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cardio.acute-hf.hcm-decompensation.v1PRODUCTION
cardio.acute-hf.hcm-decompensation.v1

Hypertrophic cardiomyopathy with acute decompensation

cardiologyacuteadult
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Detailed

Acute decompensation in HCM = dynamic LVOT obstruction (SAM-driven) + diastolic dysfunction + often AFib — preload/afterload-dependent. Treatment paradox vs ADHF: give fluids, give phenylephrine, slow HR with esmolol, AVOID inotropes / vasodilators / aggressive diuresis

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HCM-specific physiology framed before any drug ordered

Patient inputs (15)

Age input for ESC HCM-RISK-SCD; older patients more likely to have AF and require AC; mavacamten and disopyramide tolerability depends on age and frailty

HypoK in hypertrophied stiff LV worsens VT/VF risk; also affects disopyramide proarrhythmia; replete K to ≥4.0

ESC HCM-RISK-SCD component + ACC/AHA 2024 SCD risk stratification; cascade screening referral

Detect inadvertent vasodilators (ACEi, ARB, nitrates), inotropes, or aggressive diuretics that may have precipitated obstruction crisis; need to stop or switch

Bedside or formal TTE — resting + provocable LVOT gradient, SAM, septal thickness, LVEF, mitral regurgitation severity — drives ALL drug decisions

Asymmetric septal hypertrophy ≥15 mm (≥13 mm with FH or genetic confirmation) — diagnostic anchor; ≥30 mm = high-SCD-risk band per ACC/AHA 2024 HCM

LVH voltage criteria, lateral T-wave inversion, abnormal Q-waves; new AFib detection drives immediate AC + rate/rhythm strategy

Elevated in HCM acute decompensation; useful for trending decongestion but interpret cautiously (chronic elevation in HCM regardless of acute state)

Hypotension in HCM = SAM-mediated LVOT obstruction collapse — treat with FLUIDS + phenylephrine, NOT inotropes; SBP <90 with HCM precipitant triggers obstruction-crisis pathway

Tachycardia worsens LVOT gradient by shortening diastolic filling; HR target <80 bpm with esmolol IV; new AFib RVR requires emergent rate control or cardioversion

Pulmonary edema secondary to acute diastolic dysfunction + SAM-driven mitral regurgitation — SpO2 < 92% triggers NIPPV (use carefully — avoid pre-load drop)

eGFR for disopyramide (renal-adjusted), mavacamten (CYP-mediated metabolism — not strict renal cutoff but used in monitoring), AC dosing if AF

HypoMg ↑ TdP risk on disopyramide (QT prolongation); maintain Mg ≥2.0

CMR LGE quantifies fibrosis burden — high LGE (>15% LV mass) refines SCD risk upward beyond HCM-RISK-SCD score; identifies apical aneurysm

Major SCD risk modifier in HCM (ESC 2014; ACC/AHA 2024)

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

Severity triggers (5)

5 need judgement
  • informationallife_threateningsam_mediated_lvot_obstruction_crisis_worsened_by_inotropes
    HCM patient given inadvertent inotrope (dobutamine, milrinone, epinephrine drip) for hypotension — paradoxically worsens SAM-mediated LVOT obstruction with hemodynamic collapse
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateningnew_afib_with_rvr_destabilizing_diastolic_filling_in_hcm
    New-onset AFib with rapid ventricular response in HCM patient — loss of atrial kick + tachycardia → catastrophic drop in LV filling because stiff hypertrophied LV depends on atrial systole
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereseptal_reduction_therapy_decision_for_refractory_obstruction
    HCM patient with persistent NYHA III-IV symptoms + LVOT gradient ≥50 mmHg at rest or with provocation despite maximally tolerated medical therapy (BB or CCB ± disopyramide ± mavacamten) — septal reduction therapy referral
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseveremavacamten_lvef_drop_below_50pct_during_titration
    HCM patient on mavacamten with REMS echo showing LVEF drop <50% during titration — REMS-mandated dose reduction or discontinuation
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereunexplained_syncope_or_nsvt_or_high_lge_burden_meeting_icd_criteria
    HCM patient with unexplained syncope, NSVT on Holter, family history of SCD, LV thickness ≥30 mm, apical aneurysm on CMR, or high LGE burden (>15% LV mass) — primary-prevention ICD indication
    Trigger could not be auto-evaluated — needs clinician judgement.

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Recommended regimen

HCM acute decompensation — preload/afterload-dependent: fluids + phenylephrine + esmolol + AVOID inotropes/vasodilators; chronic: BB/CCB/disopyramide → mavacamten → septal reduction therapy (ACC/AHA 2024 HCM)
axis: hcm_acute_decompensation_preload_afterload_dependent_management
Selected axis "HCM acute decompensation — preload/afterload-dependent: fluids + phenylephrine + esmolol + AVOID inotropes/vasodilators; chronic: BB/CCB/disopyramide → mavacamten → septal reduction therapy (ACC/AHA 2024 HCM)" by default fallback (first axis)
  • phenylephrine
    first line
    pure_alpha_1_agonist_vasopressor
    50-200 mcg IV bolus or 0.5-3 mcg/kg/min infusion titrate to MAP ≥65 • IV • bolus or continuous infusion
    triggers: hcm_acute_decompensation_with_hypotension, sam_mediated_lvot_obstruction
    Pure α-agonist increases afterload → reduces LVOT gradient by improving aortic ejection and abolishing SAM; preferred over norepinephrine (β-component worsens obstruction) and ABSOLUTELY preferred over inotropes
    rxcui 8163
  • esmolol
    first line
    beta_1_selective_blocker_short_acting
    500 mcg/kg IV bolus then 50-300 mcg/kg/min infusion titrate to HR <80 • IV • continuous infusion
    triggers: hcm_acute_decompensation_with_tachycardia, sam_mediated_lvot_obstruction, new_afib_with_rvr_in_hcm
    Short-acting cardioselective β-blocker lengthens diastolic filling time, reduces inotropy → reduces LVOT gradient; titratable, off rapidly if hemodynamic compromise
    rxcui 203222
  • metoprolol_tartrate
    first line
    beta_1_selective_blocker
    25 mg PO BID titrate to maximally tolerated • PO • BID
    triggers: chronic_hcm_obstructive_or_non_obstructive, transition_from_iv_esmolol
    ACC/AHA 2024 HCM Class I — first-line chronic β-blockade for symptomatic HCM; reduces inotropy + lengthens diastole
    rxcui 203191
  • verapamil
    first line
    non_dhp_ccb_phenylalkylamine
    120 mg PO daily extended-release titrate to 480 mg/d max • PO • daily ER
    triggers: hcm_intolerant_to_beta_blocker, symptomatic_obstructive_or_non_obstructive_hcm
    ACC/AHA 2024 HCM Class I alternative — non-DHP CCB; CAUTION in obstructive HCM with very high gradient or heart failure (vasodilator effect can worsen gradient — start low)
    rxcui 11170
  • disopyramide
    second line
    class_ia_antiarrhythmic_negative_inotrope
    40-50 mg PO TID-QID extended-release titrate to 600-800 mg/d max; renal-adjusted • PO • TID-QID
    triggers: symptomatic_obstructive_hcm_refractory_to_bb_or_ccb, sam_with_lvot_gradient_≥50_mmhg
    ACC/AHA 2024 HCM Class IIa — negative inotrope reduces SAM and LVOT gradient; QT prolongation + anticholinergic effects + proarrhythmic — monitor QT, K, Mg
    rxcui 3541
  • mavacamten
    second line
    cardiac_myosin_inhibitor
    5 mg PO daily start; titrate q4w per echo per REMS (max 15 mg/d) • PO • daily
    triggers: symptomatic_obstructive_hcm_nyha_ii_iii_refractory_to_bb_ccb_or_disopyramide, patient_avoids_septal_reduction_therapy
    ACC/AHA 2024 HCM Class IIa — first-in-class cardiac myosin inhibitor; EXPLORER-HCM PMID 32861276 (Olivotto Lancet 2020): 30% relative improvement in pVO2 + ~47% reduction in resting LVOT gradient; VALOR-HCM 2023: reduces septal reduction therapy referrals; REMS program — echo q4w during titration to detect LVEF drop <50%
    rxcui 2600867
  • apixaban
    comorbidity specific
    doac_factor_xa_direct
    5 mg PO BID (2.5 mg BID per FDA dose-reduction criteria) • PO • BID indefinite
    triggers: any_afib_in_hcm_regardless_of_cha2ds2vasc
    ACC/AHA 2024 HCM Class I — ANY AF in HCM = anticoagulate regardless of CHA2DS2-VASc score; DOAC preferred over warfarin; RE-LY/ARISTOTLE/ROCKET-AF/ENGAGE class evidence applies
    rxcui 1364430
  • rivaroxaban
    comorbidity specific
    doac_factor_xa_direct
    20 mg PO daily with food (15 mg if CrCl 15-50) • PO • daily indefinite
    triggers: any_afib_in_hcm_apixaban_alternative
    ACC/AHA 2024 HCM Class I — DOAC alternative for AF in HCM
    rxcui 1114195
  • warfarin
    comorbidity specific
    vitamin_k_antagonist
    5 mg PO daily; INR target 2-3 • PO • daily indefinite
    triggers: mechanical_valve_or_doac_contraindicated_or_unaffordable_in_hcm_with_af
    ACC/AHA 2024 HCM Class I if DOAC contraindicated; INR 2-3
    rxcui 11289

outpatient playbook — drug actions (2)

  1. 1. continue chronic regimen at maximally tolerated dose
    rxcui 20352
    metoprolol tartrate or verapamil + disopyramide if needed + mavacamten if eligible • PO • as scheduled
    trigger: Chronic HCM
    ACC/AHA 2024 HCM Class I/IIa
  2. 2. continue AC if AF
    rxcui 1364430
    apixaban 5 mg PO BID indefinite • PO • BID
    trigger: Any AF in HCM
    ACC/AHA 2024 HCM Class I

Auto-drafted A&P note

outpatient

Subjective

- Possible entry pathways: Acute dyspnea / orthopnea / pre-syncope in patient with known HCM (resting or provocable LVOT gradient ≥30 mmHg) — sudden hemodynamic deterioration; Bedside echo: new or worsened SAM-mediated LVOT obstruction with dynamic gradient (rest ≥50 mmHg or with Valsalva/exercise provocation) in HCM patient; New-onset AFib with rapid ventricular response in HCM — loss of atrial kick → catastrophic LV filling drop because stiff hypertrophied LV depends on atrial systole.

Objective

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

Assessment

**Hypertrophic cardiomyopathy with acute decompensation** (cardio.acute-hf.hcm-decompensation.v1).
Phenotype framing: Obstructive (HOCM, resting or provocable gradient ≥30 mmHg) vs non-obstructive vs apical HCM vs end-stage dilated phenotype (rare 5% with LVEF <50% — classic ADHF GDMT applies); exclude phenocopies (TTR amyloid, Fabry, Danon, athlete heart, hypertensive HD)
Scope: Acute decompensation in HCM = dynamic LVOT obstruction (SAM-driven) + diastolic dysfunction + often AFib — preload/afterload-dependent. Treatment paradox vs ADHF: give fluids, give phenylephrine, slow HR with esmolol, AVOID inotropes / vasodilators / aggressive diuresis

No severity triggers fired against current inputs.

Plan

Regimen axis: **HCM acute decompensation — preload/afterload-dependent: fluids + phenylephrine + esmolol + AVOID inotropes/vasodilators; chronic: BB/CCB/disopyramide → mavacamten → septal reduction therapy (ACC/AHA 2024 HCM)**.
1. phenylephrine 50-200 mcg IV bolus or 0.5-3 mcg/kg/min infusion titrate to MAP ≥65 IV bolus or continuous infusion (pure_alpha_1_agonist_vasopressor, first line) — Pure α-agonist increases afterload → reduces LVOT gradient by improving aortic ejection and abolishing SAM; preferred over norepinephrine (β-component worsens obstruction) and ABSOLUTELY preferred over inotropes
2. esmolol 500 mcg/kg IV bolus then 50-300 mcg/kg/min infusion titrate to HR <80 IV continuous infusion (beta_1_selective_blocker_short_acting, first line) — Short-acting cardioselective β-blocker lengthens diastolic filling time, reduces inotropy → reduces LVOT gradient; titratable, off rapidly if hemodynamic compromise
3. metoprolol_tartrate 25 mg PO BID titrate to maximally tolerated PO BID (beta_1_selective_blocker, first line) — ACC/AHA 2024 HCM Class I — first-line chronic β-blockade for symptomatic HCM; reduces inotropy + lengthens diastole
4. verapamil 120 mg PO daily extended-release titrate to 480 mg/d max PO daily ER (non_dhp_ccb_phenylalkylamine, first line) — ACC/AHA 2024 HCM Class I alternative — non-DHP CCB; CAUTION in obstructive HCM with very high gradient or heart failure (vasodilator effect can worsen gradient — start low)
5. disopyramide 40-50 mg PO TID-QID extended-release titrate to 600-800 mg/d max; renal-adjusted PO TID-QID (class_ia_antiarrhythmic_negative_inotrope, second line) — ACC/AHA 2024 HCM Class IIa — negative inotrope reduces SAM and LVOT gradient; QT prolongation + anticholinergic effects + proarrhythmic — monitor QT, K, Mg
6. mavacamten 5 mg PO daily start; titrate q4w per echo per REMS (max 15 mg/d) PO daily (cardiac_myosin_inhibitor, second line) — ACC/AHA 2024 HCM Class IIa — first-in-class cardiac myosin inhibitor; EXPLORER-HCM PMID 32861276 (Olivotto Lancet 2020): 30% relative improvement in pVO2 + ~47% reduction in resting LVOT gradient; VALOR-HCM 2023: reduces septal reduction therapy referrals; REMS program — echo q4w during titration to detect LVEF drop <50%
7. apixaban 5 mg PO BID (2.5 mg BID per FDA dose-reduction criteria) PO BID indefinite (doac_factor_xa_direct, comorbidity specific) — ACC/AHA 2024 HCM Class I — ANY AF in HCM = anticoagulate regardless of CHA2DS2-VASc score; DOAC preferred over warfarin; RE-LY/ARISTOTLE/ROCKET-AF/ENGAGE class evidence applies
8. rivaroxaban 20 mg PO daily with food (15 mg if CrCl 15-50) PO daily indefinite (doac_factor_xa_direct, comorbidity specific) — ACC/AHA 2024 HCM Class I — DOAC alternative for AF in HCM
9. warfarin 5 mg PO daily; INR target 2-3 PO daily indefinite (vitamin_k_antagonist, comorbidity specific) — ACC/AHA 2024 HCM Class I if DOAC contraindicated; INR 2-3

Setting playbook (outpatient) — Long-term HCM management: HCM specialist clinic q3-6 mo; mavacamten REMS echo q12w maintenance; annual Holter for NSVT; cardiac MRI q3-5y; cascade family screening; ICD/septal reduction therapy if escalation needed; AC continuation for AF
10. continue chronic regimen at maximally tolerated dose metoprolol tartrate or verapamil + disopyramide if needed + mavacamten if eligible PO as scheduled — Chronic HCM (ACC/AHA 2024 HCM Class I/IIa)
11. continue AC if AF apixaban 5 mg PO BID indefinite PO BID — Any AF in HCM (ACC/AHA 2024 HCM Class I)

Non-pharmacologic actions:
- Sports clearance reassessment annually
- Cascade family screening
- ICD/septal reduction reassessment if symptomatic progression
- Pregnancy planning specialist referral if applicable

AVOID / contraindication checks:
- Absolute_contraindication_dobutamine_milrinone_epinephrine_in_hcm_obstruction (worsens SAM and LVOT gradient — never give inotropes for HCM hypotension)
- Absolute_contraindication_nitroglycerin_acei_arb_arni_in_acute_hcm_obstruction (vasodilator drops afterload → worsens LVOT gradient)
- Caution_aggressive_diuresis_in_hcm (drops preload → worsens LVOT gradient; use small doses only if frank pulmonary edema)
- Caution_dhp_ccb_amlodipine_in_hcm_obstructive (vasodilator effect worsens gradient — use non DHP only)
- Disopyramide_qt_monitoring_required_with_k_and_mg_repletion (proarrhythmic if hypoK / hypoMg)
- Mavacamten_rems_echo_lvef_monitoring_q4w_during_titration_then_q12w_maintenance (LVEF drop <50% → dose reduction or discontinuation)
- Mavacamten_avoid_with_strong_cyp3a4_inhibitors_or_inducers (clarithromycin, ketoconazole, rifampin — drug interaction algorithm in REMS)
- Decision:any_af_in_hcm_class_i_anticoagulation_regardless_of_cha2ds2vasc (ACC/AHA 2024 HCM)
- Decision:septal_reduction_therapy_at_experienced_center_for_lvot_gradient_≥50_mmhg_nyha_iii_iv_refractory_to_medical_therapy (Class I)
- Decision:icd_for_primary_prevention_per_esc_2014_hcm_risk_score_5pct_5y_or_acc_aha_2024_modifiers (LGE, family history SCD, syncope, NSVT, apical aneurysm, LV thickness ≥30 mm)

Monitoring

Regimen monitoring:
- continuous ecg telemetry for af and vt vf surveillance (Class I)
- echo q4w during mavacamten titration per rems (FDA label)
- echo q12w maintenance on mavacamten (REMS)
- qt interval baseline then q3d during disopyramide titration then quarterly (FDA label)
- k and mg repletion to above 4.0 and above 2.0 (proarrhythmia prevention)
- cmr with lge at diagnosis then q3 5y for fibrosis progression (ACC/AHA 2024)
- holter 24 48h at baseline and q1 2y for nsvt scd modifier (ESC 2014; ACC/AHA 2024)
- cascade family screening q3 5y or at genetic test completion (ESC 2014; ACC/AHA 2024)

Setting (outpatient) monitoring:
- Echo q3-6 mo or q12w on mavacamten maintenance
- Holter q1-2y
- Cardiac MRI q3-5y
- Annual SCD risk reassessment

Follow-up plan: HCM specialist clinic within 1-2 weeks; cascade family screening (genetic counseling); mavacamten REMS echo q4w during titration then q12w maintenance; ICD/septal reduction decision finalised; sports clearance shared decision; cardiac rehab if NYHA II-III
- Close-out criterion: long-term plan + family screening + follow-up cadence finalised

Monitoring phase: Telemetry continuous (VT/VF surveillance + AFib detection); daily echo if mavacamten initiation (REMS — LVEF q4w during titration to detect drop <50%); BMP daily; QT on disopyramide; if AFib AC therapeutic; serial Holter for NSVT (SCD modifier)

Disposition

Current setting: outpatient — Long-term HCM management: HCM specialist clinic q3-6 mo; mavacamten REMS echo q12w maintenance; annual Holter for NSVT; cardiac MRI q3-5y; cascade family screening; ICD/septal reduction therapy if escalation needed; AC continuation for AF

Disposition criteria:
- Indefinite HCM specialist follow-up; lifelong AC if AF; ICD if SCD criteria met; mavacamten or septal reduction per individual response

Escalation triggers (move to higher acuity):
- Symptomatic progression → escalate medical therapy or septal reduction therapy
- New VT/VF or syncope → urgent EP / ICD evaluation
- New AF → AC initiation
- End-stage dilated phenotype (LVEF <50%) → switch to cardio.hfref.core.v1 for GDMT 4-pillar

Earlier-Return Triggers

Return-precaution thresholds (watch for):
- [LIFE_THREATENING] HCM patient given inadvertent inotrope (dobutamine, milrinone, epinephrine drip) for hypotension — paradoxically worsens SAM-mediated LVOT obstruction with hemodynamic collapse
- [LIFE_THREATENING] New-onset AFib with rapid ventricular response in HCM patient — loss of atrial kick + tachycardia → catastrophic drop in LV filling because stiff hypertrophied LV depends on atrial systole
- [SEVERE] HCM patient with persistent NYHA III-IV symptoms + LVOT gradient ≥50 mmHg at rest or with provocation despite maximally tolerated medical therapy (BB or CCB ± disopyramide ± mavacamten) — septal reduction therapy referral

Citations

- ACC/AHA 2024 HCM Guideline + ESC 2023 cardiomyopathies (Arbelo) + ESC 2014 HCM (Elliott) for SCD risk [PMID:37622666](https://pubmed.ncbi.nlm.nih.gov/37622666/)
- Cited evidence (PMID 25173338) [PMID:25173338](https://pubmed.ncbi.nlm.nih.gov/25173338/)
- Cited evidence (PMID 32861276) [PMID:32861276](https://pubmed.ncbi.nlm.nih.gov/32861276/)
- Cited evidence (PMID 38587234) [PMID:38587234](https://pubmed.ncbi.nlm.nih.gov/38587234/)
- Cited evidence (PMID 23223539) [PMID:23223539](https://pubmed.ncbi.nlm.nih.gov/23223539/)

Last reconciled with current guidelines: 2026-05-15.
References