Validation of a clinical model for predicting left versus right ventricular outflow tract origin of idiopathic ventricular arrhythmias.

BACKGROUND: Prediction of the chamber of origin in patients with outflow tract ventricular arrhythmias (OTVA) remains challenging. A clinical risk score based on age, sex and presence of hypertension was associated with a left ventricular outflow tract (LVOT) origin. We aimed to validate this clinical score to predict an LVOT origin in patients with OTVA. METHODS: In a two-center observational cohort study, unselected patients undergoing catheter ablation (CA) for OTVA were enrolled. All procedures were performed using an electroanatomical mapping system. Successful ablation was defined as a ≥80% reduction of the initial overall PVC burden after 3 months of follow-up. Patients with unsuccessful ablation were excluded from this analysis. RESULTS: We included 187 consecutive patients with successful CA of idiopathic OTVA. Mean age was 52 ± 15 years, 102 patients (55%) were female, and 74 (40%) suffered from hypertension. A LVOT origin was found in 64 patients (34%). A score incorporating age, sex and presence of hypertension reached 73% sensitivity and 67% specificity for a low (0-1) and high (2-3) score, to predict an LVOT origin. The combination of one ECG algorithm (V2 S/V3 R-index) with the clinical score resulted in a sensitivity and specificity of 81% and 70% for PVCs with R/S transition at V3 . CONCLUSION: The published clinical score yielded a lower sensitivity and specificity in our cohort. However, for PVCs with R/S transition at V3, the combination with an existing ECG algorithm can improve the predictability of LVOT origin.

Ventricular tachycardia as a consequence of triggered activity.

One of the less frequent underlying mechanisms of ventricular tachycardia (VT) is triggered activity. Triggered activity refers to an extrasystole due to a premature depolarization that occurs when the amplitude of an early or delayed afterdepolarization brings the cardiac membrane to its threshold potential. Hydrochlorothiazide and hydroxyzine can prolong repolarization and QT interval and are associated with early afterdepolarizations. Cyclic AMP-mediated, delayed afterdepolarizations can occur as a result of catecholaminergic surge. Delayed afterdepolarization is classically associated with outflow tract (OT) tachycardia, a type of VT that is uniquely defined by its termination with adenosine. We present a case of triggered OT tachycardia for which intravenous amiodarone through its antiadrenergic effect may have been effective. Infusions of magnesium and a cardioselective, ß-receptor antagonist that does not prolong repolarization may have been more appropriate given the concurrent, acquired prolonged QT syndrome. After initial stabilization, considering the underlying VT mechanism may prompt the clinician to select the most appropriate, further treatment.

Estimation of the origin of ventricular outflow tract arrhythmia using synthesized right-sided chest leads.

AIMS: For successful ablation of ventricular outflow tract arrhythmia, estimation of its origin prior to the procedure can be useful. Morphology and lead placement in the right thoracic area may be useful for this purpose. Electrocardiography using synthesized right-sided chest leads (Syn-V3R, Syn-V4R, and Syn-V5R) is performed using standard leads without any additional leads. This study evaluated the usefulness of synthesized right-sided chest leads in estimating the origin of ventricular outflow tract arrhythmia. METHODS AND RESULTS: This retrospective study included 63 patients in whom successful ablation of ventricular outflow tract arrhythmia was performed. Numbers of arrhythmias originating from the left ventricle, the septum of the right ventricle, and the free wall of the right ventricle were 11, 40, and 13, respectively. In one patient, two different left ventricular outflow tract origins were found. Electrocardiographic recordings from right-sided chest leads were divided into three types as follows: those in which an R > S concordance, a transitional zone, or an R < S concordance were detected. In all left arrhythmia cases, R > S concordance was observed. A transitional zone was evident in 34 of 40 cases of right ventricular outflow tract arrhythmia originating in the ventricular septum, and an R < S concordance was observed in 6 of the 40 cases. However, an R < S concordance was found in all cases of right ventricular outflow tract arrhythmia originating in the free wall. CONCLUSION: Synthesized right-sided chest lead electrocardiography may be useful for estimating the origin of ventricular outflow tract arrhythmia.

Usefulness of T(peak) -T(end) interval to distinguish arrhythmogenic right ventricular cardiomyopathy from idiopathic right ventricular outflow tract tachycardia.

BACKGROUND: The two predominant etiologies of right ventricular tachycardia (VT) are arrhythmogenic right ventricular cardiomyopathy (ARVC) and idiopathic VT arising from the right ventricular outflow tract (RVOT). Discrimination between these two entities is critical, as their prognoses and therapeutic options differ. The Tpeak -Tend (Tpe) interval reflects the transmural repolarization dispersion and its prolongation is associated with high mortality. METHODS: We compared the sinus rhythm electrocardiogram (ECG) of 43 patients (24 male, 43 ± 16 years) with VT originating from right ventricle. Five patients under antiarrhythmic drug therapy were excluded. Tpe interval was measured in each precordial leads and compared among patients with ARVC and RVOT-VT. RESULTS: Twenty-five patients (16 male, 42 ± 16 years) met the Task Force criteria for the diagnosis of ARVC, and 13 patients (seven male, 45 ± 14 years) had idiopathic RVOT tachycardia. Patients with ARVC had significantly prolonged Tpe intervals in all precordial leads compared to patients with idiopathic RVOT VT (137.1 ± 32.6 ms vs 93.8 ± 16.9 ms; P < 0.001 in V1, 133.2 ± 35.5 ms vs 104.7 ± 16.9 ms; P = 0.01 in V2, 125.7 ± 31.5 ms vs 99.1 ± 19.6 ms; P = 0.09 in V3, 121.9 ± 26.5 ms vs 92.3 ± 19.7 ms; P = 0.001 in V4, 123.1 ± 26.5 ms vs 99.5 ± 20:1 ms; P = 0.04 in V5 and 126.9 ± 32.2 ms vs 89 ± 11.3 ms; P < 0.001 in V6, respectively). For the diagnosis of ARVC, Tpe cut-off value of 97 ms in V1 had 84% sensitivity and 62% specificity (area under curve = 0.880). CONCLUSION: In patients with VT of RV origin, the prolonged Tpe interval in sinus rhythm electrocardiogram supports the diagnosis of ARVC.

Arrhythmogenic Right Ventricular Cardiomyopathy and Differential Diagnosis with Diseases Mimicking Its Phenotypes.

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited heart muscle disease, which is characterized by fibro-fatty replacement of predominantly the right ventricle (RV). The disease can result in ventricular tachyarrhythmias and sudden cardiac death. Our understanding of the pathophysiology and clinical expressivity of ARVC has been continuously evolving. The diagnosis can be challenging due to its variable expressivity, incomplete penetrance and the lack of specific diagnostic criteria. Idiopathic RV outflow tract tachycardia, Brugada Syndrome, athlete's heart, dilated cardiomyopathy, myocarditis, cardiac sarcoidosis, congenital aneurysms and diverticula may mimic clinical phenotypes of ARVC. This review aims to provide an update on the differential diagnosis of ARVC.

A case of mistaken arrhythmogenic identity during pregnancy.

Atypical LVOT ectopy can present with an RVOT morphology on ECG and differentiation to reveal this focus is in favor of benign idiopathic ventricular ectopy over an arrhythmogenic cardiomyopathy.

Biventricular myocardial strain analysis using cardiac magnetic resonance feature tracking (CMR-FT) in patients with distinct types of right ventricular diseases comparing arrhythmogenic right ventricular cardiomyopathy (ARVC), right ventricular outflow-tract tachycardia (RVOT-VT), and Brugada syndrome (BrS).

OBJECTIVES: As underlying heart diseases of right ventricular tachyarrhythmias, ARVC causes wall-motion abnormalities based on fibrofatty myocardial degeneration, while RVOT-VT and BrS are thought to lack phenotypic MR characteristics. To examine whether cardiac magnetic resonance (CMR) feature tracking (FT) in addition to ARVC objectively facilitates detection of myocardial functional impairments in RVOT-VT and BrS. METHODS: Cine MR datasets of four retrospectively enrolled, age-matched study groups [n = 65; 16 ARVC, 26 RVOT-VT, 9 BrS, 14 healthy volunteers (HV)] were independently assessed by two distinctly experienced investigators regarding myocardial function using CMR-FT. Global strain (%) and strainrate (s-1) in radial and longitudinal orientation were assessed at RVOT as well as for left (LV) and right (RV) ventricle at a basal, medial and apical section with the addition of a biventricular circumferential orientation. RESULTS: RV longitudinal and radial basal strain (%) in ARVC (- 12.9 ± 4.2; 11.4 ± 5.1) were significantly impaired compared to RVOT-VT (- 18.0 ± 2.5, p ≤ 0.005; 16.4 ± 5.2, p ≤ 0.05). Synergistically, RVOT endocardial radial strain (%) in ARVC (33.8 ± 22.7) was significantly lower (p ≤ 0.05) than in RVOT-VT (54.3 ± 14.5). For differentiation against BrS, RV basal and medial radial strain values (%) (13.3 ± 6.1; 11.8 ± 2.9) were significantly reduced when compared to HV (21.0 ± 6.9, p ≤ 0.05; 20.1 ± 6.6, p ≤ 0.005), even in case of a normal RV ejection fraction (EF) (> 45%; n = 6) (12.0 ± 2.7 vs. 20.1 ± 6.6, p ≤ 0.05). CONCLUSIONS: CMR-FT facilitates relevant differentiation in patients with right ventricular tachyarrhythmias: between ARVC against RVOT-VT and HV as well as between BrS with even a preserved EF against HV.

Idiopathic malignant premature ventricular contractions.

The presence of premature ventricular contractions (PVCs) in patients with structurally normal hearts was once considered a benign phenomenon. However, in susceptible patients, these "benign" or idiopathic PVCs may develop malignant potential and trigger ventricular fibrillation and result in sudden cardiac death. Alternatively, idiopathic PVCs can also induce cardiomyopathy. Clinical recognition of these entities can lead to effective targeted therapy. In the first instance, treatment consists of ablating the PVC source and implanting a defibrillator, whereas in the second scenario, ablating the PVC origin can normalize left ventricular function.

Comparative analysis of diagnostic 12-lead electrocardiography and 3-dimensional noninvasive mapping.

The clinical utility of noninvasive electrocardiographic imaging has been demonstrated in a variety of conditions. It has recently been shown to have superior predictive accuracy and higher clinical value than validated 12-lead electrogram algorithms in the localization of arrhythmias arising from the ventricular outflow tract, and displays similar potential in other conditions.

Taquicardia incesante del tracto de salida del ventrículo derecho - ¿Taquimiocardiopatía?: El enigma / Incessant right ventricular outflow tract tachycardia - Tachycardiomyopathy? The enigma

RESUMEN Las taquicardias ventriculares de larga duración o incesantes pueden causar insuficiencia cardíaca, disfunción del ventrículo izquierdo y cardiomiopatía, cuadros que revierten una vez resueltas las arritmias. Se trata de un diagnóstico de exclusión: puede existir una cardiopatía de base que empeora con la taquicardia y debe precisarse si la arritmia lleva a la cardiomiopatía (puede existir un subregistro) o viceversa. Se presenta un paciente con taquicardia incesante del tracto de salida del ventrículo derecho, de larga evolución, con repercusión hemodinámica, cardiomiopatía dilatada y disfunción grave del ventrículo izquierdo, rebelde a fármacos antiarrítmicos y con dos ablaciones fallidas, pendiente de biopsia endomiocárdica y nueva ablación de su taquicardia. Se discuten algunas pistas para diferenciar las arritmias del tracto de salida del ventrículo derecho, en sus variantes benignas (las más frecuentes) y malignas. Algunas pistas para diferenciarlas son: la duración del complejo QRS, el intervalo de acoplamiento de la extrasístole y la frecuencia de la taquicardia.

ABSTRACT Long-lasting or incessant ventricular tachycardias may cause heart failure, left ventricular dysfunction, and cardiomyopathy; conditions that reverse once the arrhythmias have been solved. This is a diagnosis of exclusion: there may be a basic heart disease that worsens with the tachycardia and it must be clarified whether the arrhythmia leads to cardiomyopathy (there may be an underregistration) or vice versa. A patient with incessant right ventricular outflow tract tachycardia, with long evolution, hemodynamic repercussion, dilated cardiomyopathy and severe dysfunction of the left ventricle, refractory to antiarrhythmic drugs and with two failed ablations, pending endomyocardial biopsy and new ablation of his tachycardia is presented. Some clues to differentiate right ventricular outflow tract arrhythmias, in their benign (most frequent) and malignant variants, are discussed. Some clues to differentiate them are: the duration of the QRS complex, the coupling interval of premature contractions and the heart rate of the tachycardia.

Noninvasive electrocardiographic mapping to guide ablation of outflow tract ventricular arrhythmias.

BACKGROUND: Localizing the origin of outflow tract ventricular tachycardias (OTVT) is hindered by lack of accuracy of electrocardiographic (ECG) algorithms and infrequent spontaneous premature ventricular complexes (PVCs) during electrophysiological studies. OBJECTIVES: To prospectively assess the performance of noninvasive electrocardiographic mapping (ECM) in the pre-/periprocedural localization of OTVT origin to guide ablation and to compare the accuracy of ECM with that of published ECG algorithms. METHODS: Patients with symptomatic OTVT/PVCs undergoing clinically indicated ablation were recruited. The OTVT/PVC origin was mapped preprocedurally by using ECM, and 3 published ECG algorithms were applied to the 12-lead ECG by 3 blinded electrophysiologists. Ablation was guided by using ECM. The OTVT/PVC origin was defined as the site where ablation caused arrhythmia suppression. Acute success was defined as abolition of ectopy after ablation. Medium-term success was defined as the abolition of symptoms and reduction of PVC to less than 1000 per day documented on Holter monitoring within 6 months. RESULTS: In 24 patients (mean age 50 ± 18 years) recruited ECM successfully identified OTVT/PVC origin in 23/24 (96%) (right ventricular outflow tract, 18; left ventricular outflow tract, 6), sublocalizing correctly in 100% of this cohort. Acute ablation success was achieved in 100% of the cases with medium-term success in 22 of 24 patients. PVC burden reduced from 21,837 ± 23,241 to 1143 ± 4039 (P < .0001). ECG algorithms identified the correct chamber of origin in 50%-88% of the patients and sublocalized within the right ventricular outflow tract (septum vs free-wall) in 37%-58%. CONCLUSIONS: ECM can accurately identify OTVT/PVC origin in the left and the right ventricle pre- and periprocedurally to guide catheter ablation with an accuracy superior to that of published ECG algorithms.

Nonreentrant ventricular arrhythmias in patients with structural heart disease unrelated to abnormal myocardial substrate.

BACKGROUND: Ventricular arrhythmias in the absence of structural heart disease are commonly referred to as "idiopathic." Patients with structural heart disease have ventricular arrhythmias with the same mechanisms and sites of origin as idiopathic ventricular arrhythmias, but the prevalence of such arrhythmias is not well defined. OBJECTIVES: To identify the prevalence of nonreentrant ventricular arrhythmias unrelated to abnormal myocardial substrate in patients with structural heart disease and to compare these arrhythmias to ventricular arrhythmias in patients with structurally normal hearts. METHODS: Of 249 consecutive patients referred for ablation of ventricular arrhythmias, 97 (39%) patients had nonreentrant arrhythmias unrelated to underlying structural heart disease. Fifty-five (57%) patients had structurally normal hearts, and 42 (43%) had underlying structural heart disease. RESULTS: Compared with patients with structurally normal hearts, patients with structural heart disease were more likely to have nonreentrant ventricular arrhythmias unrelated to underlying abnormal myocardial substrate originating from the aortic cusps and left ventricular outflow tract whereas patients without structural heart disease more often had arrhythmias originating from the right ventricular outflow tract. There was a significant increase in the average left ventricular ejection fraction after ablation in patients with structural heart disease. CONCLUSION: Nonreentrant ventricular arrhythmias unrelated to abnormal myocardial substrate are common in patients with structural heart disease, and sites of origin differ from those seen in patients with structurally normal hearts. When managing structural heart disease in patients with ventricular arrhythmias, a focus on arrhythmia mechanism, origin, and relationship to underlying myocardial substrate may have important implications for future treatment options and patient outcomes.