The new European Society of Cardiology guideline for the management of cardiomyopathies: key messages for cardiac electrophysiologists

Abstract

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.

Cardiac Sarcoidosis—Diagnostic and Therapeutic Challenges

“…Scarring and inflammation promote the formation of reentrant VA. Because inflammation is often multifocal, multiple VA are common [31,32]. Involvement of the right ventricle (Figure 1) seems to indicate an increased risk for VA and may present as a phenocopy of ARVC [13,[33][34][35]. As the conduction system is often affected, AV conduction block is a "red flag", especially in younger patients without known structural heart disease [36].…”

“…These conditions include myocarditis, various conduction system diseases, genetic cardiomyopathies, and ischemic heart disease. Lyme carditis [85], giant cell myocarditis, and genetic cardiomyopathies such as laminopathies may all present with conduction system disease [21,33,86]. Notably, giant cell myocarditis (GCM) is a rare, but important phenocopy.…”

Cardiogenetics in Germany— a view and review

“…Weitere, familiäre Beschreibungen von Kardiomyopathien folgten [ 32 , 80 ] und unterstützten dabei die Entwicklung einer Klassifikation von Kardiomyopathien, erstmals durch die International Society and Federation of Cardiology (ISFC; [ 5 ]) und aktuell durch die ESC [ 84 ], wobei es zunehmend nicht nur um die Differenzierung der Polyätiologie einzelner Kardiomyopathien geht, sondern auch um eine effiziente Diagnostik, Therapie und Risikostratifizierung, unter Einschluss kardialer Bildgebungsparameter und ursächlicher, genetischer Faktoren, wie z. B. in den „Risiko-DCM-Genen“ LMNA, PLN, FLNC und RMB20 [ 42 ]. Die ursprünglichen Kardiomyopathien haben somit ihre idiopathische Genese [ 21 , 64 ] weitgehend verlassen, obgleich die Mutationsdetektionsrate bei den einzelnen Kardiomyopathie-Formen lediglich zwischen 20 und 60 % liegt.…”