Cardiovascular System - Transposition of the Great Vessels

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LA85.1 Transposition of the Great Arteries

 ICD-11
LA85.1 Transposition of the great arteries

LA85 Congenital anomaly of an atrioventricular or ventriculo-arterial connection A congenital cardiovascular malformation in which one or more of the following connections is abnormal 1) the morphologically right atrium to the morphologically right ventricle, 2) the morphologically left atrium to the morphologically left ventricle, 3) the morphologically right ventricle to the pulmonary trunk, 4) the morphologically left ventricle to the aorta. This excludes codes for hearts with a univentricular atrioventricular connection (mitral atresia, tricuspid atresia and double inlet ventricle), as these are listed under Functionally Univentricular Heart.

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

Australia Congenital heart disease 2016–17
Australia Congenital heart disease 2016–17
  • Continuous-Flow Left Ventricular Assist Device Therapy in Adults with Transposition of the Great Vessels[2] "An increasing number of children with congenital heart disease are surviving into adulthood and subsequently developing end-stage heart failure. Two example populations are adults who have been previously operated on for congenitally corrected transposition of the great arteries (CCTGA) and transposition of the great arteries (TGA). Implantation of a continuous flow left ventricular assist device (CF-LVAD) in these patients can present unusual anatomical and physiologic challenges. In this report, we describe outcomes of CF-LVAD implantation in three such patients. These cases demonstrate the feasibility of implanting a CF-LVAD in patients who have undergone surgery for CCTGA and/or TGA."
  • Contemporary management and outcomes in congenitally corrected transposition of the great arteries[3] "Congenitally corrected transposition of the great arteries (ccTGA) can occur in isolation, or in combination with other structural cardiac anomalies, most commonly ventricular septal defect, pulmonary stenosis and tricuspid valve disease. Clinical recognition can be challenging, so echocardiography is often the means by which definitive diagnosis is made. The tricuspid valve and right ventricle are on the systemic arterial side of the ccTGA circulation, and are therefore subject to progressive functional deterioration. The natural history of ccTGA is also greatly influenced by the nature and severity of accompanying lesions, some of which require surgical repair. Some management strategies leave the right ventricle as the systemic arterial pump, but carry the risk of worsening heart failure. More complex 'double switch' repairs establish the left ventricle as the systemic pump, and include an atrial baffle to redirect venous return in combination with either arterial switch or Rastelli operation (if a suitable ventricular septal defect permits). Occasionally, the anatomic peculiarities of ccTGA do not allow straightforward biventricular repair, and Fontan palliation is a reasonable option. Regardless of the approach selected, late cardiovascular complications are relatively common, so ongoing outpatient surveillance should be established in an age-appropriate facility with expertise in congenital heart disease care."
More recent papers  
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Search term: Transposition of the Great Vessels | Transposition of the Great Arteries

Older papers  
These papers originally appeared in the Some Recent Findings table, but as that list grew in length have now been shuffled down to this collapsible table.

See also the Discussion Page for other references listed by year and References on this current page.

  • MRI of surgical repair of transposition of the great vessels[4] "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.[5]

1833

The morbid anatomy of some of the most important parts of the human body (1833)[6]

"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."[5]

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.[7]

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)[8]

"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."[9]

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. Australian Institute of Health and Welfare 2019. Congenital heart disease in Australia. Cat. no. CDK 14. Canberra: AIHW.
  2. Sugiura T, Kurihara C, Kawabori M, Critsinelis AC, Civitello AB, Morgan JA & Frazier OH. (2018). Continuous-Flow Left Ventricular Assist Device Therapy in Adults with Transposition of the Great Vessels. Ann Thorac Cardiovasc Surg , , . PMID: 30101823 DOI.
  3. Kutty S, Danford DA, Diller GP & Tutarel O. (2018). Contemporary management and outcomes in congenitally corrected transposition of the great arteries. Heart , 104, 1148-1155. PMID: 29326110 DOI.
  4. Cohen MD, Johnson T & Ramrakhiani S. (2010). MRI of surgical repair of transposition of the great vessels. AJR Am J Roentgenol , 194, 250-60. PMID: 20028930 DOI.
  5. 5.0 5.1 Cockle J. (1863). A Case of Transposition of the Great Vessels of the Heart. Med Chir Trans , 46, 193-210.3. PMID: 20896216
  6. Matthew, Baillie The morbid anatomy of some of the most important parts of the human body (1833) Internet Archive
  7. 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
  8. Tanner K, Sabrine N & Wren C. (2005). Cardiovascular malformations among preterm infants. Pediatrics , 116, e833-8. PMID: 16322141 DOI.
  9. 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

Kutty S, Danford DA, Diller GP & Tutarel O. (2018). Contemporary management and outcomes in congenitally corrected transposition of the great arteries. Heart , 104, 1148-1155. PMID: 29326110 DOI.

Martins P & Castela E. (2008). Transposition of the great arteries. Orphanet J Rare Dis , 3, 27. PMID: 18851735 DOI.

Murphy DJ. (2005). Transposition of the great arteries: long-term outcome and current management. Curr Cardiol Rep , 7, 299-304. PMID: 15987628

Articles

Guven H, Billadello J & Beardslee M. (2004). An isolated single coronary artery supplying the entire myocardium in a patient with congenitally corrected transposition of the great vessels. Heart , 90, 1410. PMID: 15547014 DOI.

Formanek AG. (1990). MR imaging of congenitally corrected transposition of the great vessels in adults. AJR Am J Roentgenol , 154, 898-9. PMID: 2107695 DOI.

Hesz N & Clark EB. (1988). Cognitive development in transposition of the great vessels. Arch. Dis. Child. , 63, 198-200. PMID: 3348669

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Cite this page: Hill, M.A. (2024, March 19) 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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