Cardiovascular System - Hypoplastic Left Heart

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LA89.3 Hypoplastic Left Heart Syndrome

 ICD-11 LA89 Functionally univentricular heart
LA89.3 Hypoplastic left heart syndrome
LA89 Functionally univentricular heart - describes a spectrum of congenital cardiovascular malformations in which the ventricular mass may not readily lend itself to partitioning that commits one ventricular pump to the systemic circulation, and another to the pulmonary circulation. A heart may be functionally univentricular because of its anatomy or because of the lack of feasibility or lack of advisability of surgically partitioning the ventricular mass. Common lesions in this category typically include double inlet right ventricle (DIRV), double inlet left ventricle (DILV), tricuspid atresia, mitral atresia, and hypoplastic left heart syndrome. Other lesions which sometimes may be considered to be a functionally univentricular heart include complex forms of atrioventricular septal defect, double outlet right ventricle, congenitally corrected transposition, pulmonary atresia with intact ventricular septum, and other cardiovascular malformations. Specific diagnostic codes should be used whenever possible, and not the term “functionally univentricular heart”.

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.


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


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

  • Congenital left heart obstruction: ethnic variation in incidence and infant survival[1] |To investigate the relationship between ethnicity and health outcomes among fetuses and infants with congenital left heart obstruction (LHO). A retrospective population-based review was conducted of fetuses and infants with LHO including all terminations, stillbirths and live births from 20 weeks' gestation in New Zealand over a 9-year period. Disease incidence and mortality were analysed by ethnicity and by disease type: hypoplastic left heart syndrome (HLHS), aortic arch obstruction (AAO), and aortic valve and supravalvular anomalies (AVSA). RESULTS: Critical LHO was diagnosed in 243 fetuses and newborns. There were 125 with HLHS, 112 with AAO and 6 with isolated AVSA. The incidence of LHO was significantly higher among Europeans (0.59 per 1000) compared with Māori (0.31 per 1000; p<0.001) and Pacific peoples (0.27 per 1000; p=0.002). Terminations were uncommon among Māori and Pacific peoples. Total case fatality was, however, lower in Europeans compared with other ethnicities (42% vs 63%; p=0.002) due to a higher surgical intervention rate and better infant survival. The perinatal and infant mortality rate was 82% for HLHS, 15% for AAO and 2% for AVSA."
  • Hypoplastic left heart: the next chapter in our unsolved problem[2] "The imperfections of the Fontan are well recognised but for now it is what we have. Let us hope the next chapter brings a new treatment paradigm for HLH."
  • Are There Head Volume Alterations at 11 to 14 Weeks in Fetuses with Congenital Heart Defects? A First Trimester Case Series[3] "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

Fontan Procedure

The Fontan procedure or Fontan–Kreutzer procedure is a surgical procedure used in children with univentricular hearts.

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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)[4]

"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." [5]

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. 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.
  2. Winlaw DS. (2018). Hypoplastic left heart: the next chapter in our unsolved problem. Heart , 104, 1476-1477. PMID: 29549091 DOI.
  3. 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.
  4. Tanner K, Sabrine N & Wren C. (2005). Cardiovascular malformations among preterm infants. Pediatrics , 116, e833-8. PMID: 16322141 DOI.
  5. 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.

Reviews

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.

Fillipps DJ & Bucciarelli RL. (2015). Cardiac evaluation of the newborn. Pediatr. Clin. North Am. , 62, 471-89. PMID: 25836709 DOI.

Wagner J & Abdel-Rahman SM. (2015). Oseltamivir-warfarin interaction in hypoplastic left heart syndrome: case report and review. Pediatrics , 135, e1333-6. PMID: 25917992 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

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Cite this page: Hill, M.A. (2019, July 16) 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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