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OBJECTIVE: The \"2024 AHA/ACC/AMSSM/HRS/PACES/SCMR Guideline for the Management of Hypertrophic Cardiomyopathy\" provides recommendations to guide clinicians in the management of patients with hypertrophic cardiomyopathy.
METHODS: A comprehensive literature search was conducted from September 14, 2022, to November 22, 2022, encompassing studies, reviews, and other evidence on human subjects that were published in English from PubMed, EMBASE, the Cochrane Library, the Agency for Healthcare Research and Quality, and other selected databases relevant to this guideline. Additional relevant studies, published through May 23, 2023, during the guideline writing process, were also considered by the writing committee and added to the evidence tables, where appropriate.
UNASSIGNED: Hypertrophic cardiomyopathy remains a common genetic heart disease reported in populations globally. Recommendations from the \"2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients With Hypertrophic Cardiomyopathy\" have been updated with new evidence to guide clinicians.

UNASSIGNED: The \"2024 AHA/ACC/AMSSM/HRS/PACES/SCMR Guideline for the Management of Hypertrophic Cardiomyopathy\" provides recommendations to guide clinicians in the management of patients with hypertrophic cardiomyopathy.
UNASSIGNED: A comprehensive literature search was conducted from September 14, 2022, to November 22, 2022, encompassing studies, reviews, and other evidence on human subjects that were published in English from PubMed, EMBASE, the Cochrane Library, the Agency for Healthcare Research and Quality, and other selected databases relevant to this guideline. Additional relevant studies, published through May 23, 2023, during the guideline writing process, were also considered by the writing committee and added to the evidence tables, where appropriate.
UNASSIGNED: Hypertrophic cardiomyopathy remains a common genetic heart disease reported in populations globally. Recommendations from the \"2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients With Hypertrophic Cardiomyopathy\" have been updated with new evidence to guide clinicians.

法布雷病（FD）是一种X染色体连锁遗传疾病，因GLA基因突变，导致其编码的α半乳糖苷酶A（α-Gal A）活性降低或完全缺乏，造成代谢底物三己糖酰基鞘（Gb3）及衍生物脱乙酰基Gb3（Lyso-Gb3）在多种细胞和组织中贮积，引起多脏器病变。在心血管系统中，FD主要会导致左心室肥厚和（或）传导异常，即FD心肌病。由于FD心肌病是FD成人患者死亡的主要原因，因此结合心脏影像学、酶和底物活性、基因检测以及组织活检等方法的早期诊断，以及早期特异性酶替代疗法对于改善患者预后非常重要。本共识综合总结国内外已发表的FD心肌病诊断与治疗的相关证据，为FD心肌病的诊断与管理提供依据。.

The group of cardiomyopathies has received increasing attention over the last few years after some of the causes were identified and they could be characterized more exactly using modern imaging methods. New definitions and classification schemes were regularly provided by national and international cardiac societies. The new guidelines of the European Society of Cardiology (ESC) from 2023 on the management of cardiomyopathies are the first guidelines that comprehensively address all cardiomyopathies in one document. As these are new guidelines most of the recommendations are also new. An exception is the section on hypertrophic cardiomyopathy (HCM), which provides a targeted update of the 2014 ESC guidelines on the diagnosis and treatment of HCM. The main aim of the guidelines is to provide clear guidance for the diagnosis of cardiomyopathies, to highlight general assessment and management problems and to point out the relevant scientific evidence for the recommendations to the readership. Due to the magnitude detailed descriptions and recommendations cannot be provided for each individual cardiomyopathy phenotype; however, reference is made to the relevant literature.
UNASSIGNED: Die Gruppe der Kardiomyopathien hat in den letzten Jahren verstärkt Aufmerksamkeit erhalten, nachdem einige ihrer Ursachen identifiziert und sie mithilfe moderner Bildgebungsmethoden genauer charakterisiert werden konnten. Regelmäßig wurden von nationalen und internationalen Fachgesellschaften neue Definitionen und Klassifikationsschemata bereitgestellt. Die neue Leitlinie der European Society of Cardiology (ESC) von 2023 zum Management der Kardiomyopathien ist nun international die erste Guideline, die umfassend alle Kardiomyopathien in einem Dokument behandelt. Es handelt sich um eine neue Leitlinie, sodass die meisten Empfehlungen ebenso neu sind. Eine Ausnahme bildet der Abschnitt zur hypertrophen Kardiomyopathie (HCM), bei dem es sich um eine Aktualisierung der ESC-Leitlinie von 2014 zur Diagnose und Behandlung der HCM handelt. Das Hauptziel dieser Leitlinie besteht darin, einen klaren Leitfaden für die Diagnose von Kardiomyopathien bereitzustellen, allgemeine Bewertungs- und Managementprobleme zu betonen und den Leser auf die relevante wissenschaftliche Evidenzbasis für die Empfehlungen hinzuweisen. Aufgrund des Umfangs können keine detaillierten Beschreibungen und Empfehlungen für jede spezifische Kardiomyopathie bereitgestellt werden, jedoch wird auf die entsprechende Literatur verwiesen.

Electrocardiographic findings and arrhythmias are common in cardiomyopathies. Both may be an early indication of a specific diagnosis or may occur due to myocardial fibrosis and/or reduced contractility. Brady- and tachyarrhythmias significantly contribute to increased morbidity and mortality in patients with cardiomyopathies. Antiarrhythmic therapy including risk stratification is often challenging and plays a major role for these patients. Thus, an \"electrophysiological\" perspective on guidelines on cardiomyopathies may be warranted. As the European Society of Cardiology (ESC) has recently published a new guideline for the management of cardiomyopathies, this overview aims to present key messages of these guidelines. Innovations include a new phenotype-based classification system with emphasis on a multimodal imaging approach for diagnosis and risk stratification. The guideline includes detailed chapters on dilated and hypertrophic cardiomyopathy and their phenocopies, arrhythmogenic right ventricular cardiomyopathy, and restrictive cardiomyopathy as well as syndromic and metabolic cardiomyopathies. Patient pathways guide clinicians from the initial presentation to diagnosis. The role of cardiovascular magnetic resonance imaging and genetic testing during diagnostic work-up is stressed. Concepts of rhythm and rate control for atrial fibrillation have led to new recommendations, and the role of defibrillator therapy in primary prevention is discussed in detail. Whilst providing general guidelines for management, the primary objective of the guideline is to ascertain the disease etiology and disease-specific, individualized management.
UNASSIGNED: Arrhythmien und EKG(Elektrokardiographie)-Auffälligkeiten sind bei Kardiomyopathien häufig. Sie können ein erster Hinweis auf eine spezifische Diagnose sein oder im Verlauf einer Erkrankung als Folge von Fibrosierung und/oder reduzierter Herzfunktion auftreten. Brady- und Tachyarrhythmien tragen dabei signifikant zu einer erhöhten Morbidität und Mortalität bei Patienten mit Kardiomyopathien bei. Eine antiarrhythmische Therapie einschließlich einer Risikostratifizierung ist oft eine Herausforderung und bei der Behandlung dieser Patienten von besonderer Bedeutung. Daher ist eine elektrophysiologische Sicht auf Empfehlungen zu Kardiomyopathien sinnvoll. Da die ESC (European Society of Cardiology) kürzlich eine neue Leitlinie zum Management von Kardiomyopathien veröffentlichte, werden in der vorliegenden Arbeit deren wichtigsten Empfehlungen mit besonderem Fokus auf der kardialen Elektrophysiologie vorgestellt. Innovationen umfassen eine neue phänotypbasierte Klassifikation mit Schwerpunkt auf multimodaler Bildgebung zur Diagnostik und Risikostratifizierung. Die aktuelle Leitlinie enthält ausführliche Kapitel zur dilatativen und hypertrophen Kardiomyopathie und deren Phänokopien, der arrhythmogenen rechtsventrikulären Kardiomyopathie, der restriktiven Kardiomyopathie sowie syndromalen und metabolischen Kardiomyopathien. Pfade leiten von der Erstvorstellung bis zur Diagnose und Therapie. Die Rolle der kardiovaskulären Magnetresonanztomographie und der genetischen Diagnostik erfährt einen besonderen Stellenwert. Konzepte zur Rhythmus- und Frequenzkontrolle bei Vorhofflimmern führten zu neuen Empfehlungen, und die Rolle der Defibrillatortherapie in der Primärprävention wird ausführlich diskutiert. Das Hauptziel der neuen Leitlinie ist, die Krankheitsursachen bestmöglich zu ermitteln, um eine spezifische, individualisierte Behandlung zu ermöglichen.

Hypertrophic cardiomyopathy is a disease characterized by left ventricular hypertrophy (with or without right ventricular hypertrophy) not explained by loading conditions, the origin of which may be genetic and whose phenotypic expression is highly variable. The novelties in terms of diagnosis, clinical development, and management have been the subject of an update of the recommendations of the European Society of Cardiology (ESC).
La cardiomyopathie hypertrophique est une maladie caractérisée par une hypertrophie ventriculaire gauche (avec ou sans hypertrophie ventriculaire droite) non expliquée par les conditions de charge, dont l’origine peut être génétique et dont l’expression phénotypique est très variable. Les nouveautés en termes diagnostique, de mise au point, et de prise en charge ont fait l’objet d’une mise à jour des recommandations de la Société Européenne de Cardiologie (ESC).

OBJECTIVE: Identifying patients with hypertrophic cardiomyopathy (HCM) who are candidates for implantable cardioverter defibrillator (ICD) implantation in primary prevention for sudden cardiac death (SCD) is crucial. The aim of this study was to externally validate the 2022 European Society of Cardiology (ESC) model and other guideline-based ICD class of recommendation (ICD-COR) models and explore the utility of late gadolinium enhancement (LGE) in further risk stratification.
METHODS: Seven hundred and seventy-four consecutive patients who underwent cardiac magnetic resonance imaging were retrospectively enrolled.
RESULTS: Forty-six (5.9%) patients reached the SCD-related endpoint during 7.4 ± 2.5 years of follow-up. Patients suffering from SCD had higher ESC Risk-SCD score (4.3 ± 2.4% vs. 2.8 ± 2.1%, P < .001) and LGE extent (13.7 ± 9.4% vs. 4.9 ± 6.6%, P < .001). Compared with the 2014 ESC model, the 2022 ESC model showed increased area under the curve (.76 vs. .63), sensitivity (76.1% vs. 43.5%), positive predictive value (16.8% vs. 13.6%), and negative predictive value (98.1% vs. 95.9%). The C-statistics for SCD prediction of 2011 American College of Cardiology (ACC)/American Heart Association (AHA), 2014 ESC, 2020 AHA/ACC, and 2022 ESC models were .68, .64, .76 and .78, respectively. Furthermore, in patients without extensive LGE, LGE ≥5% was responsible for seven-fold SCD risk after multivariable adjustment. Whether in ICD-COR II or ICD-COR III, patients with LGE ≥5% and <15% showed significantly worse prognosis than those with LGE <5% (all P < .001).
CONCLUSIONS: The 2022 ESC model performed better than the 2014 ESC model with especially improved sensitivity. LGE enabled further risk stratification based on current guidelines.

Syncopal events in patients with hypertrophic cardiomyopathy (HCM) are of concern as they are a vital consideration in algorithms for risk stratification for sudden cardiac death (SCD) and ICD implantation. However, the cause of syncope is often under-investigated and/or unexplained. Current syncope guidelines do not provide a detailed definition of unexplained syncope. To address this important gap, an international panel of experts in the field of both syncope and HCM wrote a consensus document with the aim of providing practical guidance for the diagnosis and management of syncope in patients with HCM.

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