Cardiovascular System - Hypoplastic Left Heart: Difference between revisions
mNo edit summary |
mNo edit summary |
||
(20 intermediate revisions by the same user not shown) | |||
Line 1: | Line 1: | ||
{{Header}} | {{Header}} | ||
=LA89.3 Hypoplastic Left Heart Syndrome= | |||
{| | |||
|-bgcolor="FFCC00" | |||
! {{ICD-11}} {{ICD11weblink}}5417233 LA89 Functionally univentricular heart] | |||
|-bgcolor="FEF9E7" | |||
| {{ICD11weblink}}1811800027 '''LA89.3''' Hypoplastic left heart syndrome] | |||
:[https://icd.who.int/browse11/l-m/en#/http://id.who.int/icd/entity/5417233 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== | ==Introduction== | ||
[[File:Hypoplastic Left Heart.jpg|thumb|300px|alt=Hypoplastic Left Heart cartoon|Hypoplastic Left Heart]] | [[File:Hypoplastic Left Heart.jpg|thumb|300px|alt=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. | 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 {{ICD11weblink}}5417233 LA89 Functionally univentricular heart]: LA89.0 Double inlet atrioventricular connection | LA89.1 Tricuspid atresia | LA89.2 Mitral atresia | LA89.3 Hypoplastic left heart syndrome | |||
Line 18: | Line 26: | ||
|-bgcolor="F5FAFF" | |-bgcolor="F5FAFF" | ||
| | | | ||
* '''Are There Head Volume Alterations at 11 to 14 Weeks in Fetuses with Congenital Heart Defects? A First Trimester Case Series''' | * '''Congenital left heart obstruction: ethnic variation in incidence and infant survival'''{{#pmid:30824490|PMID30824490}} |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'''{{#pmid:29549091|PMID29549091}} "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'''{{#pmid:27308099|PMID27308099}} "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." | |||
|} | |} | ||
{| class="wikitable mw-collapsible mw-collapsed" | {| class="wikitable mw-collapsible mw-collapsed" | ||
! More recent papers | ! More recent papers | ||
|- | |- | ||
| [[File:Mark_Hill.jpg|90px|left]] {{Most_Recent_Refs}} | | [[File:Mark_Hill.jpg|90px|left]] {{Most_Recent_Refs}} | ||
Line 27: | Line 39: | ||
Search term: [http://www.ncbi.nlm.nih.gov/pubmed/?term=Hypoplastic+Left+Heart ''Hypoplastic Left Heart''] | Search term: [http://www.ncbi.nlm.nih.gov/pubmed/?term=Hypoplastic+Left+Heart ''Hypoplastic Left Heart''] | ||
|} | |} | ||
==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). | |||
{| | |||
| [[File:Anderson2016-fig44a.jpg|400px]] | |||
| [[File:Anderson2016-fig44b.jpg|400px]] | |||
|- | |||
| 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. | |||
|} | |||
[[Paper - Teratogenecity in the setting of cardiac development and maldevelopment#Fig44|Fig 44]] | |||
==Ultrasound== | ==Ultrasound== | ||
{| | {| | ||
| width=510px|[[File:Ultrasound - Hypoplastic left heart syndrome 01.jpg|500px]] | | width=510px|[[File:Ultrasound - Hypoplastic left heart syndrome 01.jpg|500px]] | ||
| valign=top|Ultrasound Hypoplastic Left Heart Syndrome {{GA}} 17 - 18 week | | valign=top|Ultrasound Hypoplastic Left Heart Syndrome | ||
{{GA}} 17 - 18 week (second trimester) = week 15 - 16 | |||
* '''LA''' - left atria | * '''LA''' - left atria | ||
* '''RA''' - right atria | * '''RA''' - right atria | ||
Line 38: | Line 66: | ||
* '''RV''' - right ventricle | * '''RV''' - right ventricle | ||
|} | |} | ||
{{Ultrasound Hypoplastic Left Heart Syndrome}} | |||
{| | |||
! {{GA}} 19 week | |||
|- | |||
| width=405px|[[File:Ultrasound - Hypoplastic left heart syndrome 02.jpg|400px]] | |||
| width=405px|[[File:Ultrasound - Hypoplastic left heart syndrome 03.jpg|400px]] | |||
|- | |||
| 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. | |||
|} | |||
[[File:Ultrasound - Hypoplastic left heart syndrome 04.jpg|600px]] | |||
Colour Doppler exmination shows flow from atrium into ventricle on the right side but not the left. | |||
:'''Links:''' [[Ultrasound]] | :'''Links:''' [[Ultrasound]] | ||
Line 47: | Line 91: | ||
==Treatment== | ==Treatment== | ||
== | ===Fontan Procedure=== | ||
The Fontan procedure or Fontan–Kreutzer procedure is a surgical procedure used in children with univentricular hearts. | |||
= | Search PubMed: [http://www.ncbi.nlm.nih.gov/pubmed/?term=Fontan+procedure ''Fontan procedure''] | ||
==Cardiovascular Abnormalities== | ==Cardiovascular Abnormalities== | ||
Line 61: | Line 102: | ||
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. | 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) | 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){{#pmid:16322141|PMID16322141}} | ||
"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." | "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." {{#pmid:11241431|PMID11241431}} | ||
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. | 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. | ||
Line 72: | Line 113: | ||
===Reviews=== | ===Reviews=== | ||
{{#pmid:25663264}} | |||
{{#pmid:25836709}} | |||
{{#pmid:25917992}} | |||
{{#pmid:8489880}} | |||
{{#pmid:2659180}} | |||
===Articles=== | ===Articles=== | ||
===Search Pubmed=== | ===Search Pubmed=== | ||
'''Search Pubmed:''' [http://www.ncbi.nlm.nih.gov/sites/entrez?db=pubmed&cmd=search&term=Hypoplastic%20Left%20Heart Search PubMed] | '''Search Pubmed:''' [http://www.ncbi.nlm.nih.gov/sites/entrez?db=pubmed&cmd=search&term=Hypoplastic%20Left%20Heart Search PubMed] | ||
Line 90: | Line 133: | ||
{{External Links}} | {{External Links}} | ||
* '''OMIM''' [http:// | * '''OMIM''' [http://omim.org/entry/241550 Hypoplastic Left Heart Syndrome 1] | [http://omim.org/entry/614435 Hypoplastic Left Heart Syndrome 2] | ||
* '''Medline Plus''' | * '''Medline Plus''' [https://www.nlm.nih.gov/medlineplus/ency/article/001106.htm Hypoplastic left heart syndrome] | ||
Latest revision as of 13:17, 16 June 2019
Embryology - 16 Jun 2024 Expand to Translate |
---|
Google Translate - select your language from the list shown below (this will open a new external page) |
العربية | català | 中文 | 中國傳統的 | français | Deutsche | עִברִית | हिंदी | bahasa Indonesia | italiano | 日本語 | 한국어 | မြန်မာ | Pilipino | Polskie | português | ਪੰਜਾਬੀ ਦੇ | Română | русский | Español | Swahili | Svensk | ไทย | Türkçe | اردو | ייִדיש | Tiếng Việt These external translations are automated and may not be accurate. (More? About Translations) |
LA89.3 Hypoplastic Left Heart Syndrome
ICD-11 LA89 Functionally univentricular heart |
---|
LA89.3 Hypoplastic left heart syndrome
|
Introduction
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.
More? References | Discussion Page | Journal Searches | 2019 References | 2020 References Search term: Hypoplastic Left Heart |
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).
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
Ultrasound Hypoplastic Left Heart Syndrome
GA 17 - 18 week (second trimester) = week 15 - 16
|
Hypoplastic Left Heart Syndrome |
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
History
Treatment
Fontan Procedure
The Fontan procedure or Fontan–Kreutzer procedure is a surgical procedure used in children with univentricular hearts.
Search PubMed: Fontan procedure
Cardiovascular Abnormalities
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
- ↑ 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.
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
Articles
Search Pubmed
Search Pubmed: Search PubMed
External Links
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
Glossary Links
- Glossary: A | B | C | D | E | F | G | H | I | J | K | L | M | N | O | P | Q | R | S | T | U | V | W | X | Y | Z | Numbers | Symbols | Term Link
Cite this page: Hill, M.A. (2024, June 16) 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 2024, UNSW Embryology ISBN: 978 0 7334 2609 4 - UNSW CRICOS Provider Code No. 00098G