Nodoventricular Pathway Associated With Twin AV Nodes: Complexity of Ablation in Single Ventricle Physiology
ABSTRACT We report the case of a patient with heterotaxy syndrome including complex single ventricular morphology and interrupted IVC in association with twin conduction systems and a nodoventricular accessory pathway connection. The presence of 3 distinct QRS morphologies was inadvertently discovered during a hemodynamic catheterization study and prompted formal EP testing prior to hepatic venous inclusion into the Fontan circuit and loss of access to the atrial chamber for testing and therapy. This patient underscores the importance of close surveillance and high index of suspicion of arrhythmia mechanisms in patients with heterotaxy syndrome in conjunction with single ventricle morphology.
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ABSTRACT: OPINION STATEMENT: Post-operative arrhythmias occur commonly following pediatric cardiac surgery and can result in significant hemodynamic deterioration. Arrhythmias are more likely in the immediate post-operative period due to myocardial injury and ischemia, high catecholamine levels and electrolyte disturbances. At the same time, these arrhythmias are more likely to cause hemodynamic compromise due to the relatively tenuous cardiovascular function of patients immediately after cardiac surgery. Treatment of tachyarrhythmias often requires addressing two separate goals: termination of the arrhythmia and prevention of recurrences. These two objectives necessitate different treatment strategies in reentrant arrhythmias such as orthodromic reciprocating tachycardia, atrial fibrillation and intra-atrial reentrant tachycardia, including atrial flutter. Termination usually involves vagal maneuvers, adenosine administration, rapid pacing, or direct-current cardioversion. Prevention of recurrences, on the other hand, may require antiarrhythmic medications, although removal of central venous lines in the heart should be considered. Conversely, for automatic tachyarrhythmias, such as junctional ectopic tachycardia and ectopic atrial tachycardia, termination and prevention of recurrences usually involve similar modalities, including cooling, antiarrhythmic medications and correction of electrolyte abnormalities. Bradyarrhythmias, either sinus node dysfunction or AV block, can be treated by pacing with temporary epicardial wires in the short-term. In patients with persistent AV block for 7 days, however, permanent pacemaker placement may be required.Current Treatment Options in Cardiovascular Medicine 08/2012; 14(5):443-54. DOI:10.1007/s11936-012-0195-4
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ABSTRACT: So-called heterotaxy affects lateralization of the thoracic and abdominal organs. Congenital malformations may be present in one of several organ systems. Cardiac involvement includes both structural and conduction abnormalities. Data regarding arrhythmias in heterotaxy come from case reports and small case series. We pooled available data to further characterize arrhythmias in heterotaxy. A systematic review of the literature for manuscripts describing arrhythmias in heterotaxy patients was conducted. Databases including PubMed, EMBASE, and Ovid were searched. Studies describing arrhythmias in patients with heterotaxy were included if they were in English and presented characteristics of the arrhythmias. Arrhythmia characteristics were abstracted and are presented as pooled data. Freedom from arrhythmia by age was then analyzed using Kaplan-Meier analysis. A total of 19 studies with 121 patients were included in the pooled analysis. Those with right isomerism were found to be more likely to have atrial flutter, atrial tachycardia, junctional tachycardia, and ventricular tachycardia. Those with left isomerism were more likely to have atrioventricular block, intraventricular conduction delay, sick sinus syndrome, and atrioventricular nodal reentry tachycardia. Median age of onset for all arrhythmias was 4 years with no difference by specific arrhythmia or isomerism. Those with right and left isomerism are at risk for different arrhythmias but are likely to develop arrhythmias at the same age. Those with left isomerism are more likely to require pacemaker placement due to atrioventricular block. Understanding these differences allows for focused surveillance of development of these arrhythmias. © 2015 Wiley Periodicals, Inc.Congenital Heart Disease 07/2015; DOI:10.1111/chd.12288 · 1.20 Impact Factor