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Transposition of great vessels

Transposition of the great arteries (TGA) is a group of congenital heart defects (CHDs) involving an abnormal spatial arrangement of the primary arteries (pulmonary artery and aorta). It is a type of transposition of the great vessels, and was first described in 1797 by Matthew Baillie. more...

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Description

In a normal heart, oxygen-depleted ("blue") blood is pumped from the right side of the heart, through the pulmonary artery, to the lungs where it is oxygenated. The oxygen-rich ("red") blood then returns to the left heart, via the pulmonary veins, and is pumped through the aorta to the rest of the body, including the heart muscle itself.

Transposed arteries can present a large variety of ventriculoarterial and arteriovenous discordance. The effects may range from a change in blood pressure to an interruption in circulation, depending on the nature and degree of the misplacement and the presence or absence of other defects.

Variations and Similar Defects

TGA may be defined as either dextro-TGA (d-TGA) or levo-TGA (l-TGA). With d-TGA, the aorta is anterior and to the right of the pulmonary artery, creating two separate, “circular” circulatory systems; with l-TGA, the aorta is anterior and to the left of the pulmonary artery and is accompanied by transposed ventricles; this combination results in a “corrected” circulation.

Simple and Complex TGA

TGA is often accompanied by other heart defects, the most common type being intracardiac shunts such as atrial septal defect (ASD) including patent foramen ovale (PFO), ventricular septal defect (VSD), and patent ductus arteriosus (PDA). Stenosis, or other defects, of valves and/or vessels may also be present.

When no other heart defects are present it is called 'simple' TGA; when other defects are present it is called 'complex' TGA.

Similar Defects

The following defects also involve abnormal spatial arrangement and/or structure of the great arteries:

  • Coarctation of the aorta
  • Double outlet right ventricle (DORV)
  • Left heart hypoplasia or hypoplastic left heart syndrome (HLHS)
  • Overriding aorta
  • Patent ductus arteriosus (PDA)
  • Taussig-Bing syndrome
  • Tetralogy of Fallot (TOF)
  • Truncus arteriosus
  • Vascular rings

External Links

  • Information on Transposition of the Great Arteries from Seattle Children's Hospital Heart Center

Read more at Wikipedia.org


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Double Inlet Left Ventricle with Transposition of the Great Vessels
From Medicine and Health Rhode Island, 6/1/04 by Posteraro, Anthony F III

A 40 year-old male presented to the emergency department with fever and mental status changes. Diagnostic evaluation revealed a brain abscess of uncertain etiology. The patient was known to have a complex uncorrected congenital heart condition, the details of which were incompletely understood.

The patient underwent cardiac MRI examination to further evaluate anatomy and function. Axial black blood Tl weighted images of the chest confirmed dextrocardia (Figure 1) with a single ventricle, situs solitus, and two atrioventricular valves. The patient had transposition of his aorta and pulmonary artery (Figure 2; curved arrow: main pulmonary artery; straight arrow: aorta with a tight pulmonic stenosis demonstrated on cine bright blood images as a large systolic jet (Figure 3) with maximal velocity of 6.8 m/sec. The most accurate anatomic description of this configuration is felt to be double inlet left ventricle with transposition of the great arteries (S,L,L).

A single ventricle is an exceedingly rare cardiac anomaly, estimated to be 3% of all congenital cardiac anomalies.1 By definition a true single ventricle is present when there are two atrioventricular valves emptying into a common ventricle with two great vessels arising from the single ventricle. Most cases are associated with transposition of the great vessels. Since patients have a common mixing chamber for arterial and venous blood, clinical presentation and need for palliation depends on associated lesions.2 Patients such as the one described here, who have balanced lesions with enough pulmonic stenosis to protect the pulmonary circulation, can remain relatively asymptomatic and present later in life with other complications such as peripheral embolization.

REFERENCES

1. Marin-Garcia J, Tandon R, Moller JH, Edwards JE. Single ventricle with transposition. Circulation 1974; 49 : 994.

2. Schwanz SM, Dent CL, Musa NL, Nelson DP. Single-ventricle physiology. Crit Care Clin. 2003; 19: 393-411.

ANTHONY F. POSTERARO III, MD, AND MICHAEL K. ATALAY, MD, PHD

CORRESPONDENCE:

Michael K. Atalay, MD, PhD

e-mail: Matalay@lifespan.org

Anthony F. Posteraro III, MD, is a Resident in the Department of Diagnostic Imaging, Rhode Island Hospital.

Michael K. Atalay, MD, is Director, Cross-sectional Cardiovascular Imaging, in the Department of Diagnostic Inaging, Rhode Island Hospital, and Assistant Professor, Brown Medical School.

Copyright Rhode Island Medical Society Jun 2004
Provided by ProQuest Information and Learning Company. All rights Reserved

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