An Ectopic Primary Pacemaker in Association with Isolated Persistent Left Superior Vena Cava.

We are reporting a case of a young Nepalese man, who was not known to have any past medical history, and who presented with palpitations. An electrocardiogram showed negative P-waves atrial rhythm in II, III, arteriovenous fistula, and V3-V6 with a variable block at 90-130 bpm. No positive "normal" P-waves were demonstrated in any tracing. He was found to have a congenital absence of the right superior vena cava (RSVC) along with persistent left superior vena cava (PLSVC) a condition also called isolated PLSVC (IPLSC). He underwent a treadmill stress test for further evaluation which showed a normal chronotropic response and the same persistent negative P-waves morphology. An invasive electroanatomical and activation mapping showed an absence of RSVC, the earliest atrial activation site in the anterior side of the inferior vena cava (IVC), and the absence of normal (positive) P-waves/normal sinus node (SN) activation. Considering all the available clinical data together, we believe that the patient was living with an ectopic pacemaker node that acts as a primary node and originated in the IVC/right atrium instead of the normal expected SN position. Given the high risk of complete sinoatrial nodal block in case of radiofrequency ablation, the patient was kept on medical treatment with a beta-blocker which was effective in controlling his symptoms and atrial arrhythmia.

Reduction in left atrial and pulmonary vein dimensions after ablation therapy is mediated by scar.

BACKGROUND: Ablative pulmonary vein isolation (PVI) decreases pulmonary vein (PV) and left atrial (LA) dimensions in atrial fibrillation (AF) patients. These changes are attributed to reverse structural remodeling following sinus rhythm restoration but evidence is lacking. We hypothesized that the downsizing is directly caused by the ablative energy and subsequent scar formation. METHODS: We studied cardiac magnetic resonance imaging in 21 paroxysmal AF patients before and 3 months after successful PVI and in healthy sheep (n = 12) before and after PVI of the right PV only. RESULTS: PVI decreased the PV diameter in patients and sheep by 11.0(10.3) and 9.2(11.0)%, (p < 0.001 and p = 0.020), respectively. The control left PV in sheep were unchanged. A linear correlation existed between the extent of PV scar and PVI-induced decrease in PV diameter in patients.After PVI, the LA volume decreased (103(38) vs. 92(31)ml, pre- vs. post-ablation, respectively, p = 0.006), while the right atrial (RA) volume was unchanged in patients. A decrease in active emptying fraction after ablation (26.5(10.7) vs. 21.8(10.6)%, pre- vs. post-ablation, p = 0.031) was associated with reduced contractility of the PV walls (p = 0.004). The contractility of the LA walls was unaltered (p = 0.749). CONCLUSION: The ablation-induced PV diameter reduction was similar in patients with AF and healthy sheep without AF and was associated with PV scar extent. The volume only decreased in LA and not RA after PVI, and wall contractility decreased only in ablated sites. Therefore, the PVI-induced atrial downsizing is caused by the ablative energy and subsequent scar formation.

Relationship Between Fibrosis Detected on Late Gadolinium-Enhanced Cardiac Magnetic Resonance and Re-Entrant Activity Assessed With Electrocardiographic Imaging in Human Persistent Atrial Fibrillation.

OBJECTIVES: This study sought to assess the relationship between fibrosis and re-entrant activity in persistent atrial fibrillation (AF). BACKGROUND: The mechanisms involved in sustaining re-entrant activity during AF are poorly understood. METHODS: Forty-one patients with persistent AF (age 56 ± 12 years; 6 women) were evaluated. High-resolution electrocardiographic imaging (ECGI) was performed during AF by using a 252-chest electrode array, and phase mapping was applied to locate re-entrant activity. Sites of high re-entrant activity were defined as re-entrant regions. Late gadolinium-enhanced (LGE) cardiac magnetic resonance (CMR) was performed at 1.25 × 1.25 × 2.5 mm resolution to characterize atrial fibrosis and measure atrial volumes. The relationship between LGE burden and the number of re-entrant regions was analyzed. Local LGE density was computed and characterized at re-entrant sites. All patients underwent catheter ablation targeting re-entrant regions, the procedural endpoint being AF termination. Clinical, CMR, and ECGI predictors of acute procedural success were then analyzed. RESULTS: Left atrial (LA) LGE burden was 22.1 ± 5.9% of the wall, and LA volume was 74 ± 21 ml/m2. The number of re-entrant regions was 4.3 ± 1.7 per patient. LA LGE imaging was significantly associated with the number of re-entrant regions (R = 0.52, p = 0.001), LA volume (R = 0.62, p < 0.0001), and AF duration (R = 0.54, p = 0.0007). Regional analysis demonstrated a clustering of re-entrant activity at LGE borders. Areas with high re-entrant activity showed higher local LGE density as compared with the remaining atrial areas (p < 0.0001). Failure to achieve AF termination during ablation was associated with higher LA LGE burden (p < 0.001), higher number of re-entrant regions (p < 0.001), and longer AF duration (p = 0.008). CONCLUSIONS: The number of re-entrant regions during AF relates to the extent of LGE on CMR, with the location of these regions clustering to LGE areas. These characteristics affect procedural outcomes of ablation.

Characteristics of Single-Loop Macroreentrant Biatrial Tachycardia Diagnosed by Ultrahigh-Resolution Mapping System.

BACKGROUND: Biatrial tachycardia (BiAT) is a rare form of atrial macroreentrant tachycardia, in which both atria form a critical part of the circuit. We aimed to identify the characteristics and precise circuits of single-loop macroreentrant BiATs. METHODS AND RESULTS: We identified 8 patients (median age, 59.5 years old) with 9 BiATs in a cohort of 336 consecutive patients from 2 institutions who had undergone AT catheter ablation using an automatic ultrahigh-resolution mapping system. Seven of the 8 patients had a history of persistent AF ablation, including septal or anterior left atrium ablation before developing BiAT. One of the 8 patients had a history of an atrial septal patch closure with a massively enlarged right atrium. Nine ATs (median cycle length, 334 ms; median 12 561 points in the left atrium; 8814 points in the right atrium) were diagnosed as single-loop macroreentrant BiATs. We observed 3 types of BiAT (1) BiAT with a perimitral and peritricuspid reentrant circuit (n=3), (2) BiAT using the right atrium septum and a perimitral circuit (n=3), and (3) BiAT using only the left atrium and right atrium septum (n=3). Catheter ablation successfully terminated 8 of the 9 BiATs. CONCLUSIONS: All patients who developed BiAT had an electric obstacle on the anteroseptal left atrium, primarily from prior ablation lesions. In this situation, mapping of both atria should be considered during AT. Because 3 types of single-loop BiAT were observed, ablation strategies should be adjusted to the type of BiAT circuit.

Revisiting anatomic macroreentrant tachycardia after atrial fibrillation ablation using ultrahigh-resolution mapping: Implications for ablation.

BACKGROUND: Anatomic macroreentrant atrial tachycardias (MATs) are conventionally reported to depend on the cavotricuspid isthmus, the mitral isthmus, or the left atrial roof, and are commonly seen following catheter ablation for atrial fibrillation. OBJECTIVES: To define the precise circuits of anatomic MAT with ultrahigh-resolution mapping. METHODS: In 57 patients (mean age, 62 years; 10 female) who developed ≥1 anatomic MAT, we analyzed 88 MAT circuits including 16 peritricuspid, 42 perimitral, and 30 roof-dependent circuits, using high-density mapping and entrainment. RESULTS: Of 16 peritricuspid atrial tachycardias (ATs), 8 (50.0%) showed a circuit not limited to the tricuspid annulus. However, cavotricuspid isthmus ablation terminated the tachycardia in all patients. Similarly, 26 of 42 perimitral ATs (61.9%) showed a circuit not limited to the mitral annulus, and a low-voltage zone <0.1 mV around the mitral annulus was associated with nontypical perimitral ATs (P < .0001). The practical isthmus was not in the mitral isthmus in 13 of these 26 perimitral ATs (50%). Finally, 22 of 30 roof-dependent ATs (73.3%) had a circuit not rotating around both pairs of pulmonary veins. Brief assessment of the activation direction on the posterior wall in relation to that on the septal, anterior, and lateral wall helped deduce the circuit of roof-dependent AT in 27 of 30 (90.0%). Practical isthmus was not in the roof in 8 of 22 (36.4%). Practical isthmuses mapped with the system were significantly shorter than the usual anatomic isthmuses (16.1 ± 8.2 mm vs 33.7 ± 10.4 mm) (P < .0001). CONCLUSIONS: High-density mapping successfully identified the precise circuits and the practical isthmus of anatomic MATs in patients with prior atrial fibrillation ablation.

Frontiers in non-invasive cardiac mapping: future implications for arrhythmia treatment.

Electrocardiographic mapping (ECM) is a noninvasive technique using body surface potentials and CT geometry to reconstruct epicardial maps. ECM is emerging as an important tool not only for diagnostic mapping, but also as a guide for trans-catheter ablation of complex arrhythmias such as atrial fibrillation. It provides the clinician with an immediate global view of the substrate, allowing easier pre-procedural planning, potentially improving clinical outcomes. Panoramic mapping of ventricular fibrillation (VF) is helping to develop a better understanding of its physiology, with future implications for the identification of therapeutic targets in patients with structural heart disease, as well as in idiopathic VF.

Electrogram signature of specific activation patterns: Analysis of atrial tachycardias at high-density endocardial mapping.

BACKGROUND: The significance of fractionated electrograms (EGMs) is object of debate, with multiple mechanisms described. OBJECTIVE: Using Rhythmia, a high-density mapping system, we sought to investigate the relationship between specific electrophysiological phenomena and EGM characteristics at those sites. METHODS: Twenty-five consecutive patients underwent high-density atrial mapping during atrial tachycardias. Bipolar EGMs were recorded with a 64-electrode basket catheter. The following atrial phenomena were identified: slow conduction (SC) areas, lines of block (LB), wavefront collisions (WFC), pivot sites (PS), and gaps. EGMs collected at these predefined areas were analyzed in terms of amplitude, duration, and morphology. RESULTS: Twenty-five atrial maps with 195 sites of interest (1755 EGMs) were object of our analysis. Thirty-five percent were sites of SC: fractionation had low amplitude (0.16 ± 0.07 mV) and long duration (87.8 ± 10.7 ms); wavefront collisions were seen in 38% of sites with EGMs shorter in duration (46.5 ± 4.5 ms) and higher in voltage (0.58 ± 0.13 mV); 17% were lines of block, never responsible for fractionation (0.13 ± 0.05 mV; 122.4 ms ± 24.8 ms); 9% were PS with a high degree of fractionation (0.55 ± 0.15 mV; 85.8 ± 7.9 ms). Two gaps were identified (1%) with a low degree of fractionation. CONCLUSION: Specific EGM characteristics in atrial tachycardia can be reproducibly linked to electrophysiological mechanisms. High-voltage and short-duration EGMs are associated with collision sites and PS that are unlikely to form critical sites for ablation; long-duration, low-voltage EGMs are associated with SC. However, not all SC regions will lie within the critical circuit and identification by only EGM characteristics cannot guide ablation.

Myocardial wall thinning predicts transmural substrate in patients with scar-related ventricular tachycardia.

BACKGROUND: Scar-related ventricular tachycardia (VT) arises from specific substrate according to etiology. OBJECTIVE: The purpose of this study was to evaluate the relationship between wall thinning (WT) on multidetector computed tomography (MDCT) and local abnormal ventricular activity (LAVA) in patients with ischemic cardiomyopathy (ICM), postmyocarditis (PMC), and dilated cardiomyopathy (DCM). METHODS: Forty-two patients (40 male, age 58 ± 13 years, 22 ICM, 11 PMC, 9 DCM) underwent MDCT before a combined endo-/epicardial VT ablation procedure. WT (<5 mm) and severe wall thinning (SWT) (<2 mm) area on MDCT were compared to the prevalence of endo-/epicardial LAVA during sinus rhythm. RESULTS: WT and SWT were found on MDCT in 36 (86%) and 20 (48%) with 42 ± 37 cm2 and 26 ± 24 cm2, respectively. SWT was frequently detected in ICM (ICM 77% vs PMC 27% vs DCM 0%, P <.001). LAVA were frequently observed on the endocardium in ICM and on the epicardium in PMC. Endo-/epicardial facing LAVA were frequently found within SWT areas (91% in <2 mm, 9% in 2-5 mm, and 0% in >5 mm, P < .001). In SWT areas, the presence of endocardial LAVA in ICM and epicardial LAVA in PMC predicted opposite facing LAVA with sensitivity and specificity of 78% and 48% and 79% and 98%, respectively. SWT predicted epicardial LAVA in ICM and endocardial LAVA in PMC with sensitivity and specificity of 89% and 100%, and 100% and 100%, respectively. CONCLUSION: SWT is frequently found in ICM and PMC but is not common in DCM. SWT predicts LAVA on the opposite side of the wall (epicardial in ICM and endocardial in PMC), indicating transmural VT substrate. MDCT is useful for identifying VT substrate and helpful for understanding the mechanisms of the location of VT substrate domain.

Impact of New Technologies and Approaches for Post-Myocardial Infarction Ventricular Tachycardia Ablation During Long-Term Follow-Up.

BACKGROUND: During the past years, many innovations have been introduced to facilitate catheter ablation of post-myocardial infarction ventricular tachycardia. However, the predictors of outcome after ablation were not thoroughly studied. METHODS AND RESULTS: From 2009 to 2013, consecutive patients referred for post-myocardial infarction ventricular tachycardia ablation were included. The end point of the procedure was complete elimination of local abnormal ventricular activities (LAVA) and ventricular tachycardia (VT) noninducibility. The predictors of outcome with primary end point of VT recurrence were assessed. A total of 125 patients were included (age: 64±11 years; 7 women) for 142 procedures. The left ventricle was accessed via transseptal, retrograde aortic, and epicardial approaches in 87%, 33%, and 37% of patients, respectively. Three-dimensional electroanatomical mapping system was used in 70%, multipolar catheter in 51%, and real-time image integration in 38% (from magnetic resonance imaging in 39% and multidetector computed tomography in 93%) of patients. Before ablation, VT was inducible in 75%, and endocardial/epicardial LAVA were present in 88%/75%. After ablation, complete LAVA elimination was achieved in 60%, and VT noninducibility in 83%. During a median follow-up of 850 days (interquartile range, 439-1707), VT recurrence was observed in 36%. Multivariable analysis identified 3 independent outcome predictors: the ability to achieve complete LAVA elimination (R(2)=0.29; P<0.0001; risk ratio=0.52 [0.38-0.70]), the use of real-time image integration (R(2)=0.21; P=0.0006; risk ratio=0.49 [0.33-0.74]), and the use of multipolar catheters (R(2)=0.08; P=0.05; risk ratio=0.75 [0.56-1.00]). CONCLUSIONS: Achievement of complete LAVA elimination and use of scar integration from imaging and multipolar catheters to focus high-density mapping are independent predictors of VT-free survival after catheter ablation for post-myocardial infarction ventricular tachycardia.

Characterization of the Left-Sided Substrate in Arrhythmogenic Right Ventricular Cardiomyopathy.

BACKGROUND: The correlates of left ventricular (LV) substrate in arrhythmogenic right ventricular (RV) cardiomyopathy are largely unknown. METHODS AND RESULTS: Thirty-two patients with arrhythmogenic RV cardiomyopathy (47±14 years; 6 women) were included. RV and LV dysplasia were defined from multidetector computed tomography and cardiac magnetic resonance imaging. Arrhythmias were characterized as right-sided or left-sided on 12-lead ECG recordings at baseline and during isoproterenol testing. In 14 patients, the imaging substrate was compared with voltage mapping and local abnormal ventricular activity. Imaging abnormalities were found in 32 (100%) and 21 (66%) patients on the RV and LV, respectively, intramyocardial fat on multidetector computed tomography being the most sensitive feature. LV involvement related to none of the Task Force criteria. Right-sided arrhythmias were more frequent than left-sided arrhythmias (P=0.003) although the latter were more frequent in case of LV involvement (P=0.02). The agreement between low voltage and fat on multidetector computed tomography was high on the RV when using either endocardial unipolar or epicardial bipolar data (κ=0.82 and κ=0.78, respectively) but lower on the LV (κ=0.54 for epicardial bipolar). LV local abnormal ventricular activity was found in all patients with LV involvement, and none of the others. The density of local abnormal ventricular activity within fat areas was similar between the RV and LV (P=0.57). CONCLUSIONS: LV substrate is frequent in arrhythmogenic RV cardiomyopathy, but poorly identified by current diagnostic strategies. Left-sided arrhythmias are more frequent in case of LV involvement. LV fat hosts the same density of local abnormal ventricular activity as RV fat, but is less efficiently detected by voltage mapping. These results support the need for alternative diagnostic strategies to identify LV dysplasia.

Noninvasive pacing study via pacemakers and implantable cardioverter-defibrillators for differentiating right from left atrial flutter.

BACKGROUND: Patients with atrial flutter who are implanted with a pacemaker (PM) or implantable cardioverter-defibrillator (ICD) present with the opportunity to perform a noninvasive pacing study (NIPS) using the right atrial pacing lead to differentiate right from left atrial flutter. OBJECTIVES: The purpose of this study was to study the feasibility and accuracy of NIPS to distinguish right from left atrial flutter. METHODS: We enrolled consecutive patients scheduled for an electrophysiological study or ablation procedure who were in atrial flutter and who were implanted with a PM or ICD with a functional atrial lead in the right atrial appendage. Flutter tachycardia cycle lengths (TCLs) and postpacing intervals (PPIs) were measured noninvasively via the device during the procedure. RESULTS: A total of 48 (67%) patients were studied. Right atrial flutter was present in 32 patients (of whom 29 had typical cavotricuspid isthmus-dependent flutter) and 16 (33%) patients had left atrial flutter. A PPI-TCL interval of >100 ms was 100% specific and 81% sensitive to identify left atrial flutter, with an overall accuracy of 94% and a c statistic of 0.94 (95% confidence interval 0.87-1.00). A PPI-TCL interval of ≤100 ms had a positive predictive value of 86% for diagnosing typical flutter. CONCLUSION: NIPS via PMs and ICDs with a PPI-TCL interval of >100 ms can reliably identify left atrial flutter (although we have only validated this cutoff for leads implanted in the right atrial appendage). This simple maneuver may allow planning for left-sided access and may avoid an unnecessary invasive electrophysiological study if left atrial flutter ablation is not to be considered.

Relevance of guideline-based ICD indications to clinical practice.

The implantable cardioverter-defibrillator (ICD) has established itself as life-saving therapy in patients at risk for sudden cardiac death. Remarkable technological advances have made ICDs easier and safer to implant, with improved therapeutic and diagnostic functions and reduced morbidity. Guidelines on ICD indications have been proposed by American and European scientific societies since a number of years, based upon trials and expert opinion. In the context of variable economic and political constraints, it is questionable whether these guidelines may be applied to all settings. This review discusses the guideline-based indications, critically examines their applicability to clinical practice, and discusses alternatives to ICD therapy.