Transposition physiology in the setting of concordant ventriculo‐arterial connections

Abstract

Background and Aim To review the anatomical details, diagnostic challenges, associated cardiovascular anomalies, and techniques and outcomes of management, including re‐interventions, for the rare instances of transposition physiology with concordant ventriculo‐arterial connections. Methods We reviewed clinical and necropsy studies on diagnosis and surgical treatment of individuals with transposition physiology and concordant ventriculo‐arterial connections, analyzing also individuals with comparable flow patterns in the setting of isomerism. Results Among reported cases, just over two‐thirds were diagnosed during surgery, after initial palliation, or after necropsy. Of the patients, four‐fifths presented in infancy with either cyanosis or congestive cardiac failure, with complex associated cardiac malformations. Nearly half had ventricular septal defects, and one‐fifth had abnormalities of the tricuspid valve, including hypoplasia of the morphologically right ventricle. A small minority had common atrioventricular junctions We included cases reported with isomerism when the flow patterns were comparable, although the atrioventricular connections are mixed in this setting. Management mostly involved construction of intraatrial baffles, along with correction of coexisting anomalies, either together or multistaged. Overall mortality was 25%, with one‐fifth of patients requiring pacemakers for surgically‐induced heart block. The majority of survivors were in good functional state. Conclusions The flow patterns produced by discordant atrioventricular and concordant ventriculo‐arterial connections remain an important, albeit rare, indication for atrial redirection or hemi‐Mustard's procedure with bidirectional Glenn. The procedure recruits the morphologically left ventricle in the systemic circuit, producing good long‐term functional results. The approach can also be used for those with isomeric atrial appendages and comparable hemodynamic circuits.

Can Isolated Discordant Atrioventricular Connections Survive Without Interatrial or Interventricular Shunt?

“…1 However, the term “inversion” could be misleading and the term mirror-imaged or left ventricular topology seems appropriate in our case. 2 IDAVC is characterized by aortomitral continuity while “anatomically corrected malposition” is characterized by aortomitral discontinuity (Figure 1B). 2,3…”

“…2 IDAVC is characterized by aortomitral continuity while “anatomically corrected malposition” is characterized by aortomitral discontinuity (Figure 1B). 2,3…”

“…1 However, the term "inversion" could be misleading and the term mirror-imaged or left ventricular topology seems appropriate in our case. 2 IDAVC is characterized by aortomitral continuity while "anatomically corrected malposition" is characterized by aortomitral discontinuity (Figure 1B). 2,3 Straddling of the tricuspid valve is due to malalignment of the ventricular septum and results in varying degrees of hypoplasia of the right ventricular inflow, ranging from mild (biventricular) to the extreme (univentricular)-the double inlet left ventricle-end of the spectrum.…”

“…2 IDAVC is characterized by aortomitral continuity while "anatomically corrected malposition" is characterized by aortomitral discontinuity (Figure 1B). 2,3 Straddling of the tricuspid valve is due to malalignment of the ventricular septum and results in varying degrees of hypoplasia of the right ventricular inflow, ranging from mild (biventricular) to the extreme (univentricular)-the double inlet left ventricle-end of the spectrum. 3 However, the degree of override is usually between 20% and 45% (which, in the current patient, was about 44%).…”

When the description of physiology adds to the morphological diagnosis and guides the analysis of clinical and surgical management

“…A systematized description of the morphology of congenitally malformed hearts is recognized as essential for the understanding of the important features that will determine the appropriate clinical and surgical approaches. In the current issue of the Journal of Cardiac Surgery, Ujjwal Chowdhury 1 and co‐workers reviewed extensively the literature searching for cases with transposition physiology in the presence of concordant ventriculoarterial connections, to analyze the morphological features, clinical challenges, outcomes, and types of surgical interventions.…”