Hypertrophic Obstructive Cardiomyopathy (HOCM)
Definition
HOCM is a type of hypertrophic cardiomyopathy (HCM) in which the heart muscle (especially the interventricular septum) becomes abnormally thickened (hypertrophied) and obstructs blood flow from the left ventricle to the aorta.
Pathophysiology
Primarily Genetic cause (often autosomal dominant).
The thickened septum narrows the left ventricular outflow tract (LVOT).
The mitral valve leaflet may be pulled toward the septum during contraction (systolic anterior motion, or SAM), worsening the obstruction.
This leads to increased pressure, reduced cardiac output, and sometimes arrhythmias.
Symptoms
Dyspnoea (shortness of breath)
Chest pain (especially with exertion)
Syncope (fainting), especially during exercise
Palpitations or irregular heartbeat
Sudden cardiac death (especially in young athletes)
Clinical findings
Harsh systolic murmur (heard best at left sternal border)
Increases with Valsalva or standing (less venous return)
Decreases with squatting (more venous return)
Possible bifid (spike-and-dome) pulse (two closely spaced peaks per cardiac cycle rather than the usual one).
Diagnosis
Echocardiogram (ECHO): asymmetric septal hypertrophy, SAM of mitral valve, LVOT obstruction.
ECG: left ventricular hypertrophy, deep Q waves, or repolarization abnormalities.
Genetic testing may confirm the diagnosis.
Treatment
Beta-blockers (first-line): reduce heart rate and contractility demand.
Calcium channel blockers (verapamil): alternative.
Avoid preload-reducing drugs (diuretics, nitrates).
Surgical options: septal myectomy or alcohol septal ablation.
Implantable cardioverter-defibrillator (ICD) for high-risk patients (to prevent sudden cardiac death).
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