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In up to one-third of cases of sudden death, the medico-legal autopsy finding is inconclusive, and the option to perform a molecular autopsy is covered in international guidelines. The importance of postmortem genetic testing lies in its ability to identify hereditary diseases, often those with an autosomal dominant transmission pattern, and, through consultations and screening of relatives, to identify family members with a pathogenic mutation, who are often asymptomatic, providing an opportunity to change the course of their lives. The authors present three clinical cases that highlight the importance of postmortem genetic studies and family studies, as well as the integration of the data obtained in a cardiology consultation, which may be for arrhythmology, coronary disease or cardiomyopathy, depending on the specific condition. This could modify the course of the disease in many relatives., (Copyright © 2019. Publicado por Elsevier España, S.L.U.)

The authors report the first catheter ablation of Brugada syndrome in the literature using the Rhythmia™ mapping system. Learning points include: (1) low voltage areas can be documented while mapping in some individuals, suggesting that Brugada syndrome may not be a pure ion channel disorder; (2) typical long fractionated potentials can also be identified in the endocardium, supporting the need to map the endocardium in all Brugada patients requiring ablation; (3) disappearance of the typical coved pattern following ablation does not necessarily predict cure, as the patient we present experienced ventricular fibrillation recurrence a few months later., (Copyright © 2017 Sociedade Portuguesa de Cardiologia. Publicado por Elsevier España, S.L.U. All rights reserved.)

Brugada syndrome is a hereditary cardiac channelopathy that predisposes to ventricular tachyarrhythmias and, as a consequence, to sudden death. Its diagnosis is based on a specific electrocardiographic pattern. There are few reports on this syndrome in pregnancy. This article presents the case of a 26-year-old pregnant woman with implantable cardioverter-defibrillator due to this cause, who was referred to the National Department of Heart Disease in Pregnancy where she remained under monitoring, stable, without any complication. There are controversies about whether or not the increase in female hormones is a protective factor in pregnant women with Brugada syndrome. Despite the generally benign course of this disease during pregnancy, there are still important questions to be clarified. The follow-up of patients is considered essential to guarantee maternalfetal well-being. [ABSTRACT FROM AUTHOR]

Sudden cardiac death and malignant ventricular arrhythmias --in individuals without demonstrable structural heart disease by conventional methods--, have long been a subject of controversy. Primary electrical diseases became equivalent to purely electrical diseases, and the term "channelopathy" has undergone modifications because alterations in some interacting proteins are added to genetic mutations, and because there are structural abnormalities such as inflammation, fibrosis, adiposis, myocarditis, microaneurysms, increased collagen, mechanical dysfunction and others, evidenced by more sensitive diagnostic procedures: the heart is normal in appearance but the myocytes are not. In addition to the genetic basis (ion channel dysfunction) and the clinical presentation, structural alterations are integrated and Brugada, long QT and short QT syndromes are considered as organic- functional, electroanatomic diseases, requiring their reclassification, which is transcendental to stratify risk and adopt the most appropriate therapeutic approach. [ABSTRACT FROM AUTHOR]

Brugada syndrome is included among the channelopathies, first described in 1992. It is characterized by the presence of syncope, ventricular arrhythmias and sudden death in patients with a structurally healthy heart and an electrocardiographic pattern characterized by ST segment elevation from V1 to V3 leads and morphology of the right bundle branch block. [ABSTRACT FROM AUTHOR]

Copyright of Revista Española de Cardiología (18855857) is the property of Elsevier B.V. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Revista Española de Cardiología (18855857) is the property of Elsevier B.V. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Revista Española de Cardiología (18855857) is the property of Elsevier B.V. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Revista Española de Cardiología (18855857) is the property of Elsevier B.V. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)