Novel Approaches for the Diagnosis of Concealed Nodo-Ventricular and His-Ventricular Pathways.

BACKGROUND: Confirming the presence and participation of concealed nodo-ventricular (cNV) or concealed His-ventricular (cHV) pathways in tachyarrhythmias is challenging. We describe novel observations to aid in diagnosing cNV or cHV pathways. METHODS: We present 7 cases of cNV and cHV pathway-mediated arrhythmias and focus on several laboratory observations: (1) differential ventricular overdrive pacing (VOD) from the base versus apex, (2) response to His refractory premature ventricular complexes, (3) paradoxical atriohisian response (shorter atriohisian interval during tachycardia than that during sinus rhythm) in long RP tachycardia, and (4) the role of adenosine to aid in the diagnosis. RESULTS: Three cases underwent differential VOD during tachycardia. All demonstrated a shorter postpacing interval minus tachycardia cycle length during basal pacing than apical pacing with one case exhibiting apical VOD results compatible with atrioventricular nodal reentrant tachycardia. Basal VOD was useful for localizing the ventricular connection in a case with cHV pathway. In 3 cases, His refractory premature ventricular complexes reset the tachycardia without conduction to the atrium, which excluded the involvement of an atrioventricular pathway or atrial tachycardia, or atrioventricular nodal reentrant tachycardia alone. One case had His refractory premature ventricular complexes followed by subsequent constant AA interval and then tachycardia termination, suggesting a bystander cNV pathway involvement. Two cNV pathway cases presented with long RP tachycardia had paradoxical atriohisian shortening of >15 ms, suggesting parallel activation of the atrium and the atrioventricular node. Adenosine terminated the tachycardia with retrograde block in 2 cases with cNV pathways but had no response on a cHV pathway. CONCLUSIONS: cNV and cHV pathways mediated tachyarrhythmias can present with variable clinical presentations. We emphasize the important role of differential VOD sites, His refractory premature ventricular complexes that reset or terminate the tachycardia without conduction to the atrium, paradoxical atriohisian response in long RP tachycardia, and the use of adenosine for diagnosing cNV and cHV pathways.

The Frog Sign Revisited.

The frog sign is a classic physical examination finding of typical atrioventricular nodal re-entrant tachycardia. We present the case of a 78-year-old man with recurrent, symptomatic supraventricular tachycardia referred for ablation in whom the frog sign was observed during physical examination.

Ventricular tachycardia induced by a backup pacing stimulus in a patient with a dual chamber pacemaker with AutoCapture.

Manufacturers of cardiac implantable electronic devices have incorporated automatic features to allow for remote monitoring, improve device longevity, and additional safety. Algorithms to automatically measure capture threshold and adjust output to preserve battery life are one such feature. Automatic features may occasionally result in unexpected or undesirable clinical outcomes. We report on a patient who developed ventricular tachycardia inadvertently induced by the AutoCapture. feature of an Abbott/St. Jude Medical (SJM) pacemaker.

Arrhythmogenic right ventricular cardiomyopathy in patients with biallelic JUP-associated skin fragility.

Arrhythmogenic right ventricular cardiomyopathy (ARVC), with skin manifestations, has been associated with mutations in JUP encoding plakoglobin. Genotype-phenotype correlations regarding the penetrance of cardiac involvement, and age of onset have not been well established. We examined a cohort of 362 families with skin fragility to screen for genetic mutations with next-generation sequencing-based methods. In two unrelated families, a previously unreported biallelic mutation, JUP: c.201delC; p.Ser68Alafs*92, was disclosed. The consequences of this mutation were determined by expression profiling both at tissue and ultrastructural levels, and the patients were evaluated by cardiac and cutaneous work-up. Whole-transcriptome sequencing by RNA-Seq revealed JUP as the most down-regulated gene among 21 skin fragility-associated genes. Immunofluorescence showed the lack of plakoglobin in the epidermis. Two probands, 2.5 and 22-year-old, with the same homozygous mutation, allowed us to study the cross-sectional progression of cardiac involvements in relation to age. The older patient had anterior T wave inversions, prolonged terminal activation duration (TAD), and RV enlargement by echocardiogram, and together with JUP mutation met definite ARVC diagnosis. The younger patient had no evidence of cardiac disease, but met possible ARVC diagnosis with one major criterion (the JUP mutation). In conclusion, we identified the same biallelic homozygous JUP mutation in two unrelated families with skin fragility, but cardiac findings highlighted age-dependent penetrance of ARVC. Thus, young, phenotypically normal patients with biallelic JUP mutations should be monitored for development of ARVC.

Atrial fibrillation post central retinal artery occlusion: Role of implantable loop recorders.

OBJECTIVE: This study evaluated the risk of subclinical atrial fibrillation (AF) in patients with central retinal artery occlusion (CRAO) compared to those with cryptogenic stroke using implantable loop recorders (ILR). METHODS: We conducted a retrospective analysis of 273 consecutive patients who had ILRs inserted at our institution for either cryptogenic stroke (n = 227) or CRAO (n = 46). Our primary endpoint was a time to event analysis for the new diagnosis of AF by ILR. Univariable and multivariable Cox proportional hazard models were used to determine the predictors of time-to-AF. RESULTS: A total of 64 patients were found to have newly diagnosed AF by remote monitoring of the ILR. AF was detected in 57 of 227 (25%) cryptogenic stroke patients by the end of a maximum 5.1 years follow-up and in seven of 46 (15%) CRAO patients by the end of a maximum 3.6 years follow-up (P = .215, log-rank test). The Kaplan-Meier estimates for freedom from AF was 59.4% for CRAO and 66.6% for cryptogenic stroke (P = NS, log-rank test). Baseline variables predicting AF included older patients, higher CHADS2 VASC score, longer PR interval on initial EKG evaluation, and mitral annular calcification on transthoracic echocardiogram. CONCLUSIONS: Patients with CRAO are at risk for subclinical AF, similar to those with cryptogenic stroke. Long-term monitoring to detect AF may lead to changes in pharmacotherapy to reduce the risk for subsequent stroke.

Pathophysiology, Diagnosis, and Ablation of Atrioventricular Node-dependent Long-R-P Tachycardias.

Atrioventricular (AV) node-dependent long-R-P tachycardias are a unique group of supraventricular tachycardias that include atypical AV nodal reentrant tachycardia (AVNRT), atypical AVNRT with a concealed bystander nodofascicular (NF)/nodoventricular (NV) accessory pathway inserting into the slow pathway of the AV node, the permanent form of junctional reciprocating tachycardia, and orthodromic NF/NV reciprocating tachycardia. Here, we discuss the complex pathophysiology, diagnosis, and ablation of these intriguing arrhythmias.

The various manifestations of concealed nodofascicular/nodoventricular bypass tracts.

BACKGROUND: The various arrhythmic manifestations of concealed nodofascicular (NF)/nodoventricular (NV) bypass tracts (BPTs) are poorly understood. OBJECTIVE: The purpose of the study was to define diagnostic criteria for supraventricular tachycardias (SVTs) associated with concealed nodal pathways (NPs). METHODS: We reviewed 11 patients with concealed NPs who underwent electrophysiology study and ablation for symptomatic SVT. RESULTS: Of 11 patients 7 (64% women; mean age 54 ± 16 years), NF/NV BPTs were active bystanders during atrioventricular nodal reentrant tachycardia (atypical [n = 4]; typical [n =2]) or participants during orthodromic NF/NV reentrant tachycardia (n = 5). The majority (10 of 11 [91%]) had nodal origin in the slow pathway (SP) and 7 of 11 (64%) presented as long RP SVT. Ablation of the SP targeting the right (n = 10) or left (n = 1) inferior extension eliminated concealed NP-associated SVTs in all patients. CONCLUSION: Concealed NF/NV BPTs are active bystanders equally as common as participants during SVT. They typically insert into the SP and often present as long RP SVT. SP ablation eliminates concealed NF/NV BPT-associated SVTs regardless of the mechanism.