Cardiovascular System - Transposition of the Great Vessels

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Introduction

Transposition of the Great Vessels

Characterized by aorta arising from right ventricle and pulmonary artery from the left ventricle and often associated with other cardiac abnormalities (e.g. ventricular septal defect).

  • International Classification of Diseases code 745.1
  • Australian national rate (1982-1992) 3.6/10,000 births.
  • Of 988 infants 4.1% were stillborn and 23.2% liveborn died during neonatal period.
  • slightly more common in twin births than singleton.
  • Congenital Malformations Australia 1981-1992 P. Lancaster and E. Pedisich ISSN 1321-8352
  • Neonates with transposed great arteries die without an arterial switch operation, first carried out in 1975.


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

  • MRI of surgical repair of transposition of the great vessels[1] "Our objectives are to review the surgical procedures that have been performed for the treatment of transposition of the great vessels. Using MRI, we illustrate the normal postoperative findings and many of the long-term complications for each of the surgical procedures. CONCLUSION: MRI is an extremely useful imaging method for evaluation of normal and abnormal findings after surgical repair of transposition of the aorta and pulmonary artery."

Ultrasound

<html5media height="450" width="640">File:US Transposition Great Arteries GA36week.mp4</html5media>

Ultrasound showing Transposition Great Arteries (GA 36 weeks)

Links: MP4 movie | Transposition of the Great Vessels | Ultrasound | International Classification of Diseases | Movies

History

Historic drawing of external view of heart with transposition.[2]

1833

The morbid anatomy of some of the most important parts of the human body (1833)[3]

"A very singular Malformation of the Heart, in a child about two months old, came, some time ago, into my possession : the aorta arose out of the right ventricle, and the pulmonary artery out of the left. There was no communication between the one vessel and the other, except through the small remains of the ductus arteriosus, which was just large enough to admit a crow quill. The foramen ovale was a little more closed than in a child newly born. The heart was of the common size for a child of two months old, and, except for the circumstances which have been stated, it had nothing remarkable in its structure. In this child florid blood must have always been circulating between the lungs and the left side of the heart, except for the admixture of the dark blood which passed through the small communication of the foramen ovale ; and dark blood must have been always circulating between the right side of the heart and the general mass of the body, except for the very small quantity of florid blood which passed into the aorta by the remains of the ductus arteriosus. Life must, therefore, have been supported for a very considerable length of time with hardly any florid blood distributed over the body."

1863

"The aorta arises from the fleshy part of the base of the right ventricle, at its left portion, where it presents itself in front, being quite uncovered by the pulmonary artery, and is situate between this vessel and the right auricle."[2]

Australian Statistics

Number of women who gave birth to babies with transposition of great vessels, Australia, 2002–2003

Year Number Rate1
2002 105 4.3
2003 103 4.1
2002–2003 208 4.2


1The rate is per 10,000 women who gave birth.

Based upon Table 2.10.4 of Congenital anomalies in Australia 2002–2003.[4]

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.

ICD11

  • LB01 Congenital anomaly of atrioventricular or ventriculo-arterial connections
    • LB01.1 Transposition of the great arteries
ICD-11 Structural developmental anomalies of the circulatory system (draft) 
ICD-11 Beta Draft - NOT FINAL, updated on a daily basis, It is not approved by WHO, NOT TO BE USED for CODING except for agreed FIELD TRIALS.

20 Developmental Anomalies - Structural Developmental Anomalies

Beta coding and tree structure for "structural developmental anomalies" within this section are shown in the table below.

Structural developmental anomalies of the circulatory system  
  • Structural developmental anomaly of heart and great vessels
    • LB00 Congenital heart or great vessel related acquired abnormality
    • LB01 Congenital anomaly of atrioventricular or ventriculo-arterial connections
      • LB01.1 Transposition of the great arteries
      • LB01.2 Double outlet right ventricle
      • LB01.3 Double outlet left ventricle
      • LB01.4 Common arterial trunk
      • LB01.Y Other specified congenital anomaly of atrioventricular or ventriculo-arterial connections
      • LB01.Z Congenital anomaly of atrioventricular or ventriculo-arterial connections, unspecified
    • LB02 Congenital anomaly of the mediastinal veins Congenital anomaly of atria or atrial septum
    • LB20 Congenital anomaly of atrioventricular valves or septum
    • LB21 Congenital anomaly of ventricles and ventricular septum
      • LB21.1 Congenital right ventricular outflow tract obstruction  
      • LB21.2 Double-chambered right ventricle  
      • LB21.3 Tetralogy of Fallot
      • LB21.4 Congenital left ventricular outflow tract obstruction  
      • LB21.5 Congenital ventricular septal defects 
      • LB21.Y Other specified congenital anomaly of ventricles and ventricular septum
      • LB21.Z Congenital anomaly of ventricles and ventricular septum, unspecified  
    • LB22 Functionally univentricular heart
    • LB23 Congenital anomaly of ventriculo-arterial valves and adjacent regions
    • LB24 Congenital anomaly of great arteries including arterial duct
      • LB.1 Congenital aorto-pulmonary window
      • LB.2 Congenital anomaly of pulmonary arterial tree
      • LB.3 Congenital anomaly of aorta and its branches
      • LB.4 Tracheo-oesophageal compressive syndrome
      • LB.5 Patent arterial duct
      • LB.Y Other specified congenital anomaly of great arteries including arterial duct
      • LB.Z Congenital anomaly of great arteries including arterial duct, unspecified
    • LB25 Anomalous position-orientation of heart
    • LB26 Total mirror imagery
    • LB27 Left isomerism
    • LB28 Congenital anomaly of coronary arteries
    • LB29 Structural developmental anomalies of the pericardium
    • LB2Y Other specified structural developmental anomaly of heart and great vessels
    • LB2Z Structural developmental anomaly of heart and great vessels, unspecified
  • LB30 Structural developmental anomalies of the peripheral vascular system
    • LB30.1 Capillary malformations
    • LB30.2 Lymphatic malformations
      • LB30.21 Macrocystic lymphatic malformation
      • LB30.22 Microcystic lymphatic malformation
      • LB30.23 Cystic hygroma in fetus
      • BD23.1 Primary lymphoedema
          • EK91 Yellow nail syndrome
          • LC5F.26 Noonan syndrome
      • LB30.2Y Other specified lymphatic malformations
      • LB30.2Z Lymphatic malformations, unspecified
    • LB30.3 Peripheral venous malformations
    • LB30.4 Peripheral arteriovenous malformations
    • LB30.5 Peripheral arterial malformations
    • LB30.6 Pulmonary arteriovenous fistula
    • LB30.Y Other specified structural developmental anomalies of the peripheral vascular system
    • LB30.Z Structural developmental anomalies of the peripheral vascular system, unspecified
  • LB3Y Other specified structural developmental anomalies of the circulatory system
  • LB3Z Structural developmental anomalies of the circulatory system, unspecified
CD-11 Beta Draft - NOT FINAL, updated on a daily basis, It is not approved by WHO, NOT TO BE USED for CODING except for agreed FIELD TRIALS.


See also International Classification of Diseases | Abnormalities

ICD10

ICD-10-AM codes: Q20.1, Q20.3, Q20.5

ICD-9-BPA codes: 745.10–745.19

(ICD) The World Health Organization's classification used worldwide to classify the causes of death. The current version is ICD-10 (1990) that is due for replacement by ICD-11 (2011). A modified version, Australian modification of ICD-10 (ICD-10-AM), is used to classify diagnoses in Australian hospitals. Within this classification "congenital malformations, deformations and chromosomal abnormalities" are (Q00-Q99) but excludes "inborn errors of metabolism" (E70-E90).


Links: WHO

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)[5]

"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." [6]

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>20028930</pubmed>
  2. 2.0 2.1 <pubmed>20896216</pubmed>| PMC2147784 | PDF
  3. Matthew, Baillie The morbid anatomy of some of the most important parts of the human body (1833) Internet Archive
  4. Abeywardana S & Sullivan EA 2008. Congenital anomalies in Australia 2002–2003. Birth anomalies series no. 3 Cat. no. PER 41. Sydney: AIHW National Perinatal Statistics Unit. PDF
  5. <pubmed>16322141</pubmed>
  6. <pubmed>11241431</pubmed>

Reviews

<pubmed>18851735</pubmed> <pubmed>15987628</pubmed>

Articles

<pubmed>15547014</pubmed> <pubmed>2107695</pubmed> <pubmed>3348669</pubmed>

Search Pubmed

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Cite this page: Hill, M.A. (2024, March 29) Embryology Cardiovascular System - Transposition of the Great Vessels. Retrieved from https://embryology.med.unsw.edu.au/embryology/index.php/Cardiovascular_System_-_Transposition_of_the_Great_Vessels

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© Dr Mark Hill 2024, UNSW Embryology ISBN: 978 0 7334 2609 4 - UNSW CRICOS Provider Code No. 00098G