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cardio.hcm.chronic.v1PRODUCTION
cardio.hcm.chronic.v1

Hypertrophic cardiomyopathy (chronic management)

cardiologychronicadult
Hard-required inputs
0 / 6
Care setting:

Encounter flow

9/12 authored

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

Current phase

Frame

Detailed

Confirm chronic HCM management; rule out phenocopies (Fabry, amyloid, athlete heart, HHD) (ACC/AHA 2022)

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Actions
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Advance rule
Set
Advance when

HCM diagnosis confirmed

Patient inputs (10)

HCM-RISK-SCD age input + therapy tolerability (ACC/AHA 2022)

HCM-RISK-SCD component + cascade screening (ACC/AHA 2022)

Obstructive vs non-obstructive HCM phenotype (ACC/AHA 2022)

Diagnostic threshold + risk stratification (ACC/AHA 2022)

Q-waves, repolarization abnormalities, NSVT screen (ACC/AHA 2022)

Drug dosing — disopyramide, mavacamten (ACC/AHA 2022)

HCM-RISK-SCD component (ACC/AHA 2022)

HCM-RISK-SCD component (ACC/AHA 2022)

Fibrosis burden / refines SCD risk (ACC/AHA 2022)

HCM-RISK-SCD component (ACC/AHA 2022)

* = hard-required. Engine cannot meaningfully run until these are filled.

Severity triggers (6)

6 need judgement
  • informationalseverehigh_scd_risk
    HCM-RISK-SCD ≥6% / family Hx SCD / unexplained syncope / NSVT / LV thickness ≥30 / extensive LGE (ACC/AHA 2022)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereaf_in_hcm
    Any documented AF in HCM patient regardless of CHA₂DS₂-VA (ACC/AHA 2022)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevererefractory_obstruction
    NYHA III–IV + LVOT gradient ≥50 despite mavacamten + BB/CCB + disopyramide (ACC/AHA 2022)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverepregnancy_with_obstructive_hcm
    Pregnant patient with obstructive HCM (ACC/AHA 2022)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderatemavacamten_ef_drop
    LVEF <50% on follow-up echo while on mavacamten (ACC/AHA 2022)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderatephenocopy_screen
    Atypical features — extracardiac findings, low voltage with thick walls, conduction disease (ACC/AHA 2022)
    Trigger could not be auto-evaluated — needs clinician judgement.

Workflow calculators

Run this disease's risk and dosing calculators inline.

RISK_STRATIFICATIONoptionalDrives risk stratification
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Recommended regimen

HCM — symptom + obstruction-driven stepwise (2024 AHA/ACC + 2023 ESC)
axis: hcm_obstructive_stepwisestep 1 - Step 1 — Non-obstructive HCM or asymptomatic obstructive
Selected step "Step 1 — Non-obstructive HCM or asymptomatic obstructive" — No or minimal symptoms; LVOT gradient at rest <30 (provoked may be elevated)

outpatient playbook — drug actions (5)

  1. 1. beta-blocker
    Metoprolol succinate 25–200 mg daily OR bisoprolol 2.5–10 mg • PO • daily
    trigger: Symptomatic obstructive HCM (ACC/AHA 2022)
    First-line negative inotrope (ACC/AHA 2022)
  2. 2. non-DHP CCB if BB-intolerant
    Verapamil 120–480 mg ER OR diltiazem 120–360 mg ER • PO • daily
    trigger: BB intolerance (ACC/AHA 2022)
    Alternative (ACC/AHA 2022)
  3. 3. disopyramide add-on
    100–150 mg q6h • PO • q6h
    trigger: Persistent symptoms despite BB/CCB (ACC/AHA 2022)
    Class IA negative inotrope
  4. 4. mavacamten
    5 mg PO daily, titrate q4 weeks per echo • PO • daily
    trigger: Symptomatic obstructive NYHA II–III; REMS enrolled (ACC/AHA 2022)
    EXPLORER-HCM Class I
  5. 5. AC for any AF
    Apixaban 5 mg BID • PO • BID
    trigger: Any documented AF (ACC/AHA 2022)
    Class I — independent of CHA₂DS₂-VA in HCM

Auto-drafted A&P note

outpatient

Subjective

- Possible entry pathways: LV wall thickness ≥15 mm (or ≥13 mm with FH/genetics) (ACC/AHA 2022); Exertional dyspnea / chest pain / syncope (ACC/AHA 2022); CMR with LGE — fibrosis burden + SCD risk (ACC/AHA 2022).

Objective

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

Assessment

**Hypertrophic cardiomyopathy (chronic management)** (cardio.hcm.chronic.v1).
Phenotype framing: Obstructive (HOCM) vs non-obstructive vs apical; exclude TTR amyloid (PYP), Fabry (α-Gal), Danon, AL amyloid (ACC/AHA 2022)
Scope: Confirm chronic HCM management; rule out phenocopies (Fabry, amyloid, athlete heart, HHD) (ACC/AHA 2022)

No severity triggers fired against current inputs.

Plan

Regimen axis: **HCM — symptom + obstruction-driven stepwise (2024 AHA/ACC + 2023 ESC)** — step "Step 1 — Non-obstructive HCM or asymptomatic obstructive".

Setting playbook (outpatient) — Control symptoms via stepwise negative inotrope, prevent SCD via risk-stratified ICD, screen family, advise on lifestyle, avoid worsening obstruction (ACC/AHA 2022)
1. beta-blocker Metoprolol succinate 25–200 mg daily OR bisoprolol 2.5–10 mg PO daily — Symptomatic obstructive HCM (ACC/AHA 2022) (First-line negative inotrope (ACC/AHA 2022))
2. non-DHP CCB if BB-intolerant Verapamil 120–480 mg ER OR diltiazem 120–360 mg ER PO daily — BB intolerance (ACC/AHA 2022) (Alternative (ACC/AHA 2022))
3. disopyramide add-on 100–150 mg q6h PO q6h — Persistent symptoms despite BB/CCB (ACC/AHA 2022) (Class IA negative inotrope)
4. mavacamten 5 mg PO daily, titrate q4 weeks per echo PO daily — Symptomatic obstructive NYHA II–III; REMS enrolled (ACC/AHA 2022) (EXPLORER-HCM Class I)
5. AC for any AF Apixaban 5 mg BID PO BID — Any documented AF (ACC/AHA 2022) (Class I — independent of CHA₂DS₂-VA in HCM)

Non-pharmacologic actions:
- Avoid dehydration + vasodilators (worsens obstruction) (ACC/AHA 2022)
- Cautious or no competitive sports (shared decision per 2024 guideline)
- Genetic counseling + cascade family screening (first-degree relatives every 3–5 years) (ACC/AHA 2022)
- ICD for high SCD risk (HCM-RISK-SCD ≥6%, family Hx SCD, syncope, NSVT, massive LVH ≥30 mm, extensive LGE) (ACC/AHA 2022)
- Vaccinations (ACC/AHA 2022)

AVOID / contraindication checks:
- Positive_inotrope_AVOID_in_obstructive_HCM (ACC/AHA 2022)
- Nitrates_AVOID_in_obstructive_HCM (ACC/AHA 2022)
- Diuretics_caution_can_worsen_obstruction (ACC/AHA 2022)
- ACEi_ARB_caution_can_worsen_obstruction (ACC/AHA 2022)
- DHP_CCB_AVOID_in_obstructive_HCM (ACC/AHA 2022)
- Mavacamten_REMS_enrolled_echo_monitoring (ACC/AHA 2022)

Monitoring

Regimen monitoring:
- echo q1 year baseline (ACC/AHA 2022)
- mavacamten echo q4 weeks during titration then q12 weeks (ACC/AHA 2022)
- CYP2C19 genotype for mavacamten (ACC/AHA 2022)
- LVEF threshold 50 for mavacamten dose adjust (ACC/AHA 2022)
- Holter q1-2 years for NSVT screen (ACC/AHA 2022)
- cascade family screening q3-5 years (ACC/AHA 2022)

Setting (outpatient) monitoring:
- Echo annually (ACC/AHA 2022)
- Holter q1–2 years (ACC/AHA 2022)
- Repeat HCM-RISK-SCD (ACC/AHA 2022)
- Mavacamten echo monitoring per REMS (ACC/AHA 2022)

Follow-up plan: Sports clearance (shared decision), genetic counseling, lifelong follow-up cadence (ACC/AHA 2022)
- Close-out criterion: long-term plan in place

Monitoring phase: Annual echo; serial Holter / ILR; mavacamten echo q4w during titration (REMS); cascade family screening q3–5y (ACC/AHA 2022)

Disposition

Current setting: outpatient — Control symptoms via stepwise negative inotrope, prevent SCD via risk-stratified ICD, screen family, advise on lifestyle, avoid worsening obstruction (ACC/AHA 2022)

Disposition criteria:
- Stable NYHA I-II → q6–12 month visits (ACC/AHA 2022)
- Symptomatic uncontrolled → q3 month titration (ACC/AHA 2022)
- Refractory → septal reduction referral (ACC/AHA 2022)

Escalation triggers (move to higher acuity):
- NSVT on Holter or syncope → ICD evaluation (ACC/AHA 2022)
- NYHA III–IV refractory → septal reduction (myectomy or alcohol ablation) (ACC/AHA 2022)
- AF → AC + rate/rhythm management (ACC/AHA 2022)
- End-stage non-obstructive HCM with HFrEF → advanced HF center / transplant (ACC/AHA 2022)

Earlier-Return Triggers

Return-precaution thresholds (watch for):
- [SEVERE] HCM-RISK-SCD ≥6% / family Hx SCD / unexplained syncope / NSVT / LV thickness ≥30 / extensive LGE (ACC/AHA 2022)
- [SEVERE] Any documented AF in HCM patient regardless of CHA₂DS₂-VA (ACC/AHA 2022)
- [SEVERE] NYHA III–IV + LVOT gradient ≥50 despite mavacamten + BB/CCB + disopyramide (ACC/AHA 2022)

Citations

- 2024 AHA/ACC/AMSSM/HRS/PACES/SCMR HCM Guideline + 2023 ESC Cardiomyopathies Guideline + EXPLORER-HCM mavacamten trial [PMID:38718139](https://pubmed.ncbi.nlm.nih.gov/38718139/)
- Cited evidence (PMID 37622657) [PMID:37622657](https://pubmed.ncbi.nlm.nih.gov/37622657/)
- Cited evidence (PMID 32871100) [PMID:32871100](https://pubmed.ncbi.nlm.nih.gov/32871100/)

Last reconciled with current guidelines: 2026-05-22.
References
  • 2024 AHA/ACC/AMSSM/HRS/PACES/SCMR HCM Guideline + 2023 ESC Cardiomyopathies Guideline + EXPLORER-HCM mavacamten trial — PMID:38718139
  • Cited evidence (PMID 37622657) — PMID:37622657
  • Cited evidence (PMID 32871100) — PMID:32871100