Approach to the Critical Patient

Hemodynamic priorities

Acute hemodynamic compromise in HOCM

Arrhythmia management

Rapid atrial fibrillation with hemodynamic compromise

Monitoring and escalation

Monitoring bundle for hemodynamically unstable HOCM

Immediate consults

Consultation triggers in acute HOCM

History

Presenting symptoms

Cardinal exertional symptoms

Symptom triggers

Provocative factors worsening LVOTO

Alarm features

High-risk features requiring urgent evaluation

Family and genetic history

Three-generation family history — Class I recommendation

Risk factor assessment

Structural and genetic risk factors

Medication history

Current and prior cardiac medications

Physical Exam

Vital signs

Hemodynamic assessment

Cardiac examination

Murmur characteristics — key diagnostic finding

Precordial and peripheral findings

Characteristic physical signs

Dynamic maneuver technique

Bedside provocative maneuvers

Signs of heart failure

Volume overload assessment

Differential Diagnosis

Structural cardiac causes

Aortic stenosis — most critical mimic

Hypertensive heart disease

Valvular mitral regurgitation (primary)

Infiltrative and storage diseases

Cardiac amyloidosis

Fabry disease (alpha-galactosidase A deficiency)

Danon disease and other glycogen storage diseases

Physiologic and other causes

Athlete's heart

RASopathies (Noonan syndrome)

Mitochondrial cardiomyopathy

Laboratory Tests

Cardiac biomarkers

BNP or NT-proBNP

Troponin (high-sensitivity)

Metabolic and hematologic tests

Basic metabolic panel

Complete blood count

Thyroid function tests

Targeted metabolic screening

Alpha-galactosidase A enzyme activity

Lysosomal enzyme panel if glycogen storage disease suspected

Genetic testing

Sarcomeric gene panel

Diagnostic Tests

Scoring Systems

HCM Risk-SCD Calculator (ESC model)

NYHA Functional Classification

MRI

Cardiac MRI (CMR) — gold standard for HCM characterization

CT

CT cardiac imaging in HCM — secondary role

Ultrasound

Transthoracic echocardiography (TTE) — first-line diagnostic test

ECG — screening and risk stratification

Ambulatory ECG monitoring

Disposition

Admission criteria

Indications for hospital admission

Observation indications

Step-down or observation unit

Discharge criteria

Criteria for safe discharge

Specialist referral

Referral triggers

Treatment

Acute stabilization

Acute HOCM hemodynamic crisis management

Chronic pharmacologic management

Step 1 — Beta-blocker (first-line)

Step 2 — Non-dihydropyridine calcium channel blocker (beta-blocker intolerant)

Step 3 — Disopyramide (refractory symptoms)

Step 4 — Cardiac myosin inhibitors

Septal reduction therapy

Surgical septal myectomy — gold standard intervention

Alcohol septal ablation — alternative for non-surgical candidates

SCD prevention

Implantable cardioverter-defibrillator (ICD)

AF management

Anticoagulation for all HCM patients with AF

Special Populations

Pregnancy

HCM in pregnancy — generally well-tolerated but requires specialized management

Geriatric

HCM in older adults (age 60 and above)

Pediatrics

HCM in children and adolescents

Background

Epidemiology

Prevalence and incidence

Morbidity and mortality

Pathophysiology

Core mechanism — dynamic LVOT obstruction

LVOTO classification

Diastolic dysfunction

Microvascular ischemia

Therapeutic Considerations

Rationale for treatment approach

ICD therapy evidence

Exercise and lifestyle evidence

Patient Discharge Instructions

copy discharge instructions

Discharge instructions for Hypertrophic Cardiomyopathy (HOCM)

References

Guidelines and key sources

AHA/ACC/AMSSM/HRS/PACES/SCMR 2024 Guideline for Management of HCM

2020 AHA/ACC Guideline for Diagnosis and Treatment of HCM

Braunwald E. Hypertrophic Cardiomyopathy

Maron BJ, Desai MY, Nishimura RA, et al. Diagnosis and Evaluation of HCM: JACC State-of-the-Art Review

Maron BJ, Desai MY, Nishimura RA, et al. Management of HCM: JACC State-of-the-Art Review

Landmark trials and additional references

Sikand N, Stendahl J, Sen S, Lampert R, Day S. Current Management of HCM

Leggit JC, Whitaker D. Diagnosis and Management of HCM: Updated ACC/AHA Guidelines

Fahim M, et al. Mavacamten and Aficamten in HCM: Systematic Review and Meta-Analysis

Nagueh SF, et al. ASE Recommendations for Multimodality Cardiovascular Imaging in HCM

Ommen SR, Semsarian C. HCM: A Practical Approach to Guideline-Directed Management

Meder B, Coats CJ, Leinwand LA, et al. EJHF Expert Consensus Statement on HCM

Coppiano J, Carrasco M, Asif I. Cardiomyopathy: A Guide for Primary Care