Transposition of the great arteries

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Transposition of the great arteries (TGA) is the most common cyanotic congenital cardiac anomal y with cyanosis in first 24 hours of life and can account for up to 7% of all congenital cardiac anomalies ¹.

Epidemiology

The estimated incidence is at ~1 in 5000 births. Transposition of the great arteries is an isolated abnormality in 90% of those affected and rarely is associated with a syndrome or an extra-cardiac malformation. It is most common in infants of diabetic mothers ¹.

Pathology

Occurs as a result of ventriculo~~-atrial~~-arterial discordance with the aorta arising from the right ventricle and the pulmonary trunk from the left ventricle. It can be sub divided into two main types depending on the positional relationship of the aortic valve with the pulmonary valve.

The article mainly focuses on the D ~~type~~loop transposition.

An isolated TGA is incompatible with life at birth without one of the following additional anomalies (which are a common occurrence ²).:

atrial septal defect (ASD): uncommon
ventricular septal defect (VSD): ~35%
patent ductus arteriosus (PDA): unstable due closure following birth
patent foramen ovale (PFO): unstable

Unstable associations account for 60-65% of occurrences.

Associations

Approximately 90% of TGA's occur as an isolated finding and extra cardiac syndromic associations are generally rare. ~~associations~~Associations have been described with:

maternal diabetes
congential coronary arterial anomalies

Radiographic features

Plain filmChest radiograph

A frontal chest radiograph classically shows cardiomegaly with a cardiac contours classically described as appearing like an egg on a string ¹. There is often an apparent narrowing of the superior mediastinum as result of the aortic and pulmonary arterial configuration i.e. parallel in D-loop transposition, with main pulmonary artery posterior to the aorta.

Echocardiography/ultrasound

Allows direct visualization of anomalous anatomy with the aorta and pulmonary trunk lying in parallel with absence of crossing (best seen on the base view of the fetal heart) ⁴.

Contrast CT/CTA

Allows direct visualisation of anomalous great vessel anatomy. Cardiac gated cine CT can additionally assess function.

Cardiac MRI

Allows direct visualisation of anomlaous anatomy. SSFP cine sequences can additionally assess flow dynamics.

Treatment

~~Initially~~Initial Rashkind septoplasty is usually done as palliative procedure in neonates.

Definitive surgical correction

Previously TGAs were treated with atrial switch operations, such as a Mustard repair or Senning repair, which have been superseded by arterial switch procedures ⁵.

-<p><strong>Transposition of the great arteries (TGA) </strong>is the most common <a href="/articles/cyanotic-congenital-heart-disease">cyanotic congenital cardiac anomal</a><a href="/articles/cyanotic-congenital-cardiac-anomalies">y</a> with cyanosis in first 24 hours of life and can account for up to 7% of <a href="/articles/congenital-cardiovascular-anomalies">all congenital cardiac anomalies</a> <sup>1</sup>. </p><h4>Epidemiology</h4><p>The estimated incidence is at ~1 in 5000 births. Transposition of the great arteries is an isolated abnormality in 90% of those affected and rarely is associated with a syndrome or an extra-cardiac malformation. It is most common in infants of diabetic mothers <sup>1</sup>.</p><h4>Pathology</h4><p>Occurs as a result of ventriculo-atrial discordance with the aorta arising from the right ventricle and the pulmonary trunk from the left ventricle. It can be sub divided into two main types depending on the positional relationship of the aortic valve with the pulmonary valve. </p><ul>
+<p><strong>Transposition of the great arteries (TGA) </strong>is the most common <a href="/articles/cyanotic-congenital-heart-disease">cyanotic congenital cardiac anomal</a><a href="/articles/cyanotic-congenital-cardiac-anomalies">y</a> with cyanosis in first 24 hours of life and can account for up to 7% of <a href="/articles/congenital-cardiovascular-anomalies">all congenital cardiac anomalies</a> <sup>1</sup>. </p><h4>Epidemiology</h4><p>The estimated incidence is at ~1 in 5000 births. Transposition of the great arteries is an isolated abnormality in 90% of those affected and rarely is associated with a syndrome or an extra-cardiac malformation. It is most common in infants of diabetic mothers <sup>1</sup>.</p><h4>Pathology</h4><p>Occurs as a result of ventriculo-arterial discordance with the aorta arising from the right ventricle and the pulmonary trunk from the left ventricle. It can be sub divided into two main types depending on the positional relationship of the aortic valve with the pulmonary valve. </p><ul>
~~-<a href="/articles/l-type-transposition-of-the-great-arteries">L type transposition of the great arteries</a>-<a href="/articles/congenitally-corrected-tga">congenitally corrected TGA</a>~~
+<a title="L loop transposition of the great arteries" href="/articles/l-loop-transposition-of-the-great-arteries">L loop transposition of the great arteries</a>: <a title="congenitally corrected TGA" href="/articles/congenitally-corrected-tga">congenitally corrected TGA</a>
~~-<li><a href="/articles/d-type-transposition-of-the-great-arteries">D type transposition of the great arteries</a></li>~~
-</ul><p>The article mainly focuses on the <strong>D type transposition</strong>.</p><p>An isolated TGA is incompatible with life at birth without one of the following additional anomalies (which are a common occurrence <sup>2</sup>).</p><ul>
+<li><a title="D loop transposition of the great arteries" href="/articles/d-loop-transposition-of-the-great-arteries">D loop transposition of the great arteries</a></li>
+</ul><p>The article mainly focuses on the <strong>D loop transposition</strong>.</p><p>An isolated TGA is incompatible with life at birth without one of the following additional anomalies (which are a common occurrence <sup>2</sup>):</p><ul>
-</ul><p>Unstable associations account for 60-65% of occurrences.</p><h5>Associations</h5><p>Approximately 90% of TGA's occur as an isolated finding and extra cardiac syndromic associations are generally rare. associations have been described with</p><ul>
+</ul><p>Unstable associations account for 60-65% of occurrences.</p><h5>Associations</h5><p>Approximately 90% of TGA's occur as an isolated finding and extra cardiac syndromic associations are generally rare. Associations have been described with:</p><ul>
-</ul><h4>Radiographic features</h4><h5>Plain film</h5><p>A frontal chest radiograph classically shows cardiomegaly with a cardiac contours classically described as appearing like an <a href="/articles/egg-on-a-string-sign">egg on a string</a> <sup>1</sup>. There is often an apparent narrowing of the superior mediastinum as result of the aortic and pulmonary arterial configuration.</p><h5>Echocardiography/ultrasound </h5><p>Allows direct visualization of anomalous anatomy with the aorta and pulmonary trunk lying in parallel with absence of crossing (best seen on the base view of the fetal heart) <sup>4</sup>. </p><h5>Contrast CT/CTA</h5><p>Allows direct visualisation of anomalous great vessel anatomy. Cardiac gated cine CT can additionally assess function. </p><h5>Cardiac MRI </h5><p>Allows direct visualisation of anomlaous anatomy. <a href="/articles/steady-state-free-precession-mri-2">SSFP cine sequences</a> can additionally assess flow dynamics. </p><h4>Treatment</h4><p>Initially TGAs were treated with atrial switch operations, such as a <a href="/articles/mustard-repair">Mustard repair</a> or <a href="/articles/senning-repair">Senning repair</a>, which have been superseded by <a title="Arterial switch procedure" href="/articles/arterial-switch-procedure">arterial switch procedures</a> <sup>5</sup>.</p>
+</ul><h4>Radiographic features</h4><h5>Chest radiograph</h5><p>A frontal chest radiograph classically shows cardiomegaly with a cardiac contours classically described as appearing like an <a href="/articles/egg-on-a-string-sign">egg on a string</a> <sup>1</sup>. There is often an apparent narrowing of the superior mediastinum as result of the aortic and pulmonary arterial configuration i.e. parallel in D-loop transposition, with main pulmonary artery posterior to the aorta.</p><h5>Echocardiography/ultrasound </h5><p>Allows direct visualization of anomalous anatomy with the aorta and pulmonary trunk lying in parallel with absence of crossing (best seen on the base view of the fetal heart) <sup>4</sup>. </p><h5>Contrast CT/CTA</h5><p>Allows direct visualisation of anomalous great vessel anatomy. Cardiac gated cine CT can additionally assess function. </p><h5>Cardiac MRI </h5><p>Allows direct visualisation of anomlaous anatomy. <a href="/articles/steady-state-free-precession-mri-2">SSFP cine sequences</a> can additionally assess flow dynamics. </p><h4>Treatment</h4><p>Initial <a title="Rashkind septoplasty" href="/articles/rashkind-septoplasty">Rashkind septoplasty</a> is usually done as palliative procedure in neonates.</p><h5>Definitive surgical correction</h5><p>Previously TGAs were treated with atrial switch operations, such as a <a href="/articles/mustard-repair">Mustard repair</a> or <a href="/articles/senning-repair">Senning repair</a>, which have been superseded by <a href="/articles/arterial-switch-procedure">arterial switch procedures</a> <sup>5</sup>.</p>

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