Ablation of Supraventricular Tachycardias From Concealed Left-Sided Nodoventricular and Nodofascicular Accessory Pathways.

BACKGROUND: Nodoventricular and nodofascicular accessory pathways (AP) are uncommon connections between the atrioventricular node and the fascicles or ventricles. METHODS: Five patients with nodofascicular or nodoventricular tachycardia were studied. RESULTS: We identified 5 patients with concealed, left-sided nodoventricular (n=4), and nodofascicular (n=1) AP. We proved the participation of AP in tachycardia by delivering His-synchronous premature ventricular contractions that either delayed the subsequent atrial electrogram or terminated the tachycardia (n=3), and by observing an increase in VA interval coincident with left bundle branch block (n=2). The APs were not atrioventricular pathways because the septal VA interval during tachycardia was <70 ms in 3, 1 had spontaneous atrioventricular dissociation, and in 1 the atria were dissociated from the circuit with atrial overdrive pacing. Entrainment from the right ventricle showed ventricular fusion in 4 out of 5 cases. A left-sided origin of the AP was suspected after failed ablation of the right inferior extension of atrioventricular node in 3 cases and by observing a VA increase with left bundle branch block in 2 cases. The nodofascicular and 3 of the nodoventricular AP were successfully ablated from within the proximal coronary sinus (CS) guided by recorded potentials at the roof of the CS, and 1 nodoventricular AP was ablated via a transseptal approach near the CS os. CONCLUSIONS: Left-sided nodofascicular and nodoventricular AP appear to connect the ventricles with the CS musculature in the region of the CS os. Mapping and successful ablation sites can be guided by recording potentials within or near the CS os.

Use of Programmed Ventricular Extrastimulus During Supraventricular Tachycardia to Differentiate Atrioventricular Nodal Re-Entrant Tachycardia From Atrioventricular Re-Entrant Tachycardia.

OBJECTIVES: This study hypothesized that early coupled ventricular extrastimuli (V2) stimulation might yield a more robust differentiation between atrioventricular nodal re-entrant tachycardia (AVNRT) and atrioventricular re-entrant tachycardia (AVRT). BACKGROUND: Programmed V2 during supraventricular tachycardia are useful to differentiate AVNRT from AVRT by subtracting the ventriculoatrial (VA) interval from the stimulus to atrial depolarization (stimulus atrial [SA]) interval, but all such maneuvers have limitations. METHODS: Patients with either AVNRT or AVRT were investigated. The entire tachycardia cycle length (TCL) was scanned with V2 delivered from the right ventricular apex. The SA-VA difference was calculated with V2 clearly resetting the tachycardia. The prematurity of V2 was calculated by dividing the coupling interval (CI) by the TCL. RESULTS: A total of 210 patients (102 with AVNRT) were included. The SA-VA difference was >70 ms in all AVNRT patients and was <70 ms in all AVRT patients with right and septal accessory pathways (APs), except for those with decremental APs, in whom there was an overlap between AVNRT and AVRT with left APs. However, a SA-VA difference >110 ms with a CI/TCL of <65% distinguished AVNRT from AVRT using the left AP, with sensitivity and specificity of 87% and 100%, respectively. Ventricular overdrive pacing resulted in tachycardia termination or AV dissociation in 28% of patients compared with 15% of patients using the V2 technique (p = 0.008). CONCLUSIONS: A SA-VA of >70 ms using the V2 technique differentiated AVNRT from AVRT using septal and right APs. Use of the V2 technique with a short CI differentiated AVNRT from AVRT using left APs. The V2 technique less frequently resulted in tachycardia termination compared with ventricular entrainment.

Bundle Branch Re-Entrant Ventricular Tachycardia: Novel Genetic Mechanisms in a Life-Threatening Arrhythmia.

OBJECTIVES: This study sought to investigate for an underlying genetic etiology in cases of apparent idiopathic bundle branch re-entrant ventricular tachycardia (BBRVT). BACKGROUND: BBRVT is a life-threatening arrhythmia occurring secondary to macro-re-entry within the His-Purkinje system. Although classically associated with dilated cardiomyopathy, BBRVT may also occur in the setting of isolated, unexplained conduction system disease. METHODS: Cases of BBRVT with normal biventricular size and function were recruited from 6 North American centers. Enrollment required a clinically documented wide complex tachycardia and BBRVT proven during invasive electrophysiology study. Study participants were screened for mutations within genes associated with cardiac conduction system disease. Pathogenicity of identified mutations was evaluated using in silico phylogenetic and physicochemical analyses and in vitro biophysical studies. RESULTS: Among 6 cases of idiopathic BBRVT, each presented with hemodynamic compromise and 2 suffered cardiac arrests requiring resuscitation. Putative culprit mutations were identified in 3 of 6 cases, including 2 in SCN5A (Ala1905Gly [novel] and c.4719C>T [splice site mutation]) and 1 in LMNA (Leu327Val [novel]). Biophysical analysis of mutant Ala1905Gly Nav1.5 channels in tsA201 cells revealed significantly reduced peak current density and positive shifts in the voltage-dependence of activation, consistent with a loss-of-function. The SCN5A c.4719C>T splice site mutation has previously been reported as disease-causing in 3 cases of Brugada syndrome, whereas the novel LMNA Leu327Val mutation was associated with a classic laminopathy phenotype. Following catheter ablation, BBRVT was noninducible in all cases and none experienced a clinical recurrence during follow-up. CONCLUSIONS: Our investigation into apparent idiopathic BBRVT has identified the first genetic culprits for this life-threatening arrhythmia, providing further insight into its underlying pathophysiology and emphasizing a potential role for genetic testing in this condition. Our findings also highlight BBRVT as a novel genetic etiology of unexplained sudden cardiac death that can be cured with catheter ablation.

Clinical Features and Sites of Ablation for Patients With Incessant Supraventricular Tachycardia From Concealed Nodofascicular and Nodoventricular Tachycardias.

OBJECTIVES: This study sought to describe the clinical features and sites of successful ablation for incessant nodofascicular (NF) and nodoventricular (NV) tachycardias. BACKGROUND: Incessant supraventricular tachycardias have been associated with tachycardia-induced cardiomyopathies and have been previously attributed to permanent junctional reciprocating tachycardias, atrial tachycardias, and atrioventricular nodal re-entrant tachycardias. Incessant concealed NF and NV tachycardias have not been described previously. METHODS: Three cases of incessant concealed NF and NV re-entrant tachycardias were identified from 2 centers. RESULTS: The authors describe 3 cases with incessant supraventricular tachycardia resulting from NV (2 cases) and NF (1 case) pathways. Atrioventricular nodal re-entrant tachycardia was excluded by His synchronous premature ventricular complexes that either delayed or terminated the tachycardia. Ventricular pacing showed constant and progressive fusion in cases 1 and 3. In 2 cases, there was spontaneous initiation with a 1:2 response (cases 1 and 3); the presence of retrograde longitudinal dissociation or marked decremental pathway conduction in cases 1 and 3 sustains these tachycardias. The NV pathway was successfully ablated in the slow pathway region in case 3 and at the right bundle branch in case 1. The NF pathway was successfully ablated within the proximal coronary sinus in case 2. CONCLUSIONS: This is the first report of incessant supraventricular tachycardia using concealed NF or NV pathways. These tachycardias demonstrated spontaneous initiation from sinus rhythm with a 1:2 response and retrograde longitudinal dissociation or marked decremental pathway conduction. Successful ablation was achieved at either right-sided sites or within the coronary sinus.

Use of flecainide in combination antiarrhythmic therapy in patients with arrhythmogenic right ventricular cardiomyopathy.

BACKGROUND: Antiarrhythmic therapy is commonly used for suppression of arrhythmias in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) in conjunction with implantable cardioverter-defibrillators and catheter ablation. The efficacy of combination flecainide and sotalol/metoprolol therapy for patients refractory to single agents and/or catheter ablation has not been well established. OBJECTIVE: The purpose of this study was to describe our experience with the addition of flecainide in combination with sotalol/metoprolol for treatment of arrhythmias in patients with ARVC. METHODS: We reviewed all patients within our genetic arrhythmia program with a definite diagnosis of ARVC (45 patients) and identified 8 patients treated with a combination of flecainide with sotalol/metoprolol after failure of single-agent therapy and/or catheter ablation. These patients were monitored with at least yearly clinic visits and device interrogations focused on the detection of major ventricular arrhythmias. RESULTS: Of the 8 patients reviewed, 6 demonstrated excellent arrhythmia control after initiation of combination therapy with flecainide and sotalol/metoprolol. These patients have been arrhythmia-free for an average of 35.5 months. Two patients have demonstrated recurrent arrhythmias despite combination therapy and have undergone repeat epicardial and endocardial ablation. Recurrence was noted to occur within 2 months of therapy. Patients were diverse with regard to the severity of disease as well as in the presence of genetic mutations. CONCLUSION: The addition of flecainide in combination with sotalol/metoprolol may be an effective antiarrhythmic strategy for the control of ventricular arrhythmias in patients with ARVC refractory to single-agent therapy and/or catheter ablation.

The Effect of Direct Current Stimulation versus T-Wave Shock on Defibrillation Threshold Testing.

INTRODUCTION: There are several methods to induce ventricular fibrillation (VF) during defibrillation threshold (DFT) testing. Delivering a shock at a critical time during the T wave (T-shock) is the conventional approach, while delivering a constant direct current voltage (DC stim) from the implantable cardioverter defibrillator is an alternative method. Only a few reports compare VF induction methods. The purpose of this study was to evaluate the effects and safety of DC stim versus T-shock. METHODS: We retrospectively investigated 414 consecutive patients undergoing DFT testing. We compared the two groups (DC stim and T-shock) with respect to clinical characteristics, electrocardiogram (ECG) changes, and complications. RESULTS: Ventricular arrhythmia, including ventricular tachycardia (VT) and VF, was induced by DC stim in 93 patients or T-shock in 321 patients. No more than three attempts were performed during one procedure. There was no significant difference in the baseline ECG, induced tachycardia cycle length (TCL), or complications between the two groups. However, the induced TCL was significantly shorter than the clinical TCL regardless of induction method (P = 0.001). Five patients suffered major complications (i.e., electromechanical dissociation or incessant VT). A history of atrial fibrillation was significantly greater in patients with major complications than the others (80% vs 24%, P = 0.004), and was an independent predictor on multivariate analysis. CONCLUSIONS: There is no significant difference in induced TCL or complications between the DC stim and T-shock. The induced TCL is significantly shorter than clinical TCL regardless of induction method.

Variable clinical features and ablation of manifest nodofascicular/ventricular pathways.

BACKGROUND: Manifest nodofascicular/ventricular (NFV) pathways are rare. METHODS AND RESULTS: From 2008 to 2013, 4 cases were identified with manifest NFV pathways from 3 centers. The clinical findings and ablation sites are reported. All 4 cases presented with a wide complex tachycardia but with different QRS morphologies. Case 1 showed a left bundle branch block/superior axis, case 2 showed a right bundle branch block/inferior axis, case 3 showed a left bundle branch block/inferior axis, and case 4 showed a narrow QRS tachycardia and a wide complex tachycardia with a left bundle branch block/inferior axis. Three of the 4 tachycardias had atrioventricular dissociation ruling out extranodal accessory pathways, including atriofascicular pathways. Programmed extrastimuli showed evidence of a decremental accessory pathway in 3 of the 4 cases. Coexisting tachycardia mechanisms were seen in 3 of the 4 cases (atrioventricular nodal reentry tachycardia [2] and atrioventricular reentrant tachycardia [1]). Ablation in the slow pathway region eliminated the NFV pathway in 3 (transient in 1) with the other responding to surgical closure of a large atrial septal defect. The NFV pathway was a critical part of the tachycardia circuit in 1 and proved to be a bystander in the other 3 cases. CONCLUSIONS: Manifest NFV pathways presented with variable QRS expression dependent on the ventricular insertion site and often coexisted with other tachycardia mechanisms (atrioventricular nodal reentry tachycardia and atrioventricular reentrant tachycardia). In most cases, the atrial insertion of the pathway was in or near the slow pathway region. The NFV pathways were either critical to the tachycardia circuit or served as bystanders.

Idiopathic ventricular arrhythmia originating from the cardiac crux or inferior septum: epicardial idiopathic ventricular arrhythmia.

BACKGROUND: Idiopathic ventricular arrhythmia (VA) can arise from the epicardium near the posteroseptal region (cardiac crux). There are only 2 prior reports describing idiopathic VA from the cardiac crux. The purpose of this study was to characterize the clinical and the electrocardiographic features of idiopathic crux VA. METHODS AND RESULTS: Crux VA was identified in 18 patients undergoing catheter ablation. We divided patients into 2 groups, those with VA originating from the apical crux (n=9) and the basal crux (n=9). We described the clinical and electrocardiographic characteristics of crux VA as well as the ablation results. Furthermore, we compared clinical features of crux VA with other sites of idiopathic VA. Fifteen crux VA patients (83%) had sustained ventricular tachycardia and 3 patients required implantable cardioverter defibrillator implantation because of syncope. All patients had a left superior axis and 16 patients had R>S wave in V2. In apical crux VA, all patients had a deep S wave in V6 and 8 patients (89%) had R>S wave in aVR. All apical crux patients underwent attempted ablation in the middle cardiac vein without success. In 4 of these patients, epicardial ablation with subxiphoid approach was performed successfully. All basal crux VA patients had either negative or isoelectric pattern in V1 and had R>S in V6. Patients had successful ablation within the middle cardiac vein. CONCLUSIONS: Apical versus basal crux VA is identified as a new category of idiopathic VA with distinctive electrocardiographic characteristics; ablation via the middle cardiac vein is effective for eliminating basal crux VA, whereas apical crux VA often requires a subxiphoid epicardial approach.

Safety of radiofrequency catheter ablation without coronary angiography in aortic cusp ventricular arrhythmias.

BACKGROUND: Ventricular arrhythmias (VAs) originating from the aortic root are common. Coronary angiography is typically recommended before catheter ablation to document proximity of the ablation catheter to the coronary ostia. OBJECTIVE: To investigate how often catheter ablation in the aortic root could be guided by phased-array intracardiac echocardiography (ICE) and electroanatomic mapping without requiring aortography or coronary angiography. METHODS: We reviewed consecutive patients referred for aortic root VAs to operators experienced in the use of ICE at a single center. An ICE catheter and a 3.5-mm irrigated ablation catheter were used in all cases, and the need for angiography before ablation was documented. Acute success and acute and 30-day complications were noted. RESULTS: Thirty-five patients (age 58 ± 13 years; 74% men) were referred for the ablation of VAs; 32 of 35 (91%) underwent ablation using ICE and 3-dimensional mapping without the need for coronary angiography. Successful acute ablation was achieved in 29 of 35 (83%) patients. In all cases, the catheter tip was directly visualized with ICE >1 cm from the coronary ostia. The site of origin of the earliest VA was the left cusp (17 of 35 [49%]), right cusp (9 of 35 [26%]), right-left cusp junction (8 of 35 [23%]), or right-noncoronary cusp junction (1 of 35 [3%]). There were no cases of coronary injury, embolic stroke, aortic root perforation, worsening of aortic regurgitation, or death acutely or at 30 days. CONCLUSION: Radiofrequency ablation of VAs originating from the aortic root may be safely performed using ICE and electroanatomic mapping in the majority of cases without the need for coronary angiography.

Coupling interval dispersion and body mass index are independent predictors of idiopathic premature ventricular complex-induced cardiomyopathy.

INTRODUCTION: Patients with frequent premature ventricular complexes (PVCs) might be at risk for the developing or exacerbation of left ventricular (LV) dysfunction. However, some patients with a high-PVC burden do not develop cardiomyopathy, while other patients with low-PVC burden can develop cardiomyopathy. The purpose of this study was to evaluate the positive predictors of idiopathic PVCs-induced cardiomyopathy. METHODS AND RESULTS: We investigated 214 patients undergoing successful ablation of PVCs who had no other causes of cardiomyopathy. We divided the study cohort into 2 groups: ejection fraction (EF) ≥ 50% (normal LV) and EF < 50% (LV dysfunction). We analyzed the clinical characteristics, including the electrocardiogram and findings at electrophysiology study. Among these patients, 51 (24%) had reduced LVEF and 163 (76%) had normal LV function. Patients with LV dysfunction had significantly longer coupling interval (CI) dispersion (maximum-CI-minimum-CI) and had significantly higher PVC burden compared to those with normal LV function (CI-dispersion: 115 ± 25 milliseconds vs. 94 ± 19 milliseconds; P < 0.001; PVC burden: 19% vs. 15%; P = 0.04). Furthermore, patients with LV dysfunction had significantly higher body mass index (BMI) compared to those with normal LV function (BMI > 30 kg/m(2) ; 37% vs. 13%; P = 0.001). Logistic regression analysis showed that CI-dispersion, PVC burden, and BMI (>30 kg/m(2) ) are independent predictors of PVC-induced cardiomyopathy. CONCLUSIONS: In addition to the PVC burden, the CI-dispersion and BMI are associated with PVC-induced cardiomyopathy.

The year in review of clinical cardiac electrophysiology.

This past year saw multiple important advances in the field clinical cardiac electrophysiology. Seminal articles describing new anticoagulant drugs for stroke prevention in atrial fibrillation were published. New results that raise questions regarding the safety of dronedarone and several new promising techniques in AF ablation were described. Important articles that refine our understanding of the risk of sudden death among Wolff-Parkinson-White patients were published. In the basic and translational sciences, the application of gene therapy to the study and potential treatment of arrhythmias was described, whereas genetic determinants important to the optimal treatment of inherited arrhythmia syndromes were further elucidated. Issues relevant to cardiac rhythm device therapy included investigations into the St. Jude Riata lead, new applications of device monitoring, predicting response to cardiac resynchronization therapy, and the use of pacemakers for vasovagal syncope.

Frequency of atrial flutter after adult lung transplantation.

Lung transplantation, which involves an anastomosis of the graft to the native left atrium, may increase the risk of left-side atrial flutter (AFL). Our aim was to evaluate the incidence, predisposing conditions, and course of AFL after lung transplantation in adults. Two hundred sixty-nine consecutive patients who underwent lung transplantation were studied retrospectively. All patients received a preoperative echocardiogram and postoperative electrocardiographic monitoring. All 12-lead electrocardiograms were reviewed. Typical or atypical AFL was diagnosed by 2 independent reviewers based on accepted criteria. Predictors of AFL were investigated separately using univariate and multivariate logistic regression analyses. AFL occurred in 35 of 269 patients (13%) over a mean of 12 days after transplantation. All patients who developed AFL had no previous atrial arrhythmia. Of these 35 patients, 24 (68.6%) had atypical AFL by electrocardiographic criteria. In multivariate logistic regression analysis, patients with idiopathic pulmonary fibrosis (IPF) were 2.9 times more likely to have AFL than those patients with lung transplant without IPF (p = 0.009). Other independent risk factors for AFL were advanced age and preoperative left atrial enlargement. Only 3 of 35 patients (8.6%) with AFL had persistent atrial arrhythmia and needed electrophysiologic study and ablation. In conclusion, AFL is common soon after lung transplantation. Those with IPF, advanced age, or left atrial enlargement are at increased risk. In most cases, AFL is a self-limited arrhythmia that resolves spontaneously with no need for ablation.

QTc prolongation and family history of sudden death in a patient with desmin cardiomyopathy.

This case report describes a pregnant female patient who presented with new-onset congestive heart failure symptoms and prolonged QTc, with strong family history of sudden death. Endomyocardial biopsy and genetic testing revealed myocardial desmin accumulation and a previously described mutation in the DES (desmin) gene, as well as variants in two LQT genes, SCN5A and KCNH2. The case highlights the phenotypic variability for a particular desmin genotype, and the possible interaction of desminopathy with LQT variants not independently associated with large differences in current properties or QT prolongation from wild type.

Patient perception of symptoms and quality of life following ablation in patients with supraventricular tachycardia.

OBJECTIVES: It remains unclear which symptom experiences and aspects of quality of life (QOL) change after ablation in patients with supraventricular tachycardia (SVT). To determine how patient perceptions of symptoms and QOL change after ablation, we used a single group pretest-posttest design. METHODS: Patients with SVT (n=52; mean age 41+/-17 years; 65% female) completed generic and disease-specific measures at baseline and 1 month after ablation. RESULTS: Significant improvement after ablation was noted on virtually all measures (P <.05). Patients reported decreases from baseline regarding frequency and duration of episodes, number of symptoms, and impact of SVT on routine activities. All symptoms decreased in prevalence; however, no symptoms were completely eliminated at 1-month follow-up. Women, more so than men, reported larger changes in symptom and QOL scores after ablation. CONCLUSIONS: Despite the small sample, statistically significant improvement was found after ablation in a variety of patients with different symptoms and QOL indices.

Differences in accessory pathway location by sex and race.

BACKGROUND: The etiology of accessory pathway (AP) formation is generally unknown. OBJECTIVE: The purpose of this study was to test the hypothesis that AP formation is genetically mediated by examining whether AP location differs by sex and/or race, using sex and race as proxies to distinguish genetically different individuals. METHODS: This was a single-center, retrospective cohort study of 282 consecutive patients undergoing their first electrophysiology study that revealed at least one AP between 2004 and 2008. Sex and race were compared with AP location determined by invasive electrophysiology study. RESULTS: Eighty-nine (52%) males and 40 (36%) females had a left posterior AP (P = .006). Sixty-four (57%) females had a right annular AP, compared with 55 (32%) males (P <.001). After adjusting for age and race, females had 2.8-fold greater odds of having a right annular AP compared with males (95% confidence interval [CI] 1.70-4.65 greater odds; P <.001). While right anterior (free-wall) pathways were rare in all other races (12%), a significantly larger proportion of Asians (n = 10, 26%) had a right anterior AP (P = .017). After adjusting for sex and age, Asians had 3.8-fold greater odds of having a right anterior AP compared with other races (95% CI 1.5-9.4 greater odds; P = .004). CONCLUSIONS: Females more commonly had right annular APs, and Asians had right anterior APs substantially more frequently than other races. These findings suggest that the pathogenesis of AP formation may have a genetic component.