Phänotypische Klassifizierung der Kardiomyopathien entsprechend den Leitlinien der ESC 2023

Zusammenfassung

Kardiomyopathien sind Herzmuskelerkrankungen mit struktureller und funktioneller Myokardstörung. Die ESC hat 2023 erstmals eine internationale Leitlinie zum gesamten Spektrum der Kardiomyopathien herausgebracht. Ein zentrales Element der Leitlinie ist die phänotypische Klassifizierung der Kardiomyopathien. Hierbei spielt neben morphologischen und funktionellen Merkmalen als wesentliche Neuerung auch die genaue Gewebecharakterisierung (z. B. Narbenbildung) basierend auf einer kardialen Magnetresonanztomografie (CMR) eine wesentliche Rolle. Auf dieser Grundlage erfolgt die phänotypische Einteilung in 5 Hauptformen. Zusätzlich zur dilatativen, hypertrophen und restriktiven Kardiomyopathie (DCM, HCM und RCM) wurde die nicht dilatierte linksventrikuläre Kardiomyopathie (NDLVC) neu definiert. Ferner wurde die Gruppe der arrhythmogenen rechtsventrikulären Kardiomyopathie (ARVC) aktualisiert. Die hiermit definierten Kardiomyopathie-Phänotypen sollen die Ermittlung der genauen Ätiologie vereinfachen.

Abstract

Cardiomyopathies are defined as myocardial disorders with structural and functional abnormalities of the myocardium. In 2023, the ESC published an international guideline on the entire field of cardiomyopathies for the first time. An essential element of the guideline is the phenotypic classification of cardiomyopathies. In addition to morphological and functional traits, the major innovation is the inclusion of myocardial tissue characterization traits (e.g. scar assessment) using cardiac magnetic resonance (CMR) imaging. Based on this, the phenotypic classification is made into five groups. In addition to dilated, hypertrophic and restrictive cardiomyopathy (DCM, HCM and RCM), a non-dilated left ventricular cardiomyopathy (NDLVC) was newly defined. Furthermore, the group of arrhythmogenic right ventricular cardiomyopathy (ARVC) was updated. The cardiomyopathy phenotypes defined here are a crucial first step in the diagnostic pathway and determination of the underlying aetiology.

Schlüsselwörter

Kardiomyopathien - phänotypische Klassifizierung - arrhythmogene Kardiomyopathie (ACM) - nicht dilatierte linksventrikuläre Kardiomyopathie (NDLVC)

Keywords

cardiomyopathies - phenotypic classification - arrhythmogenic cardiomyopathy (ACM) - non-dilated left ventricular cardiomyopathy (NDLVC)

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