What is the recommended treatment for a patient with hypertrophic cardiomyopathy, normal systolic function, biatrial dilation, mild aortic stenosis, mild mitral regurgitation, and moderate mitral annular calcification?

Treatment for Hypertrophic Cardiomyopathy with Valvular Disease

For this patient with nonobstructive HCM (normal systolic function, no LVOT gradient mentioned), biatrial dilation, mild aortic stenosis, and mild mitral regurgitation, the primary treatment is beta-blockers or non-dihydropyridine calcium channel blockers (verapamil or diltiazem) to manage symptoms of dyspnea and angina, with diuretics added if symptoms persist despite initial therapy. 1

Initial Medical Management for Nonobstructive HCM

First-Line Therapy

• Beta-blockers are the mainstay of initial pharmacologic therapy due to their negative inotropic effects and ability to attenuate tachycardia, which prolongs diastolic filling and improves symptoms 1
• Non-dihydropyridine calcium channel blockers (verapamil or diltiazem) are recommended alternatives for patients unable to tolerate beta-blockers or with persistent symptoms 1
• The choice between beta-blockers and calcium channel blockers should be guided by patient comorbidities and preferences 1

Adjunctive Therapy

• Oral diuretics should be added when exertional dyspnea persists despite beta-blockers or calcium channel blockers 1
• This is particularly relevant given the biatrial dilation, which suggests elevated filling pressures 1

Medications to Avoid

• Dihydropyridine calcium channel blockers (e.g., nifedipine) should NOT be used as their vasodilatory effects may worsen any latent outflow obstruction 1
• ACE inhibitors and angiotensin receptor blockers have uncertain benefit for symptom management in nonobstructive HCM with preserved ejection fraction 1

Management of Atrial Fibrillation Risk

Given the biatrial dilation, this patient is at increased risk for atrial fibrillation:

• If atrial fibrillation develops (clinical or subclinical ≥24 hours), anticoagulation with direct-acting oral anticoagulants is mandatory, independent of CHA₂DS₂-VASc score 1
• For rate control in AF, beta-blockers, verapamil, or diltiazem are recommended 1

Valvular Disease Considerations

Mild Aortic Stenosis

• The mild aortic stenosis should be monitored according to standard ESC guidelines 1
• Up to one-third of HCM patients have mild aortic regurgitation (this patient has trace), likely from high-velocity LVOT flow 1
• Serial echocardiography is needed to monitor progression of the aortic stenosis 1

Mild Mitral Regurgitation

• The mild mitral regurgitation in nonobstructive HCM is likely intrinsic valve disease (given the thickened, calcified leaflets and moderate mitral annular calcification) rather than SAM-related 1
• No specific intervention is needed for mild MR at this time beyond medical management of HCM 1
• The mildly elevated gradient across the mitral valve requires monitoring but does not change management 1

Monitoring and Follow-Up

• Regular echocardiographic surveillance is essential to assess:

  • Progression of aortic stenosis 1
  • Development of LVOT obstruction (currently none evident) 1
  • Worsening of mitral regurgitation 1
  • Left atrial size and development of atrial fibrillation 1

• Sudden cardiac death risk stratification should be performed using established HCM risk factors, though this patient's normal systolic function (68% by 3D) is reassuring 1

Critical Pitfalls to Avoid

• Do not use vasodilators aggressively as they may unmask latent LVOT obstruction 1
• Do not assume the mitral regurgitation is solely from SAM - the thickened, calcified leaflets with MAC suggest intrinsic valve pathology requiring different monitoring 1
• Do not overlook endocarditis prophylaxis if LVOT obstruction develops, as HCM patients with obstruction and LA dilation have increased endocarditis risk (3.8 per 1000 person-years) 1
• Recognize that even mild reduction in LVEF may indicate reduced ventricular reserve in the setting of mitral regurgitation, though this patient's 68% LVEF is supranormal 2

Lifestyle Modifications

• Mild to moderate-intensity recreational exercise is beneficial for improving cardiorespiratory fitness and quality of life 1
• Avoid high-intensity competitive sports until comprehensive evaluation by an HCM expert 1
• Adequate hydration and avoidance of vasodilators (including alcohol) to prevent unmasking LVOT obstruction 1