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cardio.lvnc.chronic.v1PRODUCTION
cardio.lvnc.chronic.v1

LV non-compaction / hypertrabeculation cardiomyopathy (chronic)

cardiologychronicadult
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Encounter flow

12/12 authored

Canonical 12-phase frame with authored status for this dossier.

Current phase

Frame

Detailed

Distinguish isolated hypertrabeculation TRAIT (normal function, no family/symptoms) from LVNC-cardiomyopathy — avoid overdiagnosis

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trait-vs-cardiomyopathy framed

Patient inputs (11)

Pediatric Barth/syndromic vs adult; surveillance cadence

Petersen CMR NC/C >2.3 / Jenni echo >2 — morphologic threshold (trait vs disease)

Systolic function drives whether HFrEF GDMT applies + ICD/anticoagulation

Functional status — GDMT titration + device timing

GDMT + anticoagulant dosing

Fibrosis burden — arrhythmic/SCD risk + phenotype

Barth/neuromuscular association — directed evaluation

Familial sarcomeric / neuromuscular — genetics + cascade

AF → anticoagulation + rate/rhythm

Intertrabecular thrombus → anticoagulation

VT/NSVT/conduction disease → ICD consideration

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Severity triggers (9)

9 need judgement
  • informationalseveresystolic_dysfunction_gdmt
    LVNC with reduced LVEF — full HFrEF 4-pillar GDMT applies (unlike amyloid) — 2022 ACC/AHA HF
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverethromboembolism_branch
    Prior systemic embolism / documented LV thrombus / AF / reduced EF — anticoagulate — 2023 ESC Cardiomyopathy
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverearrhythmic_scd_branch
    Sustained VT/VF, LVEF ≤35 on GDMT, or high-risk arrhythmic genotype/family — ICD evaluation — 2023 ESC Cardiomyopathy
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverepregnancy_special_pop
    Pregnancy with LVNC-cardiomyopathy — peripartum decompensation risk if systolic dysfunction; pregnancy-safe regimen (BB ± hydralazine; stop ACEi/ARNi/SGLT2i); cardio-obstetric — ESC 2018 Pregnancy
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderategenetic_familial_branch
    Familial / sarcomeric (MYH7/TTN/MYBPC3) LVNC — genetic counseling + first-degree cascade screening — 2023 ESC Cardiomyopathy
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderateneuromuscular_syndromic_branch
    Neuromuscular / syndromic association (Barth syndrome in males, mitochondrial, dystrophinopathy) — directed multidisciplinary evaluation — 2023 ESC Cardiomyopathy
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderatepediatric_branch
    Pediatric LVNC (Barth syndrome, congenital, mitochondrial) — pediatric cardiology + genetics — 2023 ESC Cardiomyopathy
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderateckd_special_pop
    CKD — GDMT + anticoagulant dose-gating — KDIGO 2024
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmildisolated_trait_avoid_overtreatment
    Isolated hypertrabeculation, normal LVEF, no arrhythmia/thrombus/family history — reassure; do NOT start lifelong therapy or over-surveil — 2023 ESC Cardiomyopathy
    Trigger could not be auto-evaluated — needs clinician judgement.

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Recommended regimen

LVNC phenotype-driven management (2023 ESC Cardiomyopathy; 2022 AHA/ACC/HFSA HF)
axis: lvnc_phenotype_drivenstep 1 - Step 1 — Isolated hypertrabeculation, normal function, no family/symptoms — REASSURE + light surveillance
Selected step "Step 1 — Isolated hypertrabeculation, normal function, no family/symptoms — REASSURE + light surveillance" — NC/C above threshold but normal LVEF, no arrhythmia, no thrombus, no family history

outpatient playbook — drug actions (2)

  1. 1. HFrEF GDMT if systolic dysfunction
    per HFrEF protocol • PO • per drug
    trigger: LVEF reduced (2022 ACC/AHA HF)
    Standard GDMT applies (unlike amyloid)
  2. 2. anticoagulation by indication
    apixaban 5 mg BID / warfarin if LV thrombus • PO • BID/daily
    trigger: AF / reduced EF / prior embolism / LV thrombus (2023 ESC Cardiomyopathy)
    Not for trabeculation alone

Auto-drafted A&P note

outpatient

Subjective

- Possible entry pathways: Echo/CMR: prominent LV trabeculation, NC/C ratio above threshold; Heart failure symptoms with hypertrabeculated LV; Systemic embolism / stroke with hypertrabeculated LV.

Objective

- No vitals, labs, or imaging entered for this encounter.

Assessment

**LV non-compaction / hypertrabeculation cardiomyopathy (chronic)** (cardio.lvnc.chronic.v1).
Phenotype framing: Isolated hypertrabeculation vs LVNC-DCM/HCM/RCM vs athlete/pregnancy physiologic vs normal
Scope: Distinguish isolated hypertrabeculation TRAIT (normal function, no family/symptoms) from LVNC-cardiomyopathy — avoid overdiagnosis

No severity triggers fired against current inputs.

Plan

Regimen axis: **LVNC phenotype-driven management (2023 ESC Cardiomyopathy; 2022 AHA/ACC/HFSA HF)** — step "Step 1 — Isolated hypertrabeculation, normal function, no family/symptoms — REASSURE + light surveillance".

Setting playbook (outpatient) — Distinguish benign hypertrabeculation from LVNC-cardiomyopathy; treat by functional phenotype; avoid overtreatment (2023 ESC Cardiomyopathy)
1. HFrEF GDMT if systolic dysfunction per HFrEF protocol PO per drug — LVEF reduced (2022 ACC/AHA HF) (Standard GDMT applies (unlike amyloid))
2. anticoagulation by indication apixaban 5 mg BID / warfarin if LV thrombus PO BID/daily — AF / reduced EF / prior embolism / LV thrombus (2023 ESC Cardiomyopathy) (Not for trabeculation alone)

Non-pharmacologic actions:
- Reassurance + light surveillance for benign isolated hypertrabeculation — 2023 ESC Cardiomyopathy
- Inherited-cardiomyopathy centre + genetics if familial/cardiomyopathy — 2023 ESC Cardiomyopathy
- Neuromuscular referral if syndromic features — 2023 ESC Cardiomyopathy

AVOID / contraindication checks:
- Do not treat isolated normal function hypertrabeculation as disease — 2023 ESC Cardiomyopathy
- Standard HFrEF GDMT DOES apply if systolic dysfunction unlike amyloid — 2022 ACC/AHA HF
- Anticoagulate only by indication not for trabeculation alone — 2023 ESC Cardiomyopathy
- Evaluate neuromuscular and syndromic associations Barth in males — 2023 ESC Cardiomyopathy

Monitoring

Regimen monitoring:
- serial echo LVEF for cardiomyopathy phenotype — 2023 ESC Cardiomyopathy
- Holter if arrhythmic features — 2023 ESC Cardiomyopathy
- minimal surveillance for benign isolated trait — 2023 ESC Cardiomyopathy
- family cascade if familial — 2023 ESC Cardiomyopathy
- BMP during GDMT titration — 2022 ACC/AHA HF

Setting (outpatient) monitoring:
- Serial echo/Holter proportionate to phenotype — 2023 ESC Cardiomyopathy

Follow-up plan: Family cascade screening (familial); avoid lifelong over-surveillance of benign hypertrabeculation
- Close-out criterion: follow-up plan documented

Monitoring phase: Serial echo + Holter (cardiomyopathy); minimal surveillance for benign isolated trait

Disposition

Current setting: outpatient — Distinguish benign hypertrabeculation from LVNC-cardiomyopathy; treat by functional phenotype; avoid overtreatment (2023 ESC Cardiomyopathy)

Disposition criteria:
- Benign isolated trait → reassurance + minimal surveillance
- LVNC-cardiomyopathy → phenotype-driven therapy + cascade

Escalation triggers (move to higher acuity):
- New systolic dysfunction → HFrEF pathway — 2022 ACC/AHA HF
- Embolic event / LV thrombus → anticoagulation — 2023 ESC Cardiomyopathy
- Sustained VT / LVEF ≤35 on GDMT → ICD evaluation — 2023 ESC Cardiomyopathy

Earlier-Return Triggers

Return-precaution thresholds (watch for):
- [SEVERE] LVNC with reduced LVEF — full HFrEF 4-pillar GDMT applies (unlike amyloid) — 2022 ACC/AHA HF
- [SEVERE] Prior systemic embolism / documented LV thrombus / AF / reduced EF — anticoagulate — 2023 ESC Cardiomyopathy
- [SEVERE] Sustained VT/VF, LVEF ≤35 on GDMT, or high-risk arrhythmic genotype/family — ICD evaluation — 2023 ESC Cardiomyopathy

Citations

- 2023 ESC Cardiomyopathy Guideline + 2022 AHA/ACC/HFSA HF Guideline; Petersen/Jenni imaging criteria [PMID:37622657](https://pubmed.ncbi.nlm.nih.gov/37622657/)
- Cited evidence (PMID 16271334) [PMID:16271334](https://pubmed.ncbi.nlm.nih.gov/16271334/)
- Cited evidence (PMID 11479219) [PMID:11479219](https://pubmed.ncbi.nlm.nih.gov/11479219/)
- Cited evidence (PMID 35379504) [PMID:35379504](https://pubmed.ncbi.nlm.nih.gov/35379504/)
- Cited evidence (PMID 31535829) [PMID:31535829](https://pubmed.ncbi.nlm.nih.gov/31535829/)

Last reconciled with current guidelines: 2026-05-16.
References