Should a patient with hypertrophic obstructive cardiomyopathy (HOCM), moderate asymmetric septal hypertrophy, and symptoms of chest pain and shortness of breath be referred to an HCM (Hypertrophic Cardiomyopathy) clinic or can cardiology manage the condition?

Referral to HCM Clinic for Symptomatic Hypertrophic Obstructive Cardiomyopathy

This patient with confirmed HOCM and active symptoms of chest pain and shortness of breath should be referred to an HCM clinic. 1

Why Referral is Strongly Recommended

The 2024 AHA/ACC guidelines explicitly state that referral to a comprehensive HCM center is reasonable when challenging management decision-making arises for this complex condition. 1 While general cardiologists can manage many aspects of HCM, this patient's symptomatic presentation with confirmed obstructive disease warrants specialized evaluation. 1

Key reasons supporting referral:

• Symptomatic obstructive disease requires specialized assessment - The patient has both chest pain and dyspnea with confirmed HOCM on cardiac MRI, indicating they may be a candidate for advanced therapies beyond basic medical management. 1

• HCM centers offer comprehensive diagnostic capabilities - These include genetic testing and counseling, advanced imaging interpretation, risk stratification for sudden cardiac death, and access to all treatment modalities including septal reduction therapies. 1

• Most cardiologists manage only a few HCM patients - The guidelines emphasize that HCM is infrequent in general practice, with most cardiologists caring for only a handful of patients, making specialized expertise valuable. 1

What General Cardiology Can Do Before Referral

While awaiting HCM clinic evaluation, the general cardiologist can initiate appropriate management:

Immediate medical therapy:

• Start beta-blockers as first-line treatment for symptomatic patients with obstructive or nonobstructive HCM (Class I recommendation). 2, 3
• Verapamil can be used as an alternative if beta-blockers are not tolerated, but use with caution in patients with high gradients or advanced heart failure. 2, 3

Essential diagnostic workup:

• Perform 12-lead ECG to identify conduction abnormalities and signs of ventricular hypertrophy. 4
• Order 48-hour ambulatory Holter monitoring to detect nonsustained ventricular tachycardia, atrial fibrillation, or bradyarrhythmias. 4, 5
• Conduct standard upright exercise testing to assess blood pressure response, reproduce symptoms, and evaluate for exercise-induced arrhythmias. 4
• Obtain exercise echocardiography if not already done, to detect provocable LVOT obstruction and assess gradient severity with physiologic stress. 4, 5

Risk stratification for sudden cardiac death:

• Assess for major risk factors including family history of premature HCM-related sudden death, unexplained syncope (particularly if exertional), nonsustained VT on Holter, abnormal blood pressure response during exercise, and extreme LV hypertrophy (≥30 mm). 4, 5

When HCM Center Referral is Particularly Critical

Mandatory referral situations per 2024 guidelines: 1

• Candidates for septal reduction therapy - Patients with severe symptoms despite optimal medical therapy and resting or provocable gradients ≥50 mm Hg should be referred to high-volume centers. 1

• Complex ICD decision-making - When primary prevention ICD decisions are challenging based on risk stratification. 1

• Genetic counseling needs - For comprehensive genetic testing and family screening coordination. 1

• Complex arrhythmia management - Catheter ablation for ventricular or complex atrial tachyarrhythmias requires specialized expertise. 1

Evidence Supporting Specialized HCM Centers

Research demonstrates tangible benefits of specialized HCM clinic attendance:

• Improved family screening - One study showed familial screening increased from 4% to 45.3% after HCM unit creation, with genetic studies rising from 14% to 70.3%. 6

• Enhanced diagnostic workup - Specialized clinics increased cardiac MRI utilization by 70%, 24-hour Holter monitoring by 39%, and stress echocardiography by 21%. 7

• Better risk stratification - Sudden death risk scoring improved from 28% to 67.2% of patients when managed in specialized units. 6

• Optimized medical therapy - Medications were optimized in 47% of patients following specialized clinic attendance. 7

Procedural Volume Matters for Outcomes

If septal reduction therapy becomes necessary, center experience is critical: 1

• Centers performing invasive septal reduction therapies should achieve ≤1% 30-day mortality, >90% symptomatic improvement, and >90% success in reducing gradients to <50 mm Hg. 1

• Low-volume centers have significantly increased mortality and morbidity rates, as well as higher mitral valve replacement rates. 1

• Operators should have performed at least 20 procedures or work within an HCM program with cumulative volume of at least 50 procedures. 2

Common Pitfalls to Avoid

• Don't assume general cardiology management alone is sufficient - Even with appropriate medical therapy initiation, this symptomatic patient needs specialized evaluation for comprehensive risk assessment and treatment planning. 1

• Don't delay referral until medical therapy fails - The 2024 guidelines support referral for complex management decisions, not just for procedural interventions. 1

• Don't overlook family screening - HCM centers systematically screen first-degree relatives with three-generation family history assessment, which is often inadequately performed in general practice. 1, 6

• Don't underestimate sudden death risk - This patient's symptoms warrant formal risk stratification that HCM centers perform systematically using validated risk scores. 4, 5, 6