Congenital Heart Block.

Congenital complete heart block (CCHB) defines atrioventricular conduction abnormalities diagnosed in utero or within the first 27 days of life. Maternal autoimmune disease and congenital heart defects are most commonly responsible. Recent genetic discoveries have highlighted our understanding of the underlying mechanism. Hydroxychloroquine shows promise in preventing autoimmune CCHB. Patients may develop symptomatic bradycardia and cardiomyopathy. The presence of these and other specific findings warrants placement of a permanent pacemaker to relieve symptoms and prevent catastrophic events. The mechanisms, natural history, evaluation, and treatment of patients with or at risk for CCHB are reviewed.

Diagnostic utility of early premature ventricular complexes in differentiating atrioventricular reentrant and atrioventricular nodal reentrant tachycardias.

BACKGROUND: His-refractory premature ventricular complexes perturbing a supraventricular tachycardia (SVT) establish the presence of an accessory pathway (AP). Earlier premature ventricular complexes (ErPVCs) may perturb SVTs but are considered nondiagnostic. OBJECTIVE: The purpose of this study was to test the hypothesis that an ErPVC will always show a difference >35 ms in its advancement of the next atrial activation during atrioventricular nodal reentrant tachycardia (AVNRT). During atrioventricular reentrant tachycardia (AVRT), a PVC delivered close to the circuit can result in greater advancement of atrial activation due to retrograde conduction via an AP. Thus, an AP response, defined as ErPVC (H1S2) advancing the subsequent atrial activation (A1-A2) more than this minimum difference (A1A2 ≤ H1S2+35 ms), establishes the presence of an AP. METHODS: Sixty-five consecutive patients with SVT were retrospectively evaluated. ErPVCs were defined when the ventricular pacing stimulus was >35 ms ahead of the His during tachycardia. RESULTS: Among the 65 cases, 43 were AVNRT and 22 AVRT. Fourteen AVRT cases had an AP response with a mean H1S2+35 ms of 336 ± 58 ms and A1A2 of 309 ± 51ms. No AVNRT cases had an AP response. The specificity of an AP response to ErPVC in predicting AVRT was 100%. CONCLUSION: An AP response to PVCs (A1A2 ≤ H1S2+35 ms) is 100% specific for the presence of an AP.

Persistent 2-for-1 Atrioventricular Node Anterograde Conduction During a Supraventricular Tachycardia.

We present a case of persistent dual AV node conduction during AV node reentry tachycardia as a new clinical manifestation of 2-for-1 AV node conduction. The interpretation of the complex physiology ponders the possibility of an accessory pathway mediated atrioventricular reentry existing with more ventricular than atrial events.

Congenital Heart Block.

Congenital complete heart block (CCHB) defines atrioventricular conduction abnormalities diagnosed in utero or within the first 27 days of life. Maternal autoimmune disease and congenital heart defects are most commonly responsible. Recent genetic discoveries have highlighted our understanding of the underlying mechanism. Hydroxychloroquine shows promise in preventing autoimmune CCHB. Patients may develop symptomatic bradycardia and cardiomyopathy. The presence of these and other specific findings warrants placement of a permanent pacemaker to relieve symptoms and prevent catastrophic events. The mechanisms, natural history, evaluation, and treatment of patients with or at risk for CCHB are reviewed.

Novel approach to intracardiac defibrillator placement in patients with atriopulmonary Fontan: Ventricular defibrillation with an atrial positioned ICD lead.

INTRODUCTION: The Fontan procedure, used to palliate univentricular physiology, eliminates direct venous access to the ventricle and complicates implantable cardioverter-defibrillator (ICD) placement. METHODS AND RESULTS: We describe two patients with Fontan palliation who underwent a novel transvenous approach to ICD placement. The approach uses a transvenous bipolar lead placed in a coronary sinus branch for ventricular sensing, and a defibrillation lead placed in the right atrium for atrial sensing and ventricular defibrillation. CONCLUSION: Transvenous ICD implantation is possible in some patients with an atriopulmonary Fontan. This approach avoids a redo sternotomy for epicardial leads and excludes the need for lead placement in the systemic circulation.

Mechanism and interpretation of two-for-one response to premature atrial complexes during atrioventricular node re-entry tachycardia.

AIMS: The response to premature atrial complexes (PACs) during tachycardia has been shown to differentiate atrioventricular nodal re-entrant tachycardia (AVNRT) from focal junctional tachycardia (JT). His refractory PAC (HrPACs) perturbing the next His (resetting with fusion) is diagnostic of AVNRT and such a late PAC fusing with the native beat cannot reset the focal source of JT. Early PAC advancing the immediate His with continuation of tachycardia suggests JT but can also occur in AVNRT due to simultaneous conduction through the AV nodal fast and slow pathways [two-for-one response (TFOR)]. The objective of this study was to evaluate the incidence and mechanism of TFOR after early premature atrial complexes (ePACs) during AVNRT and to differentiate it from the known response to ePACs during JT. METHODS AND RESULTS: Typical AVNRT cases were diagnosed using standard criteria. We evaluated the responses to scanning PACs delivered during tachycardia in 100 patients undergoing AV node slow pathway modification for AVNRT. The responses to HrPACs and ePACs delivered from coronary sinus os or high right atrium were retrospectively reviewed. In 10 patients, ePACs advanced the immediate His with continuation of tachycardia. In all 10 cases, HrPACs advanced the next His, confirming AVNRT as the mechanism, and indicating a TFOR. CONCLUSION: A TFOR can occur in a small number of patients during AVNRT and is therefore not diagnostic of JT. However, HrPACs always perturbed the next His in these cases, confirming the diagnosis of AVNRT and allowing for differentiation from JT.

Differentiating Atrioventricular Reentry Tachycardia and Atrioventricular Node Reentry Tachycardia Using Premature His Bundle Complexes.

BACKGROUND: Current maneuvers for differentiation of atrioventricular node reentry tachycardia (AVNRT) and atrioventricular reentry tachycardia (AVRT) lack sensitivity and specificity for AVRT circuits located away from the site of pacing. We hypothesized that a premature His complex (PHC) will always perturb AVRT because the His bundle is obligatory to the circuit. Further, AVNRT could not be perturbed by a late PHC (≤20 ms ahead of the His) due to the retrograde His conduction time. Earlier PHCs can advance the AVNRT circuit but only by a quantity less than the prematurity of the PHC. METHODS: High-output pacing at the distal His location delivered PHCs. AVRT was predicted when late PHCs perturbed tachycardia or when earlier PHCs led to atrial advancement by an amount equal or greater than the degree of PHC prematurity. RESULTS: Among the 73 supraventricular tachycardias, the test accurately predicted AVRT (n=29) and AVNRT (n=44) in all cases. Late PHC advanced the circuit in all 29 AVRTs and none of the AVNRTs (sensitivity and specificity, 100%). With earlier PHCs, the degree of atrial advancement was equal or greater than the PHC prematurity in 26/29 AVRTs and none of the AVNRTs (90% sensitivity and 100% specificity). The mean prematurity of the PHC required to perturb AVNRT was 48 ms (range, 28-70 ms) and the advancement less than the prematurity of the PHC (mean, 32 ms; range, 18-54 ms). CONCLUSIONS: The responses to PHCs distinguished AVRT and AVNRT with 100% specificity and sensitivity.

Electrocardiographic features and prevalence of bilateral bundle-branch delay.

BACKGROUND: Definitive diagnosis of bilateral bundle-branch delay/block may be made when catheter-induced right bundle-branch block (RBBB) develops in patients with baseline left bundle-branch (LBB) block. We hypothesized that a RBBB pattern with absent S waves in leads I and aVL will identify bilateral bundle-branch delay/block. METHODS AND RESULTS: Fifty patients developing transient RBBB pattern in lead V1 during right heart catheterization were studied. Patients were grouped according to whether the baseline ECG demonstrated a normal QRS, left fascicular blocks, or LBB block pattern. The RBBB morphologies in each group were compared. The prevalence of bilateral bundle-branch delay/block pattern was examined in our hospital ECG database. All patients with baseline normal QRS complexes (n=30) or left fascicular blocks (4 anterior, 5 posterior) developed a typical RBBB pattern. Among the 11 patients with a baseline LBB block pattern, 7 developed an atypical RBBB pattern with absent S waves in leads I and aVL and the remaining 4 demonstrated a typical RBBB. The absence of S waves in leads I and aVL during RBBB was 100% specific and 64% sensitive for the presence of pre-existing LBB block. Among the consecutive 2253 hospitalized patients with RBBB, 34 (1.5%) had the bilateral bundle-branch delay/block pattern. CONCLUSIONS: An ECG pattern of RBBB in lead V1 with absent S wave in leads I and aVL indicates concomitant LBB delay. Pure RBBB and bifascicular blocks are associated with S waves in leads I and aVL.

Development of rapid preexcited ventricular response to atrial fibrillation in a patient with intermittent preexcitation.

Intermittent preexcitation during sinus rhythm is indicative of an accessory pathway at a very low risk for sudden death. We present the case of a 49-year-old man with intermittent preexcitation who subsequently developed rapid atrial fibrillation with a shortest preexcited R-R interval of 230 milliseconds. Electrophysiology study showed intermittent preexcitation at baseline and 1:1 anterograde accessory pathway conduction to 220 milliseconds in the presence of 1 mcg/min isoproterenol infusion. The pathway was successfully ablated at the lateral mitral annulus. Accessory pathways highly sensitive to catecholamines may show intermittent preexcitation at baseline with potential for rapid conduction during atrial fibrillation and sudden death.

The surface electrocardiogram predicts risk of heart block during right heart catheterization in patients with preexisting left bundle branch block: implications for the definition of complete left bundle branch block.

BACKGROUND: Patients with left bundle branch block (LBBB) undergoing right heart catheterization can develop complete heart block (CHB) or right bundle branch block (RBBB) in response to right bundle branch (RBB) trauma. We hypothesized that LBBB patients with an initial r wave (>or=1 mm) in lead V1 have intact left to right ventricular septal (VS) activation suggesting persistent conduction over the left bundle branch. Trauma to the RBB should result in RBBB pattern rather than CHB in such patients. METHODS: Between January 2002 and February 2007, we prospectively evaluated 27 consecutive patients with LBBB developing either CHB or RBBB during right heart catheterization. The prevalence of an r wave >or=1 mm in lead V1 was determined using 118 serial LBBB electrocardiographs (ECGs) from our hospital database. RESULTS: Catheter trauma to the RBB resulted in CHB in 18 patients and RBBB in 9 patients. All 6 patients with >or=1 mm r wave in V1 developed RBBB. Among these 6 patients q wave in lead I, V5, or V6 were present in 3. Four patients (3 in CHB group and 1 in RBBB group) developed spontaneous CHB during a median follow-up of 61 months. V1 q wave >or=1 mm was present in 28% of hospitalized complete LBBB patients. CONCLUSIONS: An initial r wave of >or=1 mm in lead V1 suggests intact left to right VS activation and identifies LBBB patients at low risk of CHB during right heart catheterization. These preliminary findings indicate that an initial r wave of >or=1 mm in lead V1, present in approximately 28% of ECGs with classically defined LBBB, may constitute a new exclusion criterion when defining complete LBBB.

Differentiating junctional tachycardia and atrioventricular node re-entry tachycardia based on response to atrial extrastimulus pacing.

OBJECTIVES: The purpose of this study was to differentiate non-re-entrant junctional tachycardia (JT) and typical atrioventricular node re-entry tachycardia (AVNRT). BACKGROUND: JT may mimic AVNRT. Ablation of JT is associated with a lower success rate and a higher incidence of heart block. Electrophysiologic differentiation of these tachycardias is often difficult. METHODS: We hypothesized that JT can be distinguished from AVNRT based on specific responses to premature atrial complexes (PACs) delivered at different phases of the tachycardia cycle: when a PAC is timed to His refractoriness, any perturbation of the subsequent His indicates that anterograde slow pathway conduction is involved and confirms a diagnosis of AVNRT. A PAC that advances the His potential immediately after it without terminating tachycardia indicates that retrograde fast pathway is not essential for the circuit and confirms a diagnosis of JT. This protocol was tested in 39 patients with 44 tachycardias suggesting either JT or AVNRT based on a short ventriculo-atrial interval and apparent AV node dependence. Tachycardias were divided into 3 groups: clinically obvious AVNRT, clinically obvious JT, and clinically indeterminate rhythm. RESULTS: In the 26 cases of clinically obvious AVNRT, the sensitivity and specificity of the test were 61% and 100%, respectively. In the 9 cases of clinically obvious JT, the sensitivity and specificity were 100% and 100%, respectively. In the 9 cases of clinically indeterminate rhythm, the technique indicated AVNRT in 1 patient and JT in 7 patients, and the test was indeterminate in 1 patient. CONCLUSIONS: The response to PACs during tachycardia can distinguish JT and AVNRT with 100% specificity in adult patients.

Syncope in children: diagnostic tests have a high cost and low yield.

OBJECTIVES: To assess the use, yield, and cost-effectiveness of diagnostic tests used in the evaluation of syncope in children. STUDY DESIGN: A retrospective review of 169 pediatric patients presenting to a tertiary care center with new onset syncope was undertaken. Test results were considered diagnostic when an abnormal result correlated with the clinical diagnosis or a normal result was obtained during a syncopal episode. Costs were based on the hospital cost of testing for fiscal year 1999, using a relative value unit-based costing methodology and did not include professional fees or costs of hospitalization. RESULTS: A total of 663 tests were performed at a cost of 180,128 dollars. Only 26 tests (3.9%) were diagnostic in 24 patients (14.2%). The average cost per patient was 1055 dollars, and the cost per diagnostic result was 6928 dollars. Echocardiograms, chest radiographs, cardiac catheterizations, electrophysiology studies, and serum evaluations were not diagnostic. CONCLUSIONS: The evaluation of pediatric syncope remains expensive, and testing has a low diagnostic yield. An approach that focuses on the use of testing to verify findings from the history and physical examination or exclude life-threatening causes is justified.