Partial atrioventricular canal defect with inverted atrioventricular nodal input into an inferiorly displaced atrioventricular node

Khairy, Paul; Mercier, Lise‐Andrée; Dore, Annie; Dubuc, Marc

doi:10.1016/j.hrthm.2006.10.012

Cited by 23 publications

(11 citation statements)

References 12 publications

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“…In this regard, we show how the distal aspect of the region normally associated with the “fast” pathway is continuous with a septal muscle bundle and the common valve annulus. Contrary to the findings of Khairy and associates (Khairy et al, 2007 ), our modeling data suggests conduction along the leftward aspect of the valve annulus is slow and is annihilated by the “slow” pathway (Supplementary Videos 1 , 2 ). The preferential route for the “fast” pathway is, therefore, via the septal region.…”

Section: Discussioncontrasting

confidence: 99%

“…The simulations of atrial activation produced in the present study show preferential activation of the compact atrioventricular node via the region normally associated with the “slow” pathway (Figures 5 – 7 ). This flipping of the dual pathway physiology is consistent with previous in-vivo three-dimensional electroanatomic mapping studies, in which the slowest pathway was located superior to AVCA, while the fastest pathway was identified posterior-inferior to the compact node (Khairy et al, 2007 ; Khairy and Balaji, 2009 ). The arrangement is best observed in the right hand and left hand views shown in Figure 5 .…”

Section: Discussionsupporting

confidence: 90%

“…Patients with atrioventricular septal defect (AVSD) or atrioventricular canal defect (AVCD) have a common atrioventricular connection, this occurs due to incorrect fusion of the endocardial cushions with the atrial septum and muscular ventricular septum (Anderson et al, 2000 , 2008 ). Preoperative electrophysiological studies of AVSD patients have shown cases of atrioventricular re-entrant tachycardia (Khairy et al, 2007 ), atrial fibrillation (Daliento et al, 1991 ; Khairy et al, 2006 ) and supra-Hisian first degree AV block, and confirm inter-nodal conduction delay in the majority of patients (Fournier et al, 1986 ). Persistent AV block is present in up to 7% of patients in the immediate post-operative period and approximately 2% on follow up (Daliento et al, 1991 ; Boening et al, 2002 ), with atrial fibrillation or flutter noted in 5% of patients after surgical repair (Vetter and Horowitz, 1982 ; Daliento et al, 1991 ).…”

Section: Introductionmentioning

confidence: 97%

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Morphological Substrates for Atrial Arrhythmogenesis in a Heart With Atrioventricular Septal Defect

et al. 2018

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Due to advances in corrective surgery, congenital heart disease has an ever growing patient population. Atrial arrhythmias are frequently observed pre- and post-surgical correction. Pharmaceutical antiarrhythmic therapy is not always effective, therefore many symptomatic patients undergo catheter ablation therapy. In patients with atrioventricular septal defects (AVSD), ablation therapy itself has mixed success; arrhythmogenic recurrences are common, and because of the anatomical displacement of the atrioventricular node, 3-degree heart block post-ablation is a real concern. In order to develop optimal and safe ablation strategies, the field of congenital cardiac electrophysiology must combine knowledge from clinical electrophysiology with a thorough understanding of the anatomical substrates for arrhythmias. Using image-based analysis and multi-cellular mathematical modeling of electrical activation, we show how the anatomical alterations characteristic of an AVSD serve as arrhythmogenic substrates. Using ex-vivo contrast enhanced micro-computed tomography we imaged post-mortem the heart of a 5 month old male with AVSD at an isometric spatial resolution of 38 μm. Morphological analysis revealed the 3D disposition of the cardiac conduction system for the first time in an intact heart with this human congenital malformation. We observed displacement of the compact atrioventricular node inferiorly to the ostium of the coronary sinus. Myocyte orientation analysis revealed that the normal arrangement of the major atrial muscle bundles was preserved but was modified in the septal region. Models of electrical activation suggest the disposition of the myocytes within the atrial muscle bundles associated with the “fast pathway,” together with the displaced atrioventricular node, serve as potential substrates for re-entry and possibly atrial fibrillation. This study used archived human hearts, showing them to be a valuable resource for the mathematical modeling community, and opening new possibilities for the investigations of arrhythmogenesis and ablation strategies in the congenitally malformed heart.

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Section: Discussioncontrasting

confidence: 99%

Section: Discussionsupporting

confidence: 90%

Section: Introductionmentioning

confidence: 97%

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Morphological Substrates for Atrial Arrhythmogenesis in a Heart With Atrioventricular Septal Defect

et al. 2018

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“…Reports of other substrates in children successfully cryoablated include ectopic atrial trachycardia, 57 junctional ectopic tachycardia, 57,58 and permanent junctional reciprocating tachycardia 59 . In patients with congenital heart disease, particular indications may include presence of an intracardiac shunt in order to minimize thromboembolic risk 2 and anatomical displacement of the AV conduction system for cryomapping of the AV node and/or its inputs 61 …”

Section: Cryoablation In the Youngmentioning

confidence: 99%

Transcatheter Cryoablation Part II: Clinical Utility

Lemola

Dubuc

Khairy

2008

Pacing Clinical Electrophis

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“…3 An inverted relationship of the slow and fast pathways has also been reported in patients with this anatomy. 16 A left-sided ablation of the posterior extension of the atrioventricular node may be another approach in these patients.…”

Section: Avnrt In Atrioventricular Canal Defectsmentioning

confidence: 99%

Atrioventricular Nodal Reentrant Tachycardia in Patients With Congenital Heart Disease

Papagiannis¹,

Beissel²,

Krause³

et al. 2017

Circ: Arrhythmia and Electrophysiology

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Background-The relationship of atrioventricular nodal reentrant tachycardia to congenital heart disease (CHD) and the outcome of catheter ablation in this population have not been studied adequately. Methods and Results-A multicenter retrospective study was performed on patients with CHD who had atrioventricular nodal reentrant tachycardia and were treated with catheter ablation. There were 109 patients (61 women), aged 22.1±13.4 years. The majority, 86 of 109 (79%), had CHD resulting in right heart pressure or volume overload. Patients were divided into 2 groups: group A (n=51) with complex CHD and group B (n=58) with simple CHD. There were no significant differences between groups in patients' growth parameters, use of 3-dimensional imaging, and type of ablation (radiofrequency versus cryoablation

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Partial atrioventricular canal defect with inverted atrioventricular nodal input into an inferiorly displaced atrioventricular node

Cited by 23 publications

References 12 publications

Morphological Substrates for Atrial Arrhythmogenesis in a Heart With Atrioventricular Septal Defect

Morphological Substrates for Atrial Arrhythmogenesis in a Heart With Atrioventricular Septal Defect

Transcatheter Cryoablation Part II: Clinical Utility

Atrioventricular Nodal Reentrant Tachycardia in Patients With Congenital Heart Disease

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