sample size, our case series suggests that alterations in HV may potentially be apparent as early as 11 to 14 weeks in CHD fetuses, particularly those with HLH. Larger prospective studies are needed to validate our findings."
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Anatomy

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).

Anderson2016-fig44a.jpg Anderson2016-fig44b.jpg
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.

Fig 44

Ultrasound

Ultrasound - Hypoplastic left heart syndrome 01.jpg Ultrasound Hypoplastic Left Heart Syndrome

GA 17 - 18 week (second trimester) = week 15 - 16


  • LA - left atria
  • RA - right atria
  • LV - left ventricle
  • RV - right ventricle
Hypoplastic left heart syndrome movie icon.jpg
 ‎‎Hypoplastic Left
Heart Syndrome
Page | Play
GA 19 week
Ultrasound - Hypoplastic left heart syndrome 02.jpg Ultrasound - Hypoplastic left heart syndrome 03.jpg
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.

Ultrasound - Hypoplastic left heart syndrome 04.jpg

Colour Doppler exmination shows flow from atrium into ventricle on the right side but not the left.


Links: Ultrasound

History

Treatment

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

Cardiovascular Abnormalities

Data shown as a percentage of all major abnormalities based upon published statistics using the same groupings as Congenital Malformations Australia 1981-1992 P. Lancaster and E. Pedisich ISSN 1321-8352.

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)[2]

"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." [3]

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.


References

  1. <pubmed>27308099</pubmed>
  2. <pubmed>16322141</pubmed>
  3. <pubmed>11241431</pubmed>

Reviews

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Articles

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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

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© Dr Mark Hill 2019, UNSW Embryology ISBN: 978 0 7334 2609 4 - UNSW CRICOS Provider Code No. 00098G