The Spectrum of Congenital Heart Disease with Transposition of the Great Arteries from the Cardiac Registry of the University of Padua

Abstract

Transposition of the great arteries (TGA) is a cardiac condition in which the arterial trunks arise from the inappropriate ventricle: the aorta from the right ventricle and the pulmonary trunk from the left ventricle [discordant ventriculo-arterial (VA) connection]. In complete TGA, the discordant VA connection is associated with situs solitus or inversus and concordant atrioventricular (AV) connection. The hemodynamic consequence of these combined connections is that systemic and pulmonary circulations function in “parallel” rather than in “series”. The presence of situs solitus or inversus associated with both AV and VA discordant connections characterizes a different anatomical complex known as “corrected TGA.” In these hearts, the double discordance at AV and VA levels permits a normal sequence of the blood flow from the right atrium to the pulmonary artery and from the left atrium to the aorta. The systemic and pulmonary circulation in these hearts functions regularly in series, and the blood sequence is “physiologically corrected.” Thus, the term transposition, either complete or corrected, identifies two precise, different anatomical complexes, both characterized by VA discordance. However, among congenital heart disease (CHD), there are other anatomical complexes with discordant VA connection in the setting of isomeric atrial situs (right or left) or of univentricular AV connections (double inlet or absent connections). In these latter conditions, the term “transposition” is still necessary to stress that the great arteries are “transposed” in relation to the ventricular septum (aorta from the right ventricle and pulmonary trunk from the left ventricle) but certainly does not figure out the anatomical complexes named complete or corrected transposition. We reviewed the hearts with discordant VA connection of our Anatomical Collection, consisting of 1,640 specimens with CHD, with the aim to discuss the anatomy and the frequency of the anatomical variants of TGA and to clarify terminology and classification. The knowledge of the precise anatomy of these malformation are really important for clinical diagnosis and surgical planning.

A Rare Case of Coronary Artery Embolism in a Patient with d-Transposition of the Great Arteries with Prior Mustard Repair

“…TGA is a rare congenital cyanotic heart malformation with a prevalence of 4.73 per 10,000 live births [ 1 ]. In TGA, there is ventriculoarterial discordance, with the aorta arising from the right ventricle and the pulmonary trunk arising from the left ventricle [ 4 ]. This leads to the development of two parallel systems of pulmonary and systemic circulation instead of normal flow in series [ 4 ].…”

“…In TGA, there is ventriculoarterial discordance, with the aorta arising from the right ventricle and the pulmonary trunk arising from the left ventricle [ 4 ]. This leads to the development of two parallel systems of pulmonary and systemic circulation instead of normal flow in series [ 4 ]. d-TGA is the more common variety of TGA compared to levo-TGA(l-TGA) [ 5 ].…”

Capabilities of cardiac magnetic resonance imaging and magnetic resonance angiography of the great vessels in children with transposition of the great vessels

“…Различают простую (в сочетании с открытым артериальным протоком и открытым овальным окном) и сложную формы ТМС (в сочетании с дефектом межжелудочковой перегородки -ДМЖП, стенозом легочной артерии -ЛА, коарктацией аорты и другими ВПС и сосудов) [2]. Вследствие того, что аорта отходит от анатомически правого желудочка (ПЖ), а легочной ствол -от анатомически левого желудочка (ЛЖ), малый и большой круги кровообращения разомкнуты и функционируют не последовательно, а параллельно [3]. Такая циркуляция крови может быть совместимой с жизнью ребенка при наличии шунта на уровне желудочков, предсердий или магистральных сосудов [4].…”

The possibilities of computed tomography using heart-axis-oriented multiplanar reformations in diagnostics of the great arteries transposition

“…Транспозиция магистральных сосудов -это порок сердца, при котором аорта отходит от морфологически правого желудочка, а легочная артерия -от морфологически левого желудочка (дискордантные желудочково-артериальные соединения), при этом морфологически правое предсердие соединяется с морфологически правым желудочком, морфологически левое предсердиес морфологически левым желудочком (конкордантное предсердно-желудочковое соединение) [3,5,6,9,11,17]. Это приводит к тому, что малый и большой круги кровообращения разомкнуты и функционируют не последовательно (как в норме), а параллельно [13]. Условием жизнеспособности ребенка является наличие сопутствующих внутрисердечных и внесердечных компенсирующих дефектов [12].…”