From the Guidelines
For a patient with small left ventricular size, mild concentric left ventricular hypertrophy, low resting LVOT gradient (7 mmHg) that increases with Valsalva (19 mmHg), and recurrent syncope following coughing, I recommend starting beta-blocker therapy with metoprolol 25-50 mg twice daily or bisoprolol 2.5-5 mg once daily, as this approach is supported by the most recent guidelines 1.
Key Considerations
- The patient's symptoms are likely due to transient increases in LVOT obstruction during coughing, which reduces cardiac output and cerebral perfusion.
- Beta-blockers reduce heart rate, contractility, and LVOT obstruction, which can help prevent syncope episodes triggered by coughing (cough syncope) 2.
- If beta-blockers are contraindicated or poorly tolerated, consider non-dihydropyridine calcium channel blockers like verapamil 120-240 mg daily in divided doses.
- Avoid vasodilators, diuretics, and positive inotropic agents as they may worsen LVOT obstruction.
- The patient should maintain adequate hydration and avoid triggers like prolonged standing, alcohol, and excessive heat.
Monitoring and Follow-up
- Close follow-up with serial echocardiography every 6-12 months is essential to monitor for disease progression 1.
- If medical therapy fails to control symptoms, referral for evaluation for septal reduction therapy (myectomy or alcohol septal ablation) may be necessary.
Additional Recommendations
- Comprehensive 2D echocardiography has a primary role in establishing the diagnosis of HCM, determining hypertrophy pattern, presence of LV apical aneurysms, LV systolic and diastolic function, mitral valve function, and presence and severity of LVOTO 1.
- Routine follow-up of patients with HCM is an important part of optimal care, with serial TTE performed every 1 to 2 years to assess for changes in LV systolic and diastolic function, wall thickness, chamber size, LVOTO, and concomitant valvular disease 1.
From the Research
Management of Hypertrophic Cardiomyopathy with Obstruction and Syncope
- The patient's condition, characterized by small left ventricular size, mild concentric left ventricular hypertrophy, and a resting LVOT gradient of 7, with a peak gradient of 19 during Valsalva maneuver, is consistent with hypertrophic obstructive cardiomyopathy (HOCM) 3.
- The recurrent syncope following coughing suggests a high-risk condition, potentially indicating an increased risk of sudden cardiac death (SCD) 4, 5.
- Management of HOCM involves activity restriction, prevention of sudden cardiac death, control of symptoms, and screening of relatives 3.
- Pharmacologic treatment of symptoms in patients with HOCM consists of negative inotropic drugs, such as beta blockers, and disopyramide; a nondihydropyridine calcium channel blocker (CCB), usually verapamil, may be used in patients with noncardiac side-effects of beta blockers 3.
- For patients with intolerable symptoms despite optimal conservative therapy, septal reduction therapy (SRT) should be considered, and should be performed by experienced operators in institutions with multidisciplinary HCM programs 3.
Evaluation and Management of Syncope
- Syncope is a clinical syndrome defined as a relatively brief self-limited transient loss of consciousness (TLOC) caused by a period of inadequate cerebral nutrient flow 4, 5.
- Establishing an accurate basis for the etiology of syncope is crucial in order to initiate effective therapy 4, 5.
- A thorough history and physical examination, including orthostatic assessment, are crucial for making the diagnosis of syncope 5.
- Short-term risk assessment should be performed to determine the need for admission, and monitoring is indicated until a diagnosis is made 5, 6.
- Mechanism-specific therapy, such as counteracting hypotension or bradycardia, is highly effective in preventing recurrences of syncope 7.