#arrhythmogenic-cardiomyopathy #ARVC #ALVC #diagnostic-criteria #cardiac-magnetic-resonance #late-gadolinium-enhancement

The 2023 European Task Force Criteria for Diagnosis of Arrhythmogenic Cardiomyopathy

Authors, Journal, Affiliations, Type, DOI

Authors: Francesca Graziano, Alessandro Zorzi, Simone Ungaro, Barbara Bauce, Ilaria Rigato, Alberto Cipriani, Martina Perazzolo Marra, Kalliopi Pilichou, Cristina Basso, Domenico Corrado
Journal: Reviews in Cardiovascular Medicine, 2024;25(9):348
Affiliations: Department of Cardiac, Thoracic and Vascular Sciences and Public Health, University of Padova; Department of Sports Medicine, Semmelweis University, Budapest
Type: Review article (several authors were members of the 2023 Task Force)
DOI: https://doi.org/10.31083/j.rcm2509348

Overview

A member-authored review of the 2023 European Task Force criteria, which revised the 2020 Padua criteria after a 2022 Florence consensus conference. The diagnostic process becomes explicitly three-step — ventricular involvement, phenotypic definition, then aetiology and classification — and the six categories are split into separate RV and LV blocks. The most consequential changes are the tightening of LV morpho-functional criteria, the promotion of low limb-lead voltages to major, the demotion of RV LGE to minor with EMB histology becoming major, and the removal of the requirement for a pathogenic variant to diagnose ALVC. The authors propose renaming the disease "scarring/arrhythmogenic cardiomyopathy" to reflect that non-ischaemic scar and arrhythmic risk, not aetiology, define it.

Keywords

Arrhythmogenic cardiomyopathy; ARVC; ALVC; biventricular ACM; European Task Force criteria; Padua criteria; cardiac magnetic resonance; late gadolinium enhancement; NDLVC; diagnosis

Key Takeaways

Three-Step Diagnostic Process

Ventricular involvement — identify ARVC (RV-dominant), biventricular ACM, or ALVC (LV-dominant) from morpho-functional and structural criteria.
Phenotypic definition — count major and minor criteria, but only in the relevant ventricular block(s): RV criteria only for ARVC, LV criteria only for ALVC, both for biventricular disease.
Aetiology and classification — desmosomal ACM, non-desmosomal/genocopy ACM, idiopathic ACM after negative evaluation, or phenocopy (sarcoidosis, myocarditis, amyloidosis, Chagas disease, congenital shunts).

Six Categories (RV and LV Blocks)

Morpho-functional: RV major = regional akinesia/dyskinesia/aneurysm plus global RV dilatation or systolic dysfunction; RV minor = regional wall-motion abnormality alone. LV minor = global LV systolic dysfunction with or without dilatation — the 2020 "regional LV hypokinesia/akinesia" criterion is removed.
Structural: RV major = fibrous replacement on EMB; RV minor = unequivocal LGE in ≥1 RV region (the 2020 transmural LGE major criterion is downgraded). LV major = "ring-like" LGE of ≥3 segments; LV minor = LGE of 1–2 Bull's Eye segments. Patchy/focal LGE is non-diagnostic; septal junctional LGE is excluded.
Repolarisation: RV major = negative T waves V1–V3 or beyond at ≥14 years, without complete RBBB and not preceded by J-point/ST elevation (which marks benign early repolarisation). RV minor = V1–V2 in males ≥14; negative T beyond V3 with complete RBBB; negative T beyond V3 at <14 years. LV minor = negative T in V4–V6 without complete LBBB.
Depolarisation/conduction: RV minor = epsilon wave; terminal activation duration ≥55 ms. LV major = low QRS voltages (<0.5 mV peak-to-peak) in all limb leads, after excluding amyloidosis, obesity, emphysema, pericardial effusion and inappropriate low-pass filtering.
Arrhythmias: RV major = >500 PVCs/24 h or NSVT/SVT with LBBB morphology and non-inferior axis; RV minor = LBBB with inferior axis (RVOT pattern) and cardiac arrest from VF or VT of unknown morphology. LV minor = frequent or exercise-induced PVCs with RBBB morphology or multiple RBBB morphologies (excluding fascicular); NSVT/SVT with RBBB morphology; cardiac arrest of unknown morphology.
Family history/genetics: major = pathogenic ACM variant in the patient; ACM in a first-degree relative meeting criteria; ACM confirmed pathologically in a first-degree relative. Minor = likely pathogenic variant (split from major in 2020); unconfirmed ACM in a first-degree relative; premature SCD <35 from suspected ACM; ACM in a second-degree relative.

Diagnostic Thresholds

Definite: 2 major, or 1 major + 2 minor, or 4 minor.
Borderline: 1 major + 1 minor, or 3 minor.
Possible: 1 major, or 2 minor.
At least one major or minor criterion from category 1 or 2 is mandatory at every level; ACM is a structural disease and cannot be diagnosed on genetics, ECG or arrhythmia alone.

What Changed from the 2020 Padua Criteria

Category	2023 change
LV morpho-functional	Regional LV hypokinesia/akinesia criterion removed
RV tissue	EMB histology becomes major without "limited indication"; transmural RV LGE downgraded major → minor
LV tissue	New major for "ring-like" LGE ≥3 segments; 1–2 segments minor
RV repolarisation	Major redefined at ≥14 years and excludes J-point/ST elevation
LV depolarisation	Low limb-lead voltages upgraded minor → major
RV arrhythmia	Non-inferior axis specified for major; cardiac arrest of unknown morphology added as minor
LV arrhythmia	Exercise-induced PVCs and RBBB VT added as minor; cardiac arrest added
Genetics	Likely pathogenic variant split out as minor

ALVC: The Central Definition Change

2020 Padua: ALVC required a pathogenic ACM variant because LV phenotype alone overlaps with DCM, myocarditis and sarcoidosis.
2023 European TF: ALVC can be diagnosed on clinical criteria regardless of aetiology or genotype, by analogy with DCM and HCM being primary or secondary. This admits post-myocarditis scar and genotype-negative idiopathic cases that Padua excluded.
The authors argue the risk of SCD in ALVC is substantial irrespective of aetiology and even with preserved LVEF, so a diagnosis should not depend on a genetic result.

Nomenclature: "Scarring/Arrhythmogenic Cardiomyopathy"

The 2023 document proposes "scarring/arrhythmogenic cardiomyopathy": "scarring" names the pathobiological hallmark — non-ischaemic fibro-fatty replacement — and "arrhythmogenic" the clinical consequence. The label is intended to apply across aetiologies.
This directly conflicts with the 2023 ESC cardiomyopathy guidelines, which diagnose ACM using the 2010 Task Force criteria and classify LV-dominant disease as DCM or NDLVC by end-diastolic volume. The European TF relies on tissue characterisation; ESC relies on morphological parameters.
The authors reject the ESC combination of "2010 ARVC plus NDLVC" as clinically less useful, because NDLVC mixes patients with and without arrhythmogenic LV scar.

Aetiology and Phenocopies

Desmosomal variants account for ~50% of inherited ACM; non-desmosomal genes (ion channels, cytoskeletal proteins) produce "genocopies", often within inherited neuromuscular disorders.
Phenocopies include cardiac sarcoidosis, post-viral myocarditis, autoimmune cardiomyopathy and congenital anomalies. EMB is particularly important for sarcoidosis, where non-caseating granulomas establish the alternative diagnosis.
Inflammation does not exclude genetic ACM. Myocardial inflammation can accompany desmosomal disease as a "hot phase", and post-myocarditis scar does not rule out a genetic substrate.

Practical Points

Echocardiography must include the infero-basal (sub-tricuspid) RV, often missed on standard views; an off-axis apical 2-chamber view is advised.
Use age-, sex- and body-surface-area-normalised nomograms, including athlete-specific values, for all chamber measurements.
Electroanatomic voltage mapping is not a diagnostic criterion but can improve RV scar sensitivity during an electrophysiology study or ablation.
SAECG is no longer diagnostic but may retain a role in risk stratification by showing late potentials.

Limitations of the document

Member-authored review of criteria its authors wrote, so it is a justification as much as an appraisal; no independent validation data are presented.
The 2023 criteria remain unvalidated in prospective cohorts, and the review gives no sensitivity/specificity or outcome data.
The evidence for several changes is thin — for example, promoting low limb-lead voltages to major rests on a small number of Padua imaging-ECG correlation studies, and removing regional LV hypokinesia reduces sensitivity without published accuracy data.
The "scarring/arrhythmogenic cardiomyopathy" rename is a proposal, not an adopted international classification, and the review does not engage with the ESC 2023 arguments beyond rejecting them.
Diagnosis-only — no risk stratification, ICD thresholds or therapy.
The comparison with ESC 2023 is one-sided, presented by authors who disagree with the ESC classification.

Key Concepts Mentioned

Key Entities Mentioned

Wiki Pages Updated

The ingest entry is in log.md.