Isolated CCAVB is associated with a long-term risk for the development of DCM. Risk factors may be SSA/SSB antibodies, increased heart size at initial evaluation and the absence of pacemaker-associated improvement.

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The aim of this study was to evaluate the effect of pacemaker (PM) therapy in patients with isolated congenital complete atrioventricular block (CCAVB). Patients with CCAVB eventually quality for PM implantation, however, timing remains controversial. Retrospective evaluation of left ventricular end-diastolic diameter (LVEDD), shortening fraction (SF), and cardiothoracic ratio (CTR) in 149 CCAVB patients, before, at, and after PM implantation was carried out. LVEDD shows an average increase of 0.48%/month in non-PM patients, and an average decrease of 0.88%/month in PM patients. SF shows an average increase of 0.10%/month in non-PM, and an average decrease of 0.32%/month in PM patients. CTR shows an average increase of 0.02%/month in non-PM, and an average decrease of 0.19%/month in PM patients. The difference between the non-PM and PM groups is significant (P = 0.05) for all variables. Symptomatic patients show no significant change in LVEDD after PM therapy (from 66.5% before to 68.5% after PM therapy). Asymptomatic patients do show a significant (P < 0.001) decrease in LVEDD after PM therapy (from 78.4% before to 73.3% after PM therapy). CTR does not differ significantly between symptomatic and asymptomatic patients before PM therapy (58% and 57%, respectively). CTR does differ significantly (P < 0.001) between symptomatic and asymptomatic patients after PM therapy (52% and 48%, respectively). Heart size and SF are increased in most patients with isolated CCAVB. PM implantation is associated with a decrease in heart size and normalization of SF in most patients. Indications for PM therapy in children may require reevaluation in asymptomatic patients with increased cardiac size and decreased cardiac function.

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Background-Many patients with congenital heart disease require permanent pacing for rhythm management but cannot undergo transvenous lead placement. In others, epicardial scarring prohibits adequate sensing and pacing thresholds using epicardial leads. This study describes long-term lead performance using a transmural atrial (epicardial to endocardial) pacing approach in patients with congenital heart disease. Methods and Results-For transmural atrial (TMA) lead access, a bipolar, steroid-eluting transvenous lead was placed from the epicardium via purse-string incision or atriotomy and affixed to atrial endocardium. Records were reviewed for patient data and acute and long-term lead performance for TMA leads placed 1998 to 2004. Forty-two of 48 TMA leads remain active at last follow-up. Two leads fractured, 4 were functional at >5-year follow-up but no longer active. Freedom from lead failure 98% (95% confidence interval, 86%-100%) at mean follow-up 7.8 years. TMA leads gave excellent sensing and pacing characteristics at implant and chronically. Median acute and chronic sensing thresholds were 3 and 2.8 mV, respectively; median acute and chronic pacing thresholds at 0.5 ms were 0.9 and 0.7 V, respectively. TMA leads performed similarly in Fontan patients. Overdrive pacing for intra-atrial re-entrant tachycardia was successful in 7 of 8 patients. One patient with high baseline risk died of stroke 7 years after implant. No lead-associated thrombi were observed. Conclusions-TMA pacing leads had excellent longevity, initial, and chronic functional properties and provide an alternative to epicardial leads in patients with congenital heart disease. Patients who cannot receive transvenous leads, have epicardial scarring or have intra-atrial re-entrant tachycardia could benefit most from routine use of this technique. (Circ Arrhythm Electrophysiol. 2014;7:652-657.)

Catheter stability is paramount to safe and effective RFCA in septal locations. Use of these techniques resulted in acceptable success rates and low recurrence rate for RFCA of septal tachycardia substrates while avoiding inadvertent AV block in these young patients.

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