Non-Dilated Left Ventricular Cardiomyopathy (NDLVC)

Details

Non-dilated left ventricular cardiomyopathy (NDLVC) is a newly defined phenotypic category introduced in the 2023 ESC Guidelines, replacing "hypokinetic non-dilated cardiomyopathy." It captures a clinically important group of patients with myocardial disease who do not meet classical definitions of DCM, ARVC, or other established cardiomyopathies. NDLVC has a high risk of malignant ventricular arrhythmias and sudden cardiac death, disproportionate to the degree of LV systolic impairment.

Key Facts

Definition and Phenotype

• NDLVC is defined as: non-ischaemic LV scarring or fatty replacement (with or without wall motion abnormalities) OR isolated global LV hypokinesia without scarring. (sources/esc-cmp-2023)
• Replaces terminology including: DCM without LV dilatation, ALVC, left-dominant ARVC, arrhythmogenic DCM. (sources/esc-cmp-2023)
• LV is not dilated by definition; wall thickness is generally normal (not hypertrophied).
• Arrhythmias (ventricular ectopy, conduction disease) and early structural changes on CMR are often the first clinical manifestations. (sources/esc-cmp-2023)

Diagnosis

• CMR with LGE is the key diagnostic modality — it confirms non-ischaemic myocardial fibrosis essential for diagnosis in most cases. (sources/esc-cmp-2023)
• Characteristic LGE distributions by genotype:
  • DSP, FLNC, PLN → ring-like and/or subepicardial LGE pattern
  • LMNA → septal mid-wall LGE
  • TTN, BAG3, DMD, RBM20, myocarditis → more heterogeneous, often less scar, lower LVEF (sources/esc-cmp-2023)
• ECG monitoring is of particular importance; arrhythmia and conduction disease often precede structural changes. (sources/esc-cmp-2023)
• Genetic testing is recommended in all NDLVC patients. (sources/esc-cmp-2023)

Genetics

• Key genes: DSP, FLNC (truncating variants), DES, LMNA, PLN.
• Substantial genetic overlap with DCM and ARVC.
• DSP variants cause a unique form with high prevalence of LV fibrosis and myocardial inflammatory episodes (myocarditis-like). (sources/esc-cmp-2023)
• Penetrance is age-related; precautionary long-term evaluation of first-degree relatives is recommended. (sources/esc-cmp-2023)

Management and SCD Prevention

• Heart failure management: follows 2021 ESC Heart Failure Guidelines.
• SCD risk is the dominant clinical concern in NDLVC.
• High-risk genotypes with substantially elevated arrhythmic risk (regardless of LVEF): PLN, TMEM43, DES, DSP, LMNA, FLNC (truncating), RBM20. (sources/esc-cmp-2023)
• ICD recommendations:
  • Secondary prevention (cardiac arrest, haemodynamically compromised sustained VT): Class I, Level C. (sources/esc-cmp-2023)
  • Primary prevention with HF symptoms + LVEF ≤35% after OMT: Class IIa, Level A. (sources/esc-cmp-2023)
  • High-risk genotype + LVEF >35% with additional risk factors (NSVT, syncope, LGE): Class IIa, Level C. (sources/esc-cmp-2023)
  • High-risk genotype + LVEF >35% without additional risk factors: Class IIb, Level C. (sources/esc-cmp-2023)
  • No high-risk genotype + LVEF >35% with additional risk factors (syncope, LGE): Class IIb, Level C. (sources/esc-cmp-2023)
• Gene-specific risk calculators: LMNA (https://lmna-risk-vta.fr); PLN p.Arg14del (https://plnriskcalculator.shinyapps.io/final_shiny). (sources/esc-cmp-2023)
• Exercise restrictions apply: high-intensity competitive sports should be discouraged in high-risk NDLVC patients. (sources/esc-cmp-2023)

Contradictions / Open Questions

• ESC 2023 NDLVC vs. HRS 2019 ALVC — irreconcilable nomenclature: ESC 2023 introduced NDLVC as a replacement for ALVC, left-dominant ARVC, and arrhythmogenic DCM within their classification. HRS 2019 retains ALVC as a subtype of ACM. Patients who would be labeled ALVC under HRS 2019 criteria may or may not meet NDLVC criteria (which require non-ischaemic LV scar or isolated hypokinesia) — the definitions do not map cleanly onto each other. (sources/esc-cmp-2023, sources/acm-hrs-2019)
• ICD thresholds for LVEF >35% high-risk genotype — ESC 2022 vs. ESC 2023 inconsistency: ESC CMP 2023 recommends ICD for high-risk genotype (PLN, TMEM43, DES, DSP, LMNA, FLNC, RBM20) + LVEF >35% with additional risk factors (Class IIa, Level C). ESC VA 2022 uses a multi-risk-factor LVEF <50% + ≥2 factors framework. These two guidelines use different thresholds for the same patient population, creating internal ESC inconsistency. (sources/esc-cmp-2023, sources/VA-SCD-ESC-2022)
• NDLVC SCD risk out of proportion to LVEF: The hallmark of NDLVC is that arrhythmic risk is disproportionately high relative to LV systolic function — patients with LVEF >50% may still be at high SCD risk if they carry a high-risk genotype (especially PLN or DSP). Standard LVEF-based ICD criteria underestimate risk in this population, but the evidence base for genotype-guided ICD in NDLVC with preserved EF is largely observational. (sources/esc-cmp-2023)

Connections

• Related to entities/DSP
• Related to entities/LMNA
• Related to entities/PLN
• Related to entities/FLNC
• Related to entities/DCM
• Related to entities/ARVC
• Related to entities/ALVC
• Related to concepts/Late-Gadolinium-Enhancement
• Related to concepts/Phenotypic-Approach-to-Cardiomyopathy
• Related to concepts/Arrhythmogenic-Cardiomyopathy
• Related to concepts/Sudden-Cardiac-Death
• Related to concepts/Cascade-Family-Screening

Sources

• sources/VA-SCD-ESC-2022
• sources/acm-hrs-2019
• sources/esc-cmp-2023