Cardiovascular System - Transposition of the Great Vessels

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Contents

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.


Abnormal Links: Tutorial Abnormalities | Ventricular Septal Defects | Atrial Septal Defects | Patent Ductus Arteriosus‎ | Tetralogy of Fallot | Transposition of the Great Vessels | International Classification of Diseases - Cardiovascular


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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."

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

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

About Data: 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. Mervyn D Cohen, Tiffanie Johnson, Sabeena Ramrakhiani MRI of surgical repair of transposition of the great vessels. AJR Am J Roentgenol: 2010, 194(1);250-60 PMID:20028930
  2. 2.0 2.1 J Cockle A Case of Transposition of the Great Vessels of the Heart. Med Chir Trans: 1863, 46();193-210.3 PMID:20896216 | 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. Kirsty Tanner, Nilofer Sabrine, Christopher Wren Cardiovascular malformations among preterm infants. Pediatrics: 2005, 116(6);e833-8 PMID:16322141
  6. C A Loffredo, J Hirata, P D Wilson, C Ferencz, I W Lurie Atrioventricular septal defects: possible etiologic differences between complete and partial defects. Teratology: 2001, 63(2);87-93 PMID:11241431

Reviews

Paula Martins, Eduardo Castela Transposition of the great arteries. Orphanet J Rare Dis: 2008, 3();27 PMID:18851735

Daniel J Murphy Transposition of the great arteries: long-term outcome and current management. Curr Cardiol Rep: 2005, 7(4);299-304 PMID:15987628


Articles

H Guven, J Billadello, M Beardslee An isolated single coronary artery supplying the entire myocardium in a patient with congenitally corrected transposition of the great vessels. Heart: 2004, 90(12);1410 PMID:15547014

A G Formanek MR imaging of congenitally corrected transposition of the great vessels in adults. AJR Am J Roentgenol: 1990, 154(4);898-9 PMID:2107695

N Hesz, E B Clark Cognitive development in transposition of the great vessels. Arch. Dis. Child.: 1988, 63(2);198-200 PMID:3348669


Search Pubmed

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External Links

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Cite this page: Hill, M.A. (2014) Embryology Cardiovascular System - Transposition of the Great Vessels. Retrieved April 23, 2014, from http://embryology.med.unsw.edu.au/embryology/index.php?title=Cardiovascular_System_-_Transposition_of_the_Great_Vessels

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Dr Mark Hill 2014, UNSW Embryology ISBN: 978 0 7334 2609 4 - UNSW CRICOS Provider Code No. 00098G
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