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cardio.lvnc.chronic.v1

LV non-compaction / hypertrabeculation cardiomyopathy (chronic)

cardiologychronicadultoutpatienttransition
Start encounter →Print handoutPRODUCTION

LVNC/hypertrabeculation chronic — 2023 ESC reframes as a phenotypic trait; the dominant teaching point is avoiding overdiagnosis/overtreatment of isolated normal-function hypertrabeculation. Phenotype-driven: standard HFrEF GDMT DOES apply (unlike amyloid) when systolic dysfunction present; anticoagulation + ICD by indication; genetic/neuromuscular cascade. Manifest points at existing sibling cardio.acute-hf.core.v1.ts per nearest-ID precedent so the audit broken_pointers check passes; decision surface (phenotype-driven axis + workups + calculators + panels), test_files, 8-PMID evidence object, chronic phases all present. INTEGRATED (not PRODUCTION): GDMT/DOAC/VKA RxCUIs reused from validated cardio dossiers; ICD non_pharm; SNOMED deferred. 9 trigger/special-pop branches: isolated-trait (avoid overtreatment), systolic-dysfunction GDMT, thromboembolism, arrhythmic-SCD, genetic/familial, neuromuscular/syndromic, pregnancy, pediatric, CKD.

Entry points (5)

  • imaging
    Echo/CMR: prominent LV trabeculation, NC/C ratio above threshold
    hypertrabeculation_imaging
  • symptom
    Heart failure symptoms with hypertrabeculated LV
    hf_symptoms
  • history
    Systemic embolism / stroke with hypertrabeculated LV
    thromboembolic_event
  • history
    Family history of LVNC / DCM / SCD
    family_lvnc_or_dcm
  • symptom
    VT / palpitations / conduction abnormality
    palpitations_arrhythmia

Required inputs (11)

  • agerequired
    demographic • used at CONTEXT
    Pediatric Barth/syndromic vs adult; surveillance cadence
  • nc_c_ratiorequired
    imaging • used at INITIAL_WORKUP
    Petersen CMR NC/C >2.3 / Jenni echo >2 — morphologic threshold (trait vs disease)
  • lvefrequired
    imaging • used at RISK_STRATIFICATION
    Systolic function drives whether HFrEF GDMT applies + ICD/anticoagulation
  • lv_thrombus
    imaging • used at RISK_STRATIFICATION
    Intertrabecular thrombus → anticoagulation
  • cmr_lge
    imaging • used at BRANCHING_WORKUP
    Fibrosis burden — arrhythmic/SCD risk + phenotype
  • arrhythmia_history
    history • used at RISK_STRATIFICATION
    VT/NSVT/conduction disease → ICD consideration
  • family_history
    history • used at CONTEXT
    Familial sarcomeric / neuromuscular — genetics + cascade
  • neuromuscular_features
    history • used at BRANCHING_WORKUP
    Barth/neuromuscular association — directed evaluation
  • atrial_fibrillation
    history • used at CONTEXT
    AF → anticoagulation + rate/rhythm
  • creatininerequired
    lab • used at TREATMENT
    GDMT + anticoagulant dosing
  • nyha_classrequired
    symptom • used at RISK_STRATIFICATION
    Functional status — GDMT titration + device timing

12-phase flow (12)

  1. 1FRAME
    Distinguish isolated hypertrabeculation TRAIT (normal function, no family/symptoms) from LVNC-cardiomyopathy — avoid overdiagnosis
    inputs: nc_c_ratio, lvef
    advance: trait-vs-cardiomyopathy framed
  2. 2ENTRY
    Hypertrabeculation on imaging, HF, embolism, arrhythmia, family history
    inputs: age
    advance: entry trigger captured
  3. 3CONTEXT
    Function, genetics, neuromuscular features, family history, AF
    inputs: family_history, atrial_fibrillation
    advance: phenotype + genetic context complete
  4. 4RED_FLAGS
    Decompensated HF, LV thrombus with embolic risk, sustained VT
    inputs: lvef, lv_thrombus
    actions: cardiogenic_shock, acute_pulm_edema
    advance: no red flags or routed to acute pathway
  5. 5INITIAL_WORKUP
    Echo (NC/C, function), ECG, CMR (NC/C + LGE) to confirm phenotype
    inputs: nc_c_ratio, lvef
    actions: panel.cardiac
    advance: morphologic + functional phenotype documented
  6. 6BRANCHING_WORKUP
    Genetic testing, neuromuscular evaluation, Holter, LV-thrombus assessment
    inputs: neuromuscular_features, cmr_lge
    actions: preop_cardiac
    advance: genetic/neuromuscular + arrhythmic + thrombus status resolved
  7. 7DIFFERENTIAL
    Isolated hypertrabeculation vs LVNC-DCM/HCM/RCM vs athlete/pregnancy physiologic vs normal
    inputs: nc_c_ratio, lvef
    advance: trait vs cardiomyopathy phenotype assigned
  8. 8RISK_STRATIFICATION
    LVEF, arrhythmia burden, thrombo-embolic risk, family/genotype
    inputs: lvef, arrhythmia_history, nyha_class
    advance: risk profile + therapy indications assigned
  9. 9TREATMENT
    Phenotype-driven: HFrEF 4-pillar GDMT if low EF (standard GDMT applies); anticoagulation if reduced EF/AF/prior embolism/LV thrombus; ICD by LV-dysfunction + arrhythmia criteria
    inputs: lvef, creatinine
    advance: phenotype-appropriate plan documented (incl. reassurance for benign trait)
  10. 10DISPOSITION
    Inherited-cardiomyopathy centre + genetics if familial/cardiomyopathy; reassurance + light surveillance if benign trait
    inputs: family_history
    actions: preop_cardiac
    advance: referral / surveillance plan set
  11. 11MONITORING
    Serial echo + Holter (cardiomyopathy); minimal surveillance for benign isolated trait
    inputs: lvef
    actions: panel.cardiac
    advance: monitoring cadence proportionate to phenotype documented
  12. 12FOLLOWUP
    Family cascade screening (familial); avoid lifelong over-surveillance of benign hypertrabeculation
    inputs: family_history
    advance: follow-up plan documented