Discordant atrioventricular connexion with concordant ventriculo-arterial connexion (so-called “isolated ventricular inversion”) with usual atrial arrangement (situs solitus)

What is anatomically corrected malposition?

“…attesting to the existence of this malformation. It remains a fact, nonetheless, that many paediatric cardiologists and surgeons remain unaware of the significance of the malformation, or else are unsure of its make-up, in particular its distinction from other malformations which are currently described using arcane terms such as "isolated ventricular inversion", [23][24][25][26][27][28][29][30] or "isolated ventricular discordance". 31,32 These terms are not immediately meaningful, and it could be argued that the term "anatomically corrected malposition" would better be applied to the normal heart, in which any malpositions are certainly anatomically corrected.…”

Problems in the diagnosis of discordant atrioventricular with concordant ventriculo-arterial connections: anatomical considerations, surgical management, and long-term outcome

“…28 Reported cases with postoperative heart block concerned cases of discordant atrioventricular connections in atrial situs solitus. 5,7,26 Interestingly, both the patients with ventricular septal defect closure without complications in this series had atrial situs inversus. Moreover, it is generally acknowledged that ventricular septal defect closure from the morphologically right ventricle is to be preferred in order to diminish post-operative heart block.…”

“…More than 40 cases of isolated or complex forms of discordant atrioventricular with concordant ventriculo-arterial connections have been reported, including cases with heterotaxy. [1][2][3][4][5][6][7][8]13 The challenge of diagnosing this rare cardiac defect, especially if it is part of a complex CHD, has not changed since its first description. The main problem in all historical patients was that the correct anatomical diagnosis was only made at autopsy.…”

“…It is of note that none of our patients had postoperative complete heart block, although this has already been reported in patients having discordant atrioventricular with concordant ventriculo-arterial connections and ventricular septal defects who were successfully operated. 5,7,13,26 Conduction bundle pathways are not really documented in discordant atrioventricular with concordant ventriculo-arterial connections even if electrophysiological perioperative explorations have been performed; however, several anatomical and electrophysiological studies in hearts with corrected transposition of the great arteries in atrial situs inversus {I, D, D} have shown that, in this case, the atrioventricular node and the atrioventricular connection tissue are generally located posteriorly, contrary to corrected transposition of the great arteries in atrial situs solitus {S, L, L}, even if exceptions exist, particularly when there is associated pulmonary stenosis or atresia, leading to a better alignment between the atrial and ventricular septa. [27][28][29][30] In the presence of discordant atrioventricular connections, the location of the atrioventricular node would, thus, depend on the alignment between the atrial and ventricular septa, normally aligned in atrial situs inversus and malaligned in atrial situs solitus.…”

“…1,2 Reports about this complex cardiac defect remain rare and are mainly case descriptions and small patient series, often lacking information on long-term follow-up. [3][4][5][6][7][8] Atrial switch operation by Mustard or Senning technique is the surgical method of choice. [3][4][5][6][7][8] We report here a series of eight patients with discordant atrioventricular and concordant ventriculoarterial connections by discussing the difficulties in correctly describing the anatomy and obtain an accurate pre-operative diagnosis in these complex settings.…”