What is a good blood pressure target for a patient with apical Hypertrophic Cardiomyopathy (HCM) and an apical aneurysm who has developed Atrial Fibrillation (AF), and can they live without an Implantable Cardioverter-Defibrillator (ICD)?

Blood Pressure Management and ICD Necessity in Apical HCM with Aneurysm and AF

For patients with apical hypertrophic cardiomyopathy (HCM) with an apical aneurysm and atrial fibrillation (AF), a target blood pressure of <130/80 mmHg is recommended, and an ICD is absolutely necessary due to the extremely high risk of sudden cardiac death (4.7% per year) in this specific patient population. 1

Blood Pressure Management

Target BP for HCM with Apical Aneurysm and AF

• Target: <130/80 mmHg 2
• This target is based on evidence showing that lower BP goals are appropriate for patients with target-organ damage and cardiovascular disease
• The Prospective Studies Collaboration demonstrated a strong log-linear association between BP and cardiovascular disease risk across a wide range of blood pressures 2

Medication Selection for BP Control

1. First-line agents:

   • Beta-blockers (preferred in children and most adults)
   • Non-dihydropyridine calcium channel blockers (verapamil, diltiazem)
   • These medications also help with rate control for AF 2

2. Titration considerations:

   • Monitor for bradycardia and AV conduction block
   • Target resting heart rate <100 BPM 2
   • Assess rate control during exercise

3. Additional agents if needed:

   • Low-dose diuretics (loop or thiazide) for volume overload 2
   • Use cautiously to prevent symptomatic hypotension

ICD Necessity in Apical HCM with Aneurysm

High-Risk Features of Apical Aneurysm in HCM

• Patients with apical aneurysms have a sudden death event rate of 4.7% per year 1
• The combined rate of HCM-related deaths and life-saving aborted events is 6.4% per year - three times higher than HCM patients without aneurysms 1
• Recurrent monomorphic ventricular tachycardia is common in this population 1, 3

Evidence Supporting ICD Implantation

• In the largest study of HCM patients with apical aneurysms, 24% required life-saving interventions over a mean follow-up of just 4.4 years 1
• ICDs have proven highly effective in HCM patients, with appropriate intervention rates of 6.8% per year 4
• The subcutaneous ICD is also effective in HCM patients without pacing needs 5

Additional Management for AF in HCM

1. Mandatory anticoagulation regardless of CHA₂DS₂-VASc score 2

   • Direct oral anticoagulants (DOACs) as first-line option
   • Vitamin K antagonists (warfarin, target INR 2.0-3.0) as second-line option
   • Non-anticoagulated patients with apical aneurysms have a 1.1% annual thromboembolic event rate 1

2. Rhythm control strategy preferred 2

   • Amiodarone or disopyramide with rate-controlling agents 2
   • Catheter ablation for refractory symptoms 2

Monitoring and Follow-up

• Echocardiography every 1-2 years to assess for changes in LV function, wall thickness, and LVOT obstruction 2
• More frequent monitoring with changes in clinical status 2
• Consider cardiac MRI to better visualize apical aneurysm if TTE is suboptimal 2

Common Pitfalls to Avoid

1. Do not use digoxin for rate control as it may increase outflow gradient in HCM 2
2. Do not withhold anticoagulation based on CHA₂DS₂-VASc score alone 2
3. Do not delay ICD implantation in patients with apical aneurysms due to extremely high risk 1, 3
4. Do not allow participation in high-intensity competitive sports regardless of treatment 2
5. Do not aggressively lower BP below target as excessive lowering of diastolic BP may impair coronary perfusion 2