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NDLVC 2023 Classification Application — EECC MCQ

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ModerateCardiomyopathyNDLVC 2023 Classification ApplicationEECC

A 55-year-old man presents with HF. Echocardiography shows non-dilated LV with LVEF 42%, global hypokinesis, and GLS -10% (severely impaired). CMR shows diffuse subepicardial LGE and mid-wall fibrosis but no LV dilatation. The 2023 ESC classification would categorise this as which phenotype?

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Correct answer: A — Non-dilated left ventricular cardiomyopathy (NDLVC) — a new ESC classification category for patients with LV systolic dysfunction and/or non-ischaemic scar WITHOUT LV dilatation; it encompasses some patients previously labelled as early DCM or arrhythmogenic LV cardiomyopathy

NDLVC is a NEW phenotypic category introduced by the 2023 ESC Cardiomyopathy Guidelines for patients who have: (1) LV systolic dysfunction (LVEF <50% or GLS impairment) WITHOUT LV dilatation (LVEDV not meeting DCM criteria); AND/OR (2) non-ischaemic LV scar (LGE in a non-coronary pattern) WITHOUT LV dilatation. This category addresses a gap in previous classifications where patients with: (a) early/pre-dilated cardiomyopathy; (b) arrhythmogenic LV cardiomyopathy (ALVC — left-dominant ARVC variant); (c) myocarditis transitioning to chronic cardiomyopathy; did not fit neatly into DCM (no dilatation), HCM (no hypertrophy), or ARVC (no RV involvement). NDLVC has significant arrhythmic risk (particularly with desmosomal mutations like DSP, FLNC, PLN), and ICD decisions should consider genotype alongside LVEF. Genetic testing is particularly important in NDLVC to identify high-risk arrhythmogenic genotypes.

Reference: ESC (2023): Cardiomyopathies Guidelines