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    Benefit of Implantable Cardioverter-Defibrillators in Patients with Catecholaminergic Polymorphic Ventricular Tachycardia - Reply

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    In Reply: We thank Maass and Cox for their comment on our work,1 and for stimulating the discussion on the role of implantable cardioverter-defibrillators (ICDs) in patients with catecholaminergic polymorphic ventricular tachycardia (CPVT). In our cohort, we did not observe an excess of crude mortality rates in ICD carriers (0.15 per 100 person-years) compared with noncarriers (0.56 per 100 person-years; P = .38). Considering that in this relatively young population, the dominant contributor to mortality is sudden cardiac death, we assessed the likelihood of survival at the occurrence of the first life-threatening arrhythmic event (LTAE; composite of sudden cardiac death, aborted cardiac arrest, or hemodynamically nontolerated sustained ventricular tachycardia), a previously validated hard end point.2,3 Our data show that patients without an ICD were 25-fold more likely to die at the occurrence of the first LTAE compared with ICD carriers, supporting the conclusion that ICD confers a survival benefit in high-risk patients with CPVT.1 However, when discussing the benefits and drawbacks of ICD, mortality should not be the only factor considered, since patients without an ICD may survive an out-of-hospital cardiac arrest with neurological disability secondary to anoxic brain injury, as in the case of 2 of 6 patients without an ICD who survived a cardiac arrest in our cohort. We agree with the authors that appropriate ICD shocks are not always necessary, especially on self-terminating arrhythmias. We acknowledge that a typographic mistake occurred during the editing of the article, and the sentence cited by the authors regarding the episodes terminated by the ICD should read, “Overall, all 15 episodes of VF [ventricular fibrillation] were successfully interrupted, while only 3 of 6 episodes (50%) of hemodynamically unstable, polymorphic fast VT [ventricular tachycardia] were terminated (P < .001).”4 We thank the authors for giving us the opportunity to clarify that the majority of ICD shocks on LTAE occurred on ventricular fibrillation, which are known to not cease spontaneously. This is also explained by our long-standing collaboration with Maurizio Gasparini, MD, which led to us to adopt into our clinical practice ICD programming with a long detection to deliver a shock only on ventricular fibrillation, a strategy demonstrated to reduce the rate of both appropriate and inappropriate shocks without differences in mortality.5 Regarding the 3 of 6 shocks (50%) on polymorphic VT that failed to terminate the arrhythmia, this is not surprising, since it is known that substrate (and arrhythmia mechanism) is a potent predictor of shock efficacy.6 Lastly, neither flecainide nor verapamil nor left cardiac sympathetic denervation have been demonstrated to reduce mortality in patients with CPVT at the time of the publication of this work. Considering that the practice of modern medicine should be based on the best evidence available, at the present time, these therapies cannot represent an alternative for ICD

    European Society of Cardiology Congress 2019 Together With the World Congress of Cardiology in Paris: The Scientific Chairs Give the Inside Track on Achieving Excellence and Engagement

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    How do you attract 33 500 people from around the world to a cardiovascular conference? Offer plenty of important new science. That was the recipe for the European Society of Cardiology (ESC) and its Congress 2019, held in Paris over 5 days this summer. Chairing the Program Committee of any congress is challenging enough, but organizing the Scientific Program of the world’s largest cardiovascular congress is a daunting task. This year, ESC President Professor Barbara Casadei appointed 2 chairs: Professor Silvia Priori and Professor Marco Roff

    What a congress!

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    The 2019 Annual Congress of the European Society of Cardiology (ESC), held in collaboration with the World Congress of Cardiology of the World Heart Federation, took place in Paris from August 31 to September 4. With more than 33 500 attendees, this meeting of the largest congress of cardiovascular (CV) medicine in the world achieved an all-time record of attendance. For the first time, it was hosted in a venue in downtown Paris, Port de Versailles, which has recently been extensively renovated. Access was easy thanks to a wide range of public transportations including the Metro. At the entrance, the ‘wow effect’ was created by a circular LED screen measuring more than 120 m long and 2 m high. The compact setting of the venue, with the vast majority of the activities taking place in the central three-story building, immediately conquered the attendees allowing delegates to easily become oriented and feel comfortable. Despite the attractiveness of Paris and the nice weather, the sessions were packed until the end of the day

    Programmed Electrophysiological Stimulation for Risk Prediction in Patients with Brugada Syndrome: Closing Time?,La estimulacion electrica programada para la prediccion del riesgo en pacientes con sindrome de Brugada: ?tiempo de cierre?

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    En un reciente artículo publicado en Revista Española de Cardiología, Rodríguez-Mañero et al.1 presentan su experiencia en la validación de 3 puntuaciones multiparamétricas diferentes propuestas a lo largo de la última década para ayudar a predecir el riesgo de muerte súbita cardiaca (MSC)2–4 en pacientes con síndrome de Brugada (SBr). La población de esta cohorte retrospectiva multicéntrica la formaron 831 pacientes con SBr de los que se llevó a cabo un estudio electrofisiológico (EFS) con estimulación eléctrica programada (EEP) y un seguimiento clínico durante una media de 10 años. El hallazgo clave del estudio fue que las 3 puntuaciones tenían una capacidad predictiva solo moderada, principalmente al aplicarlas a pacientes asintomáticos1. Los resultados obtenidos tienen interés, ya que el uso de desfibriladores automáticos implantables (DAI) continúa siendo la única opción para proteger a los pacientes con SBr que presentan un riesgo de MSC «suficientemente alto». Sin embargo, la decisión de implantar un DAI debe ponderarse cuidadosamente, ya que no está exenta de consecuencias

    Cardiac ryanodine receptors: Is a severe loss-of-function not so severe after all?

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    Cardiac ryanodine receptors: is a severe loss-of-function not so severe after al

    Genetic Abnormalities of the Sinoatrial Node and Atrioventricular Conduction

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    The peculiar electrophysiological properties of the sinoatrial node and the cardiac conduction system are key components of the normal physiology of cardiac impulse generation and propagation. Multiple genes and transcription factors and metabolic proteins are involved in their development and regulation. In this review, we have summarized the genetic underlying causes, key clinical findings, and the latest available clinical evidence. We will discuss clinical diagnosis and management of the genetic conditions associated with conduction disorders that are more prevalent in clinical practice, for this reason, very rare genetic diseases presenting sinus node or cardiac conduction system abnormalities are not discussed.Sin financiaciónNo data JCR 20210.422 SJR (2021) Q3, 200/356 Cardiology and Cardiovascular MedicineNo data IDR 2021UE

    Going Beyond Counting First Authors in Author Co-citation Analysis

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    The present study examines one of the fundamental aspects of author co-citation analysis (ACA) - the way co-citation counts are defined. Co-citation counting provides the data on which all subsequent statistical analyses and mappings are based, and we compare ACA results based on two different types of co-citation counting - the traditional type that only counts the first one among a cited work's authors on the one hand and a non-traditional type that takes into account the first 5 authors of a cited work on the other hand. Results indicate that the picture produced through this non-traditional author co-citation counting contains more coherent author groups and is therefore considerably clearer. However, this picture represents fewer specialties in the research field being studied than that produced through the traditional first-author co-citation counting when the same number of top-ranked authors is selected and analyzed. Reasons for these effects are discussed
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