Clinical Commander

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cardio.hcm.chronic.v1

Hypertrophic cardiomyopathy (chronic management)

cardiologychronicadultoutpatient
Start encounter →Print handoutPRODUCTION

5-step regimen ladder (asymptomatic → BB/CCB → disopyramide/mavacamten → septal reduction → AF + SCD prevention) authored without RxCUIs (atoms file pending); outpatient + transition setting playbooks; six severity triggers including high SCD risk, mavacamten EF drop, AF in HCM, refractory obstruction, phenocopy screen, pregnancy. Manifest is a Batch 23 scaffold; full atoms / regimen / phenotypes / evidence ids pending. No `_design-brief.md` in src/lib/tier3/problem-package/packages/hypertrophic-cardiomyopathy — author one before INTEGRATED. Registry lacks an HCM-RISK-SCD calculator entry — add to clinical-tools-registry.ts before promoting to PRODUCTION. Mavacamten REMS dosing requires structured echo monitoring; build a treatment_service when authoring atoms.treatment.ts.

Entry points (4)

  • imaging
    LV wall thickness ≥15 mm (or ≥13 mm with FH/genetics) (ACC/AHA 2022)
    lvh_15mm
  • symptom
    Exertional dyspnea / chest pain / syncope (ACC/AHA 2022)
    exertional_dyspnea_or_syncope
  • imaging
    CMR with LGE — fibrosis burden + SCD risk (ACC/AHA 2022)
    cmr_lge
  • history
    Family history of SCD / known sarcomere variant (ACC/AHA 2022)
    family_history_scd

Required inputs (10)

  • agerequired
    demographic • used at CONTEXT
    HCM-RISK-SCD age input + therapy tolerability (ACC/AHA 2022)
  • lv_wall_thicknessrequired
    imaging • used at INITIAL_WORKUP
    Diagnostic threshold + risk stratification (ACC/AHA 2022)
  • lvot_gradientrequired
    imaging • used at DIFFERENTIAL
    Obstructive vs non-obstructive HCM phenotype (ACC/AHA 2022)
  • la_size
    imaging • used at RISK_STRATIFICATION
    HCM-RISK-SCD component (ACC/AHA 2022)
  • cmr_lge
    imaging • used at RISK_STRATIFICATION
    Fibrosis burden / refines SCD risk (ACC/AHA 2022)
  • ecgrequired
    imaging • used at INITIAL_WORKUP
    Q-waves, repolarization abnormalities, NSVT screen (ACC/AHA 2022)
  • family_history_scdrequired
    history • used at CONTEXT
    HCM-RISK-SCD component + cascade screening (ACC/AHA 2022)
  • unexplained_syncope
    history • used at CONTEXT
    HCM-RISK-SCD component (ACC/AHA 2022)
  • nsvt_on_holter
    history • used at RISK_STRATIFICATION
    HCM-RISK-SCD component (ACC/AHA 2022)
  • creatininerequired
    lab • used at TREATMENT
    Drug dosing — disopyramide, mavacamten (ACC/AHA 2022)

12-phase flow (9)

  1. 1FRAME
    Confirm chronic HCM management; rule out phenocopies (Fabry, amyloid, athlete heart, HHD) (ACC/AHA 2022)
    inputs: lv_wall_thickness
    advance: HCM diagnosis confirmed
  2. 2ENTRY
    Symptom / family-history / imaging trigger (ACC/AHA 2022)
    inputs: age
    advance: entry trigger captured
  3. 3CONTEXT
    Family history (sarcomere variants), athletic history, AF history, NYHA class (ACC/AHA 2022)
    inputs: family_history_scd, unexplained_syncope
    advance: context complete
  4. 4INITIAL_WORKUP
    Echo (rest + provocative for LVOT gradient), ECG, Holter, CMR with LGE, genetic testing referral (ACC/AHA 2022)
    inputs: lv_wall_thickness, lvot_gradient, ecg, cmr_lge
    actions: hcm_diagnosis, panel.cardiac
    advance: phenotype + fibrosis burden documented
  5. 5DIFFERENTIAL
    Obstructive (HOCM) vs non-obstructive vs apical; exclude TTR amyloid (PYP), Fabry (α-Gal), Danon, AL amyloid (ACC/AHA 2022)
    inputs: lvot_gradient
    advance: phenotype assigned
  6. 6RISK_STRATIFICATION
    HCM-RISK-SCD calculator; ICD decision per AHA/ESC; AF stroke risk if paroxysmal AF
    inputs: family_history_scd, la_size, cmr_lge, nsvt_on_holter
    advance: SCD risk class + ICD decision documented
  7. 7TREATMENT
    Symptomatic obstructive: BB / non-DHP CCB / disopyramide → mavacamten (EXPLORER-HCM Class I); refractory → septal myectomy or alcohol septal ablation; AF → AC; SCD-high → ICD; lifestyle (avoid dehydration, vasodilators)
    inputs: lvot_gradient, creatinine
    advance: symptoms controlled and SCD prevention plan in place
  8. 8MONITORING
    Annual echo; serial Holter / ILR; mavacamten echo q4w during titration (REMS); cascade family screening q3–5y (ACC/AHA 2022)
    advance: monitoring schedule documented
  9. 9FOLLOWUP
    Sports clearance (shared decision), genetic counseling, lifelong follow-up cadence (ACC/AHA 2022)
    advance: long-term plan in place