In the ESC 2023 guidelines, cardiomyopathies are conservatively defined as \'myocardial disorders in which the heart muscle is structurally and functionally abnormal, in the absence of coronary artery disease, hypertension, valvular disease, and congenital heart disease sufficient to cause the observed myocardial abnormality\'. They are morpho-functionally classified as hypertrophic, dilated, restrictive, and arrhythmogenic right ventricular cardiomyopathy with the addition of the left ventricular non-dilated cardiomyopathy that describes intermediate phenotypes not fulfilling standard disease definitions despite the presence of myocardial disease on cardiac imaging or tissue analysis. The new ESC guidelines provide \'a guide to the diagnostic approach to cardiomyopathies, highlight general evaluation and management issues, and signpost the reader to the relevant evidence base for the recommendations\'. The recommendations and suggestions included in the document provide the tools to build up pathways tailored to specific cardiomyopathy (phenotype and cause) and define therapeutic indications, including target therapies where possible. The impact is on clinical cardiology, where disease-specific care paths can be assisted by the guidelines, and on genetics, both clinics and testing, where deep phenotyping and participated multi-disciplinary evaluation provide a unique tool for validating the pathogenicity of variants. The role of endomyocardial biopsy remains underexploited and confined to particular forms of restrictive cardiomyopathy, myocarditis, and amyloidosis. New research and development will be needed to cover the gaps between science and clinics. Finally, the opening up to disciplines such as bioinformatics, bioengineering, mathematics, and physics will support clinical cardiologists in the best governance of the novel artificial intelligence-assisted resources.

Heart failure, a growing concern in the United States, significantly impacts both morbidity and mortality. Classified by ejection fraction, heart failure with preserved ejection fraction (HFpEF) now accounts for half of all cases and is steadily rising. Unlike its counterpart, heart failure with reduced ejection fraction (HFrEF), HFpEF lacks clear management guidelines. Recognizing this critical gap, we aim to review existing recommendations and formulate effective management strategies for HFpEF.

UNASSIGNED: The impact of recent consensus definitions of cancer therapy-related cardiac dysfunction (CTRCD) from the European Society of Cardiology cardio-oncology guidelines on the reported incidence of CTRCD has not yet been assessed.
UNASSIGNED: The aim of this study was to assess the: 1) cumulative incidence; 2) point prevalence during and after adjuvant therapy; and 3) prognostic value of CTRCD as defined by different asymptomatic CTRCD guideline criteria.
UNASSIGNED: The cumulative incidence and point prevalence of CTRCD were retrospectively assessed in 118 patients participating in the PRADA (Prevention of Cardiac Dysfunction During Adjuvant Breast Cancer Therapy) trial. Asymptomatic CTRCD was assessed using alternative cardiac troponin (cTn) 99th percentile upper reference limits (URLs) to define cTnT and cTnI elevation.
UNASSIGNED: The cumulative incidence of moderate or severe CTRCD was low (1.7%), whereas the cumulative incidence of mild asymptomatic CTRCD was higher and differed markedly according to the biomarker criteria applied, ranging from 49.2% of patients when cTnT greater than the sex-specific 99th percentile URL was used to define cTn elevation to 9.3% when sex-neutral cTnI was used. The point prevalence of CTRCD was highest at the end of anthracycline therapy (47.8%) and was driven primarily by asymptomatic cTn elevation. CTRCD during adjuvant therapy was not prognostic for CTRCD at extended follow-up of 24 months (Q1-Q3: 21-29 months) after randomization.
UNASSIGNED: Mild asymptomatic CTRCD during adjuvant breast cancer therapy was frequent and driven mainly by cTn elevation and was not prognostic of subsequent CTRCD. The incidence of mild, asymptomatic CTRCD differed markedly depending on the cTn assay and whether sex-neutral or sex-dependent URLs were applied. (Prevention of Cardiac Dysfunction During Adjuvant Breast Cancer Therapy [PRADA]; NCT01434134).

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.

UNASSIGNED: Cardiovascular disease continues to be the leading cause of death globally. Clinical practice guidelines aimed at improving disease management and positively impacting major cardiac adverse events recommend genetic testing for inherited cardiovascular conditions such as dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy (HCM), long QT syndrome (LQTS), hereditary amyloidosis, and familial hypercholesterolemia (FH); however, little is known about how consistently practitioners order genetic testing for these conditions in routine clinical practice. This study aimed to assess the adoption of guideline-directed genetic testing for patients diagnosed with DCM, HCM, LQTS, hereditary amyloidosis, or FH.
UNASSIGNED: This retrospective cohort study captured real-world evidence of genetic testing from ICD-9-CM and ICD-10-CM codes, procedure codes, and structured text fields of de-identified patient records in the Veradigm Health Insights Ambulatory EHR Research Database linked with insurance claims data. Data analysis was conducted using an automated electronic health record analysis engine. Patient records in the Veradigm database were sourced from more than 250,000 clinicians serving over 170 million patients in outpatient primary care and specialty practice settings in the United States and linked insurance claims data from public and private insurance providers. The primary outcome measure was evidence of genetic testing within six months of condition diagnosis.
UNASSIGNED: Between January 1, 2017, and December 31, 2021, 224,641 patients were newly diagnosed with DCM, HCM, LQTS, hereditary amyloidosis, or FH and included in this study. Substantial genetic testing care gaps were identified. Only a small percentage of patients newly diagnosed with DCM (827/101,919; 0.8%), HCM (253/15,507; 1.6%), LQTS (650/56,539; 1.2%), hereditary amyloidosis (62/1,026; 6.0%), or FH (718/49,650; 1.5%) received genetic testing.
UNASSIGNED: Genetic testing is underutilized across multiple inherited cardiovascular conditions. This real-world data analysis provides insights into the delivery of genomic healthcare in the United States and suggests genetic testing guidelines are rarely followed in practice.

Arrhythmogenic cardiomyopathy (ACM) is a heart muscle disease characterized by prominent \"non-ischemic\" myocardial scarring predisposing to ventricular electrical instability. Diagnostic criteria for the original phenotype, arrhythmogenic right ventricular cardiomyopathy (ARVC), were first proposed in 1994 and revised in 2010 by an international Task Force (TF). A 2019 International Expert report appraised these previous criteria, finding good accuracy for diagnosis of ARVC but a lack of sensitivity for identification of the expanding phenotypic disease spectrum, which includes left-sided variants, i.e., biventricular (ABVC) and arrhythmogenic left ventricular cardiomyopathy (ALVC). The ARVC phenotype together with these left-sided variants are now more appropriately named ACM. The lack of diagnostic criteria for the left ventricular (LV) phenotype has resulted in clinical under-recognition of ACM patients over the 4 decades since the disease discovery. In 2020, the \"Padua criteria\" were proposed for both right- and left-sided ACM phenotypes. The presently proposed criteria represent a refinement of the 2020 Padua criteria and have been developed by an expert European TF to improve the diagnosis of ACM with upgraded and internationally recognized criteria. The growing recognition of the diagnostic role of CMR has led to the incorporation of myocardial tissue characterization findings for detection of myocardial scar using the late‑gadolinium enhancement (LGE) technique to more fully characterize right, biventricular and left disease variants, whether genetic or acquired (phenocopies), and to exclude other \"non-scarring\" myocardial disease. The \"ring-like\' pattern of myocardial LGE/scar is now a recognized diagnostic hallmark of ALVC. Additional diagnostic criteria regarding LV depolarization and repolarization ECG abnormalities and ventricular arrhythmias of LV origin are also provided. These proposed upgrading of diagnostic criteria represents a working framework to improve management of ACM patients.

Danon disease is a rare X-linked autophagic vacuolar cardioskeletal myopathy associated with severe heart failure that can be accompanied with extracardiac neurologic, skeletal, and ophthalmologic manifestations. It is caused by loss of function variants in the LAMP2 gene and is among the most severe and penetrant of the genetic cardiomyopathies. Most patients with Danon disease will experience symptomatic heart failure. Male individuals generally present earlier than women and die of either heart failure or arrhythmia or receive a heart transplant by the third decade of life. Herein, the authors review the differential diagnosis of Danon disease, diagnostic criteria, natural history, management recommendations, and recent advances in treatment of this increasingly recognized and extremely morbid cardiomyopathy.

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The Korean Society of Heart Failure guidelines aim to provide physicians with evidence-based recommendations for diagnosing and managing patients with heart failure (HF). In Korea, the prevalence of HF has been rapidly increasing in the last 10 years. HF has recently been classified into HF with reduced ejection fraction (HFrEF), HF with mildly reduced ejection fraction (EF), and HF with preserved EF (HFpEF). Moreover, the availability of newer therapeutic agents has led to an increased emphasis on the appropriate diagnosis of HFpEF. Accordingly, this part of the guidelines will mainly cover the definition, epidemiology, and diagnosis of HF.

The Korean Society of Heart Failure guidelines aim to provide physicians with evidence-based recommendations for diagnosing and managing patients with heart failure (HF). In Korea, the prevalence of HF has been rapidly increasing in the last 10 years. HF has recently been classified into HF with reduced ejection fraction (EF), HF with mildly reduced EF, and HF with preserved EF (HFpEF). Moreover, the availability of newer therapeutic agents has led to an increased emphasis on the appropriate diagnosis of HFpEF. Accordingly, this part of the guidelines will mainly cover the definition, epidemiology, and diagnosis of HF.