LV non-compaction / hypertrabeculation cardiomyopathy (chronic)
Encounter flow
12/12 authoredCanonical 12-phase frame with authored status for this dossier.
Frame
Distinguish isolated hypertrabeculation TRAIT (normal function, no family/symptoms) from LVNC-cardiomyopathy — avoid overdiagnosis
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
* = hard-required. Engine cannot meaningfully run until these are filled.
Severity triggers (9)
- informationalseveresystolic_dysfunction_gdmtLVNC with reduced LVEF — full HFrEF 4-pillar GDMT applies (unlike amyloid) — 2022 ACC/AHA HFTrigger could not be auto-evaluated — needs clinician judgement.
- informationalseverethromboembolism_branchPrior systemic embolism / documented LV thrombus / AF / reduced EF — anticoagulate — 2023 ESC CardiomyopathyTrigger could not be auto-evaluated — needs clinician judgement.
- informationalseverearrhythmic_scd_branchSustained VT/VF, LVEF ≤35 on GDMT, or high-risk arrhythmic genotype/family — ICD evaluation — 2023 ESC CardiomyopathyTrigger could not be auto-evaluated — needs clinician judgement.
- informationalseverepregnancy_special_popPregnancy with LVNC-cardiomyopathy — peripartum decompensation risk if systolic dysfunction; pregnancy-safe regimen (BB ± hydralazine; stop ACEi/ARNi/SGLT2i); cardio-obstetric — ESC 2018 PregnancyTrigger could not be auto-evaluated — needs clinician judgement.
- informationalmoderategenetic_familial_branchFamilial / sarcomeric (MYH7/TTN/MYBPC3) LVNC — genetic counseling + first-degree cascade screening — 2023 ESC CardiomyopathyTrigger could not be auto-evaluated — needs clinician judgement.
- informationalmoderateneuromuscular_syndromic_branchNeuromuscular / syndromic association (Barth syndrome in males, mitochondrial, dystrophinopathy) — directed multidisciplinary evaluation — 2023 ESC CardiomyopathyTrigger could not be auto-evaluated — needs clinician judgement.
- informationalmoderatepediatric_branchPediatric LVNC (Barth syndrome, congenital, mitochondrial) — pediatric cardiology + genetics — 2023 ESC CardiomyopathyTrigger could not be auto-evaluated — needs clinician judgement.
- informationalmoderateckd_special_popCKD — GDMT + anticoagulant dose-gating — KDIGO 2024Trigger could not be auto-evaluated — needs clinician judgement.
- informationalmildisolated_trait_avoid_overtreatmentIsolated hypertrabeculation, normal LVEF, no arrhythmia/thrombus/family history — reassure; do NOT start lifelong therapy or over-surveil — 2023 ESC CardiomyopathyTrigger could not be auto-evaluated — needs clinician judgement.
Workflow calculators
Run this disease's risk and dosing calculators inline.
Recommended regimen
LVNC phenotype-driven management (2023 ESC Cardiomyopathy; 2022 AHA/ACC/HFSA HF)outpatient playbook — drug actions (2)
- 1. HFrEF GDMT if systolic dysfunctionper HFrEF protocol • PO • per drugtrigger: LVEF reduced (2022 ACC/AHA HF)Standard GDMT applies (unlike amyloid)
- 2. anticoagulation by indicationapixaban 5 mg BID / warfarin if LV thrombus • PO • BID/dailytrigger: AF / reduced EF / prior embolism / LV thrombus (2023 ESC Cardiomyopathy)Not for trabeculation alone
Auto-drafted A&P note
outpatientSubjective
- 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.
- 2023 ESC Cardiomyopathy Guideline + 2022 AHA/ACC/HFSA HF Guideline; Petersen/Jenni imaging criteria — PMID:37622657
- Cited evidence (PMID 16271334) — PMID:16271334
- Cited evidence (PMID 11479219) — PMID:11479219
- Cited evidence (PMID 35379504) — PMID:35379504
- Cited evidence (PMID 31535829) — PMID:31535829