Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC)
Details
ARVC is the right-dominant phenotype of concepts/Arrhythmogenic-Cardiomyopathy: progressive fibro-fatty replacement of right ventricular myocardium, with biventricular involvement in most patients and life-threatening ventricular arrhythmias as the early and dominant presentation rather than heart failure. It is primarily a disease of the cardiac desmosome — a "desmosomopathy" — with autosomal dominant inheritance and a prevalence of approximately 0.078%, and it accounts for up to 20% of sudden cardiac deaths in young individuals. (sources/acm-hrs-2019, rating: very high; sources/esc-cmp-2023, rating: very high)
Diagnosis uses the ACM criteria. The 2010 Modified Task Force criteria are the historical standard and are still cited by the 2023 ESC cardiomyopathy guidelines; the 2020 Padua criteria and the 2023 European Task Force criteria are the current specialist framework. At least one morpho-functional or structural criterion is mandatory in every version, because ARVC is a structural heart disease. (sources/acm-padua-criteria-ijc-2020, rating: high; sources/acm-european-tf-criteria-rcm-2024, rating: high)
Key Facts
Epidemiology
- Prevalence ≈0.078% in adults. (sources/esc-cmp-2023, rating: very high)
- Up to 20% of sudden cardiac deaths in young individuals are attributable to ARVC. (sources/acm-hrs-2019, rating: very high)
- LV involvement is common on sensitive imaging: most patients develop LV involvement detectable on CMR even when clinically RV-dominant; 50% of index patients and 17% of family members have an arrhythmic phenotype with LV dysfunction. (sources/acm-hrs-2019)
- Early-onset AF cohort: 2.9% carried a P/LP variant in an ACM/ARVC-associated gene (0.4% restricted to ClinGen strong/definitive genes), making ACM/ARVC the third most common cardiomyopathy syndrome overlapping early-onset AF after DCM (7.2%) and HCM (3.3%). (sources/eoaf-jama-2021, rating: high)
Pathophysiology
- Desmosomopathy: desmosomal protein mutations disrupt intercellular adhesion at the intercalated disc, triggering apoptosis, fibro-fatty replacement and conduction slowing. (sources/acm-hrs-2019, rating: very high)
- PKP2 loss reduces NaV1.5 surface expression and connexin-43 at intercalated discs, contributing to conduction slowing and arrhythmia vulnerability. (sources/gene-therapy-arrhythmia-2025, rating: high)
- Exercise is a disease accelerant — a dose-dependent relationship with structural penetrance, arrhythmic events and progression, particularly in PKP2 carriers. Full detail: concepts/Exercise-Restriction-in-ARVC. (sources/acm-hrs-2019)
- Circadian regulation: PKP2, DSG2 and DSC2 are among the most consistently rhythmically expressed cardiac genes across four independent databases, with PKP2 expression BMAL1-dependent — a possible circadian window of reduced desmosomal integrity, unexplored clinically. (sources/circadian-scd-jmcc-2025, rating: high)
Genetics
- Autosomal dominant inheritance predominates. Desmosomal genes account for most familial cases: PKP2 (most common), DSP, DSG2, DSC2, JUP. Non-desmosomal genes (TMEM43, CDH2, CTNNA3) encode desmosomal binding partners or disrupt desmosomal function indirectly. (sources/acm-hrs-2019, rating: very high)
- Recessive cardiocutaneous forms: Naxos disease (JUP) and Carvajal syndrome (DSP). (sources/acm-hrs-2019)
- Loss-of-function PKP2 is the most prevalent mechanism — compromised intercellular adhesion, fibrosis and impaired connexin-43 expression. (sources/gene-therapy-arrhythmia-2025, rating: high)
- ClinGen validity: definitive for PKP2, DSG2, DSC2, TMEM43 and DSP (for ACM with woolly hair and keratoderma); moderate for PLN and DES; limited for CDH2, CTNNA3, SCN5A, MYBPC3, MYH7 and LMNA. RYR2 is refuted and TTN is disputing; TNNC1, TNNI3, TNNT2 and TPM1 have no known disease relationship and should be removed from ARVC-specific panels. See concepts/ClinGen-Gene-Disease-Validity. (sources/clingen-summary-2026-05-09, rating: high)
- Testing yield 10–50% overall, rising to 63% when the panel includes PKP2, DSP, DSG2, DSC2, JUP, TMEM43 and PLN in patients meeting Task Force criteria. Digenic inheritance and compound heterozygosity occur in up to 20%. (sources/genetic-cmp-jcf-2018, rating: very high)
- Overlap with ALVC and DCM: desmosomal variants are found in patients clinically diagnosed with DCM, and a broader cardiomyopathy panel may identify non-desmosomal variants. Genotype also determines the exercise advice given to carriers and relatives. (sources/genetic-cmp-jcf-2018)
Clinical Presentation
- Consider ARVC in exercise-related palpitations or syncope, cardiac arrest survivors, and individuals with >500 PVCs/24 h or LBBB-morphology VT without other heart disease. (sources/acm-hrs-2019, rating: very high)
- A normal ECG is reported in up to 12%. ECG changes are dynamic and progressive and typically precede structural change. (sources/acm-hrs-2019)
- LV involvement may dominate: palpitations or syncope can reflect LV scar before RV disease is obvious, and the phenotype may be biventricular at presentation. (sources/acm-hrs-2019)
- Family history is often the only clue in relatives, which is why cascade ECG and CMR are performed before symptoms appear. (sources/acm-hrs-2019)
Diagnosis
Diagnostic Criteria
ARVC uses the right-ventricular block of the ACM framework. The Major and Minor columns are the current 2023 European Task Force criteria. The ESC 2023 cardiomyopathy guideline uses the 2010 Task Force criteria instead; the final column gives the 2010 criterion that ESC applies and what changed.
| Category | Major (2023 European TF) | Minor (2023 European TF) | ESC 2023 guideline uses 2010 TFC — and what changed |
|---|---|---|---|
| 1. Morpho-functional | Regional RV akinesia, dyskinesia or aneurysm plus global RV dilatation or systolic dysfunction | Regional RV akinesia, dyskinesia or aneurysm of the free wall alone | ESC 2023 (2010): major/minor graded by severity of RV dilatation/dysfunction. 2023: one major regardless of severity; isolated wall-motion abnormality added as minor |
| 2. Structural | Fibrous replacement on EMB in ≥1 sample, with or without fatty tissue | Unequivocal RV LGE in ≥1 region (2 orthogonal views), excluding the tricuspid valve | ESC 2023 (2010): EMB graded by residual myocytes (major <60%, minor 60–75%); no LGE criterion. 2023: EMB major, RV LGE minor; Padua had made LGE major |
| 3. Repolarisation | Negative T waves V1–V3 or beyond at ≥14 years, no complete RBBB, not preceded by J-point/ST elevation | Negative T V1–V2 in males ≥14; negative T beyond V3 with complete RBBB; negative T beyond V3 at <14 years | ESC 2023 (2010): TWI V1–V3 or beyond after "completed puberty", no complete RBBB, no J-point caveat; minor V1–V2 or V1–V4 with RBBB. 2023: ≥14 years, J-point/ST-elevation exclusion, paediatric and RBBB splits |
| 4. Depolarisation/conduction | — | Epsilon wave; terminal activation duration ≥55 ms in V1–V3 without complete RBBB | ESC 2023 (2010): epsilon wave major; SAECG minor; TAD minor. 2023: epsilon minor; SAECG removed; TAD minor |
| 5. Arrhythmias | >500 PVCs/24 h or NSVT/SVT, LBBB morphology, non-inferior axis | LBBB with inferior axis (RVOT pattern); cardiac arrest from VF/VT of unknown morphology | ESC 2023 (2010): LBBB VT with superior/indeterminate axis major; RVOT/inferior or unknown axis minor. 2023: non-inferior axis major; RVOT minor; unknown-morphology arrest minor |
| 6. Family history/genetics | Pathogenic ACM variant in the patient; affected first-degree relative meeting criteria; pathological confirmation in a first-degree relative | Likely pathogenic variant; unconfirmed affected first-degree relative; premature SCD <35 years from suspected ACM; affected second-degree relative | ESC 2023 (2010): pathogenic and likely pathogenic variants both major. 2023: pathogenic major, likely pathogenic minor |
(sources/acm-european-tf-criteria-rcm-2024, rating: high; sources/acm-padua-criteria-ijc-2020, rating: high; sources/acm-hrs-2019, rating: very high)
Diagnostic thresholds
| Diagnosis | Criteria |
|---|---|
| Definite | 2 major, or 1 major + 2 minor, or 4 minor from different categories |
| Borderline | 1 major + 1 minor, or 3 minor |
| Possible | 1 major, or 2 minor |
- At least one criterion from category 1 or 2 is mandatory at every level — genetics, ECG or arrhythmia alone cannot diagnose ARVC. (sources/acm-padua-criteria-ijc-2020)
- ARVC requires no LV involvement. If LV structural or morpho-functional criteria are also met, the diagnosis is biventricular ACM; isolated LV disease is ALVC. The three-step process — ventricular involvement, phenotype, aetiology — is on concepts/ACM-Diagnostic-Criteria.
- Full 2010 criteria (the version ESC 2023 applies): concepts/ARVC-Task-Force-Criteria.
ECG
- T-wave inversion V1–V3 is the commonest repolarisation abnormality. The 2023 J-point/ST-elevation caveat matters most in athletes, where benign early repolarisation is common and would otherwise mimic the major criterion. (sources/acm-european-tf-criteria-rcm-2024, rating: high)
- Epsilon wave — major under the 2010 criteria, but minor under Padua (2020) and the 2023 European TF criteria because identification varies with filtering, sampling rate and observer. (sources/acm-padua-criteria-ijc-2020; sources/acm-european-tf-criteria-rcm-2024)
- Terminal activation duration ≥55 ms in V1–V3, measured from the S-wave nadir to the end of QRS — minor criterion. (sources/acm-hrs-2019)
- LBBB-morphology VT — major if the axis is non-inferior (superior or indeterminate); minor if inferior (the RVOT pattern). (sources/acm-hrs-2019; sources/acm-european-tf-criteria-rcm-2024)
- Low limb-lead QRS voltages (<0.5 mV) mark LV involvement and are a major criterion under the 2023 European TF criteria, after excluding obesity, emphysema, pericardial effusion and cardiac amyloidosis. (sources/acm-european-tf-criteria-rcm-2024)
Imaging
- CMR is the most sensitive modality and is Class I in all suspected ARVC (ESC 2022). It provides morpho-functional assessment and tissue characterisation in one study. (sources/VA-SCD-ESC-2022, rating: very high)
- RV LGE: major under Padua (2020), minor under the 2023 European TF criteria because the thin RV wall limits spectral resolution and contrast-to-noise ratio. Specificity is high, sensitivity limited. (sources/acm-padua-criteria-ijc-2020; sources/acm-european-tf-criteria-rcm-2024)
- LV LGE: a "ring-like" subepicardial or mid-myocardial stria of ≥3 segments is major; 1–2 segments is minor. Septal junctional LGE and patchy/focal LGE are non-diagnostic. (sources/acm-european-tf-criteria-rcm-2024)
- Use age-, sex- and BSA-normalised nomograms, including athlete-specific values, for all RV and LV measurements — physiological RV dilatation in rowers and canoeists exceeds population limits. (sources/acm-padua-criteria-ijc-2020)
- Echocardiography must include the infero-basal (sub-tricuspid) RV, often missed on standard views; an off-axis apical 2-chamber view is advised. (sources/acm-european-tf-criteria-rcm-2024)
Endomyocardial Biopsy
- Major structural criterion under the 2023 European TF criteria when replacement-type fibrosis, with or without fatty tissue, is found in ≥1 sample. (sources/acm-european-tf-criteria-rcm-2024)
- Reserved for selected cases: sporadic genotype-negative probands in whom the diagnosis depends on excluding a phenocopy, particularly cardiac sarcoidosis or myocarditis. (sources/acm-padua-criteria-ijc-2020)
- Sampling error limits sensitivity; negative biopsy does not exclude ARVC, and septal biopsy is generally unhelpful. (sources/acm-hrs-2019)
Provocation Testing
- Ajmaline induces a type 1 Brugada pattern in 16% of ARVC patients (Peters et al., n=55) — among the highest false-positive rates for sodium-channel-blocker testing in any disease. Exclude ARVC before attributing a positive test to Brugada syndrome. (sources/pharmacological-provocation-europace-2025, rating: high)
- Isoproterenol infusion (45 µg/min × 3 min) has been proposed for early identification: positive = polymorphic PVCs (>3 morphologies) plus couplets, or LBBB-pattern VT atypical for RVOT-VT; 89% of ARVC patients versus 8% of controls had polymorphic VT in the Denis series. Additive value beyond the criteria is uncertain. See concepts/Pharmacological-Provocation-Testing. (sources/pharmacological-provocation-europace-2025)
Risk Stratification
- Major risk factors: NSVT, VT inducibility at EPS, LVEF ≤49%. Minor: male sex, >1000 PVCs/24 h, RV dysfunction meeting Task Force major criteria, proband status, ≥2 desmosomal variants. (sources/acm-hrs-2019, rating: very high)
- ARVC risk calculator (2019, acm-risk.com): adequate in PKP2-ARVC, overpredicts in gene-elusive ARVC and performs poorly in DSP-ARVC; applies only to definite ARVC by the 2010 criteria. (sources/ACM-Genotype-Mx-JCE-2024, rating: high)
- Gene-specific calculators: DSP (Carrick 2024) is usable in genotype-positive individuals irrespective of phenotype; PLN p.Arg14del (Verstraelen 2021) is usable pre-diagnosis. (sources/ACM-Genotype-Mx-JCE-2024)
- All calculators predict VA only, not heart-failure events — an important gap in DSP and PLN, where HF is a cardinal manifestation. (sources/ACM-Genotype-Mx-JCE-2024)
- EPS predictive value is poor (PPV 35%, NPV 70%) despite inducibility counting as a major risk factor; ESC 2022 classifies PES for risk stratification as Class IIb. (sources/acm-hrs-2019; sources/VA-SCD-ESC-2022)
Family Screening
- Baseline evaluation in at-risk relatives yields ~33%, with a further ~33% at 4-year serial follow-up; electrical abnormalities precede structural changes. A new algorithm refines follow-up by additional minor criteria, symptoms and age 20–30 years. (sources/ACM-Genotype-Mx-JCE-2024, rating: high)
- Genotype-negative relatives in a family with a confirmed pathogenic variant may be released from regular screening (COR IIb) but should be educated to return if symptoms develop. (sources/acm-hrs-2019, rating: very high)
- Full pathway: concepts/Cascade-Family-Screening.
Management
Exercise and Sport
- Definite ARVC: competitive or frequent high-intensity endurance exercise is contraindicated — HRS 2019 COR III (Harm), LOE B-NR; ESC 2022 Class I, Level B. Reducing exercise after presentation improves the arrhythmic course. (sources/acm-hrs-2019, rating: very high; sources/VA-SCD-ESC-2022)
- Genotype-positive/phenotype-negative relatives: counsel that high-intensity endurance exercise increases the likelihood of developing ARVC and VA — HRS 2019 COR I, LOE B-NR; ESC 2022 Class IIb. (sources/acm-hrs-2019; sources/VA-SCD-ESC-2022)
- AHA/ACC 2025 separates PKP2 from non-PKP2 ACM: clinical PKP2 — risks likely outweigh benefits; clinical non-PKP2 — competitive sport can be considered after expert shared decision-making; genotype-positive/phenotype-negative (any genotype) — reasonable to consider with 6–12-month imaging surveillance. (sources/competitive-sports-aha-2025, rating: very high)
- Gene-specific evidence: restriction is warranted in PKP2, TMEM43 and gene-elusive ARVC; exercise does not influence penetrance, VA or HF in PLN p.Arg14del; the DSP effect is uncertain. Full detail: concepts/Exercise-Restriction-in-ARVC. (sources/ACM-Genotype-Mx-JCE-2024, rating: high)
Antiarrhythmic Therapy
- Beta-blockers are first-line; ESC 2022 gives Class I in ARVC with NSVT or sustained VA. (sources/VA-SCD-ESC-2022, rating: very high)
- Flecainide should be considered when single-agent therapy fails (ESC 2023). (sources/esc-cmp-2023, rating: very high)
- In PKP2cKO mice, flecainide eliminated VA through combined RyR2 and Na⁺ channel blockade; a human RCT is pending (NCT03685149). (sources/ACM-Genotype-Mx-JCE-2024, rating: high)
ICD
- COR I: cardiac arrest with VT/VF; haemodynamically not tolerated sustained VT. (sources/acm-hrs-2019, rating: very high; sources/VA-SCD-ESC-2022)
- COR IIa: syncope suspected due to VA; haemodynamically tolerated sustained VT; ≥3 major risk factors, or 2 major + 2 minor. (sources/acm-hrs-2019)
- ESC 2022 Class IIa: symptomatic patients with moderate RV/LV dysfunction plus NSVT or inducible SMVT; Class IIa for ICDs with ATP capability for SMVT up to high rates. (sources/VA-SCD-ESC-2022)
- PES for risk stratification in patients with symptoms highly suspicious of VA: ESC 2022 Class IIb. (sources/VA-SCD-ESC-2022)
Catheter Ablation
- Combined endocardial and epicardial approach is required; 60–80% long-term freedom from VT. (sources/acm-hrs-2019, rating: very high)
- Not curative — it does not prevent SCD or reduce mortality. (sources/acm-hrs-2019)
Heart Failure
- RV failure evidence is limited: ACE inhibitors/ARBs, beta-blockers, mineralocorticoid receptor antagonists and diuretics are reasonable (COR IIa, LOE C-EO); isosorbide dinitrate for preload reduction may be considered (COR IIb). (sources/acm-hrs-2019, rating: very high)
Emerging Therapies
Gene Therapy
- AAV9-PKP2 gene replacement in a PKP2-IVS10-1G>C knock-in mouse model: early delivery fully prevented the phenotype; late delivery improved function, reduced fibrosis, eliminated PVCs and achieved 100% survival (Bradford 2023). (sources/gene-therapy-arrhythmia-2025, rating: high)
- PKP2a delivery in PKP2-deficient mice restored intercalated-disc proteins, prevented RV dilation, reduced arrhythmias and improved conduction velocity and survival, including with late-stage therapy (van Opbergen 2024). (sources/gene-therapy-arrhythmia-2025)
- Clinical-stage candidates: LX2020 (Lexeo), RP-A601 (Rocket), TN-401 (Tenaya) — all PKP2-targeted, early-phase. (sources/gene-therapy-arrhythmia-2025)
- Dose ceiling: effective AAV-PKP2 doses are estimated at high 10¹³ to low 10¹⁴ vg/kg — at the threshold of immunogenic and hepatotoxic risk. MyoAAV4A has higher cardiac transduction with lower hepatic uptake, and local delivery may lower required doses. (sources/ACM-Genotype-Mx-JCE-2024, rating: high)
PKP2 and Early-Onset Atrial Fibrillation
- PKP2 truncating variants are enriched in early-onset AF, at the same variant classes considered P/LP for ARVC, and associate with AF after accounting for prevalent cardiomyopathy. (sources/genetic-eoaf-ehj-2024, rating: high)
- Exercise restriction applies when PKP2 is identified through EOAF testing, even before Task Force criteria are met. (sources/genetic-eoaf-ehj-2024)
- Longitudinal ARVC assessment is indicated: CMR and criteria evaluation to detect early features not yet clinically apparent. Related overview: concepts/Early-Onset-Atrial-Fibrillation. (sources/genetic-eoaf-ehj-2024)
Guideline Comparison
| Topic | HRS 2019 | ESC 2022 | ESC 2023 |
|---|---|---|---|
| Nomenclature | Uses "ACM" as umbrella; ARVC is one subtype | ARVC as a distinct entity | Rejects "ACM" as a diagnostic category; LV-dominant disease → entities/NDLVC |
| Diagnostic criteria | 2010 Task Force Criteria | 2010 Task Force Criteria | 2010 criteria; Padua acknowledged but not adopted |
| CMR in suspected ARVC | — | Class I | — |
| Genetic testing in suspected/definite ARVC | — | Class I | — |
| Beta-blockers with NSVT/sustained VA | — | Class I | — |
| Exercise restriction, definite ARVC | COR III (Harm), LOE B-NR | Class I, Level B | Restriction maintained |
| Exercise restriction, genotype+/phenotype− | COR I, LOE B-NR | Class IIb | — |
| Syncope suspected due to VA → ICD | COR IIa | — | — |
| Epsilon wave reliability | Major criterion (2010) | — | Caution: poor sensitivity/specificity, high interobserver variability |
The 2023 European Task Force criteria are a fourth framework, not a guideline, and are not represented in this table. Under them the epsilon wave is minor, RV LGE is minor, EMB histology is major, and the major arrhythmia criterion requires a non-inferior axis. concepts/ACM-Diagnostic-Criteria compares all four versions. (sources/acm-european-tf-criteria-rcm-2024, rating: high)
Contradictions / Open Questions
- Nomenclature remains unresolved. HRS 2019 uses ACM as an umbrella, Padua and the 2023 European TF retain a narrower scar-defined ACM that includes ARVC, and ESC 2023 rejects ACM altogether. The class-level argument is on concepts/Arrhythmogenic-Cardiomyopathy; for ARVC the practical consequence is that a biventricular patient may be labelled ARVC, biventricular ACM or NDLVC depending on the document. (sources/acm-hrs-2019; sources/esc-cmp-2023, rating: very high)
- Exercise restriction data remain PKP2-centric. The dose-dependent exercise–penetrance relationship is best established for PKP2 carriers. Data for DSP, LMNA, FLNC and other NDLVC/ALVC genes are insufficient, and the effect appears absent in PLN p.Arg14del — so recommendations for non-PKP2 genotypes rest on extrapolation. (sources/acm-hrs-2019; sources/ACM-Genotype-Mx-JCE-2024)
- Epsilon wave — the criteria did update, but not everywhere. The 2010 criteria made it major; Padua and the 2023 European TF criteria downgraded it to minor because identification varies with filtering, sampling rate and observer. ESC 2023 still cites the 2010 criteria, so one centre can count epsilon as major while another cannot — the same ECG finding is worth different points depending on the document. (sources/acm-padua-criteria-ijc-2020; sources/acm-european-tf-criteria-rcm-2024; sources/esc-cmp-2023)
- 2010 versus Padua versus 2023: no version is validated. The 2010 criteria are RV-only and contain no LV criteria. Padua and the 2023 European TF criteria add LV criteria but neither has been prospectively validated, and Padua was derived and tested in the same Padua cohort. ESC 2023 still recommends the 2010 criteria, so the specialist and general-guideline standards diverge. (sources/acm-padua-criteria-ijc-2020; sources/acm-evolving-criteria-jaha-2021, rating: high; sources/acm-european-tf-criteria-rcm-2024)
- RV LGE changed from major to minor in three years. Padua made transmural RV LGE major; the 2023 European TF criteria demoted it to minor and promoted EMB histology to major, citing the technical limitations of imaging the thin RV wall. The same finding carries different diagnostic weight depending on the version applied. (sources/acm-padua-criteria-ijc-2020; sources/acm-european-tf-criteria-rcm-2024)
- ClinGen refutes sarcomeric genes in ARVC. TNNC1, TNNI3, TNNT2 and TPM1 — all established HCM genes — have "no known disease relationship" with ARVC by ClinGen expert curation. RYR2 has refuting evidence and TTN is disputing. Positive sarcomeric results should not be attributed to ARVC. (sources/clingen-summary-2026-05-09, rating: high)
- EPS utility is internally inconsistent. HRS 2019 counts VT inducibility at EPS as a major ICD risk factor, yet the same document reports PPV 35% and NPV 70%; ESC 2022 downgrades PES to Class IIb for risk stratification. (sources/acm-hrs-2019; sources/VA-SCD-ESC-2022)
- Risk calculators are genotype-specific but applied genotype-blind. The 2019 ARVC calculator is adequate in PKP2-ARVC, overpredicts in gene-elusive disease and performs poorly in DSP-ARVC, while all calculators predict only VA and ignore heart-failure events. (sources/ACM-Genotype-Mx-JCE-2024)
- Flecainide status differs between sources. ESC 2023 recommends flecainide when single-agent therapy fails, whereas the 2025 genotype-based review states that only beta-blockers are currently recommended in ARVC, with flecainide efficacy shown only in the PKP2cKO mouse and a human RCT pending. (sources/esc-cmp-2023; sources/ACM-Genotype-Mx-JCE-2024)
Connections
- Phenotype and classification: concepts/Arrhythmogenic-Cardiomyopathy · entities/ALVC · entities/NDLVC · concepts/Phenotypic-Approach-to-Cardiomyopathy
- Diagnostic criteria: concepts/ACM-Diagnostic-Criteria · concepts/ARVC-Task-Force-Criteria · concepts/Late-Gadolinium-Enhancement · concepts/Pharmacological-Provocation-Testing
- Genetics and mechanism: entities/PKP2 · entities/DSP · entities/SCN5A · concepts/Desmosome · concepts/Final-Common-Pathway · concepts/ClinGen-Gene-Disease-Validity · concepts/Genetic-Testing-in-Cardiomyopathy · concepts/Cascade-Family-Screening
- Arrhythmia and outcomes: concepts/Sudden-Cardiac-Death · concepts/Electrical-Storm · concepts/Circadian-Rhythm-Cardiac-Electrophysiology · concepts/Early-Onset-Atrial-Fibrillation
- Therapy and lifestyle: concepts/Exercise-Restriction-in-ARVC · concepts/Sports-Cardiology-SDM · concepts/AAV-Gene-Delivery · concepts/Gene-Silencing-Therapy · concepts/SupRep-Therapy
Sources
- sources/ACM-Genotype-Mx-JCE-2024
- sources/VA-SCD-ESC-2022
- sources/acm-hrs-2019
- sources/acm-padua-criteria-ijc-2020
- sources/acm-evolving-criteria-jaha-2021
- sources/acm-european-tf-criteria-rcm-2024
- sources/circadian-scd-jmcc-2025
- sources/clingen-summary-2026-05-09
- sources/competitive-sports-aha-2025
- sources/eoaf-jama-2021
- sources/esc-cmp-2023
- sources/gene-therapy-arrhythmia-2025
- sources/genetic-cmp-jcf-2018
- sources/genetic-eoaf-ehj-2024
- sources/pharmacological-provocation-europace-2025