Hypertrophic obstructive cardiomyopathy is characterised by left ventricular outflow tract obstruction, myocardial hypertrophy, and diastolic dysfunction. It is commonly caused by genetic mutations in sarcomere proteins. There is a hypertrophied interventricular septum, which blocks blood from flowing through the aortic valve.
It peaks at 20 – 30 years, and affects men and women equally.
- Associated conditions
- Friedreich’s Ataxia
- Wolff-Parkinson White Syndrome
- Exacerbating factors
- Reduced preload (reduced left ventricular end diastolic volume)
- Hypovolemia
- Tachycardia (reduced ventricular filling)
- Valsava
- Arrhythmia
- Reduce afterload
- Positive inotropes (digoxin and dobutamine)
- Beta agonists
- Nitrates
- ACEIs
- Reduced preload (reduced left ventricular end diastolic volume)
- Signs and symptoms of HOCM
- Syncope (most common presentation)
- Systolic ejection murmur at the left lower sternal border
- Increases with Valsalva (reduced preload)
- Decreases with squatting or handgrip
- S4 gallop
- Bifid carotid pulse
- Angina
- Palpitations due to atrial or ventricular arrhythmias
- Signs of heart failure in advanced disease
- Sudden death
- Differentials
- Aortic stenosis
- Coronary artery disease
- Athletic heart syndrome
- Investigations
- Echocardiography
- Asymmetric septal hypertrophy
- Systolic anterior motion of the mitral valve
- Left ventricular outflow tract obstruction
- Diastolic dysfunction
- Electrocardiogram
- Left ventricular hypertrophy
- T-wave inversion
- Arrhythmias
- Cardiac MRI to assess myocardial hypertrophy, fibrosis, and left ventricular function
- Exercise stress test
- Genetic testing
- Echocardiography
- Treatment
- Beta blockers (metoprolol) to increase diastolic filling
- Calcium channel blockers (verapamil) in place of beta blockers
- Dysopyramide (negative inotrope) and amiodarone are second-line
- Anticoagulation in case of atrial fibrillation or a history of thromboembolic events
- Avoid dehydration
- Avoid strenuous activities
- Avoid medications that reduce left-ventricular volume – ACEIs/ARBs, nitrates, positive inotropes, and beta agonists
- Catheter septal ablation or myocardectomy
- Implantable cardioverter-defibrillator (ICD) for primary or secondary prevention of sudden cardiac death in high-risk patients
- Complications
- Heart failure
- Atrial fibrillation
- Sudden cardiac death
- Mitral regurgitation
- Infective endocarditis
- Syncope
- Stroke
- Pulmonary hypertension