Value of Electrocardiography to Distinguish Fabry Disease from Sarcomeric Hypertrophic Cardiomyopathy.

Fabry disease (FD) is a rare genetic disorder that leads to left ventricular hypertrophy (LVH), frequently misdiagnosed as hypertrophic cardiomyopathy (HCM). We sought to assess the value of electrocardiography for distinguishing FD from HCM. We retrospectively reviewed and compared standard electrocardiograms and echocardiograms from 26 patients with FD and LVH and 33 sarcomeric patients with HCM, matched for gender, age, and degree of LVH. The mean age of patients with FD was 46 years (interquartile range) (28 to 53) and of HCM 50 (30 to 61) years (p = 0.27). Of them, 16 (61%) and 25 (76%) were male, respectively (p = 0.26). Indexed left ventricular mass was 166 g/m2 in FD versus 181 g/m2 in HCM (p = 0.88). All patients with FD and 30 (91%) with HCM were in sinus rhythm (p = 0.25). A higher prevalence of right bundle branch block (RBBB) was observed in FD (27%) versus HCM (6%) (p = 0.03). The PR interval was shorter in FD, 140 ms (120-160) versus 160 ms (140 to 180) (p = 0.004). P-wave duration was longer in patients with FD, 100 ms (80 to 120) versus 80 ms (80 to 100) (p = 0.01). The PQ interval (PR interval minus P-wave duration) was shorter in patients with FD, 40 ms (20 to 45) versus 80 ms (40 to 80) (p = 0.001). There were no differences regarding P-wave amplitude, QRS complex duration, corrected QT length, conduction or repolarization abnormalities, Sokolow-Lyon index, and Cornell index. After multivariate adjustments for RBBB, PR interval, P-wave duration, and PQ interval, a PQ interval ≤40 ms and RBBB were significantly associated with FD. In conclusion, there are electrocardiogram characteristics, such as the presence of RBBB or a PQ interval ≤40 ms, that may be helpful for screening and reducing the delay in FD diagnosis.

Utilidad de un sistema de registro de una derivación electrocardiográfica y transmisión inalámbrica durante la pandemia por COVID-19 / Electrocardiographic Recording System and Wireless Transmission During the COVID-19 Pandemic

RESUMEN Introducción: Se ha comunicado que algunos tratamientos utilizados para la infección por COVID-19 pueden ocasionar alteraciones del intervalo QT y arritmias graves. La medición por electrocardiograma (ECG) convencional requiere personal adicional y riesgo de contagio. Nuevas tecnologías para obtención de un ECG conectados a teléfonos inteligentes (smartphones) proporcionan una alternativa para evaluación del QTc. Objetivo: El objetivo fue evaluar la factibilidad de un dispositivo para registro electrocardiográfico de un canal, para la medición del intervalo QT en pacientes con sospecha o confirmación de infección por COVID-19, antes de recibir drogas que prolongan el intervalo QT. Material y métodos: Se obtuvieron registros de ECG con un dispositivo Kardia Mobile (KM) con trasmisión a un smarthphone. La sección de electrofisiología cardíaca centralizó la recepción por medio electrónico de los ECG en formato de archivo pdf y realizó las mediciones de los intervalos QTm y QTc. Resultados: Se estudiaron 31 pacientes, edad promedio 61 años (rango 20-95 años), sospechosos de presentar infección por COVID-19 enrolados para tratamiento con hidroxicloroquina, azitromicina, ritonavir y lopinavir. Los registros pudieron ser leídos en todos los casos, y debieron repetirse en dos casos. Los valores del intervalo QTc promedio en varones y mujeres fue 423 mseg (rango 380-457 mseg) y 439 mseg (rango 391-540 mseg), respectivamente. El tiempo de respuesta desde el envío del ECG al grupo de análisis fue 11 min (rango 1-155). Conclusiones: Los registros ECG obtenidos con dispositivos KM, para trasmisión a un smartphone a un grupo central de lectura, permitieron la medición del intervalo QTc en todos los pacientes.

ABSTRACT Background: Some therapies used for COVID-19 can prolong the QT interval and produce severe arrhythmias. QT interval measured from a standard electrocardiogram (ECG) requires additional personnel and risk of infection. Novel technologies to obtain an ECG connected to smartphones provide an alternative for the evaluation of corrected QT interval (QTc). Objective: The aim of this study was to evaluate the feasibility of using a single-lead ECG device to measure the QT interval in patients with suspected or confirmed COVID-19 before receiving treatment with drugs that can prolong the QT interval. Methods: The ECG was obtained with a KardiaMobile (KM) device and transmitted to a smartphone. The ECG recordings were saved as pdf files and electronically submitted to the electrophysiology section which centralized the reception and assessed the measured QT and QTc intervals. Results: A total of 31 patients (mean age 61 years, range 20-95 years) with suspected COVID-19 enrolled for treatment with hydroxychloroquine, azithromycin, ritonavir or lopinavir were analyzed. The recordings could be read in all the cases and had to be repeated in two cases. The mean value of the QTc interval was 423 ms (range 380-457 ms) in men and 439 ms (range 391-540 ms) in women. The response time since the ECG recording was submitted for analysis was 11 min (range 1-155). Conclusions: The QTc interval could be measured from ECG recordings obtained with KM devices connected to a smartphone and transmitted to a centralized reading center in all patients.

Catheter ablation in Patients with Electrical storm: The Calm after the Tempest / Ablación por catéter en pacientes con tormenta eléctrica: La calma tras la tempestad

Background: Catheter ablation (CA) has been shown to be effective in patients with recurrent ventricular tachycardia (VT); how-ever, its role in patients with electrical storm (ES) has not been studied in randomized trials. Objective: The aim of this study was to analyze ES cases treated with CA. Methods: This was a retrospective analysis of patients treated with CA for ES due to sustained monomorphic VT (SMVT). Procedure success was defined as lack of inducible VT at the end of ablation, partial success as the induction of non-clinical VT and failure as inducible clinical V T. Results:Sixteen procedures were performed in 14 patients: 10 successful, 3 partially successful and 3 failures. All patients were free from ventricular arrhythmia immediately after ablation. Ten patients (71.4%) were free from VT and 86.7% free from ES [8 (3-30)-month follow-up]. Five patients (35.7%) died from causes unrelated to arrhythmia. Conclusions: Catheter ablation is associated with acute suppression of VT in all patients with ES due to SMVT and with a recurrence-free outcome in most of them.

Introducción: La ablación por catéter (AC) ha demostrado que es beneficiosa en pacientes con taquicardia ventricular (TV) recur­rente, pero su rol en pacientes con tormenta eléctrica (TE) no se ha estudiado en ensayos aleatorizados. Objetivo: Analizar los casos de TE tratados con AC. Material y métodos: Análisis retrospectivo de pacientes con TE debida a TV monomorfa sostenida (TVMS) tratados mediante AC. Se definió éxito del procedimiento a la ausencia de TV inducible al final de la ablación, éxito parcial a la inducción de TV no clínica y no éxito a la inducibilidad de la TV clínica. Resultados:Se realizaron 16 procedimientos en 14 pacientes: 10 exitosos, 3 éxito parcial y 3 no exitosos. Todos los pacientes evolucionaron sin arritmia ventricular inmediatamente posablación. Diez pacientes (71,4%) evolucionaron sin TV y el 86,7% sin TE [seguimiento 8 (3-30) meses]. Cinco pacientes (35,7%) murieron de causa no arrítmica. Conclusiones: La AC se asocia con una supresión aguda de la TV en todos los pacientes con TE debida a TVMS y con una evolución sin recurrencia en la mayoría de ellos.

Diagnóstico diferencial entre doble respuesta ventricular y extrasistolia hisiana bigeminada / Differential diagnosis between dual ventricular response and bigeminy arising from the bundle of his

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