A cardiomyopathy with diastolic dysfunction due to hypertrophy, fibrosis and myocyte disarray

What are the defining characteristics of hypertrophic cardiomyopathy?

Hypertrophic Cardiomyopathy

Anything that pressure loads the ventricle:

Aortic Stenosis
Longstanding hypertension 
Athlete&#039;s heart

What are some other conditions that can cause a morphologically similar picture?

Trick question!

There is a wide range of morphology but some classic ones are septal hypertrophy as well as an apical variant.

Patchy fibrosis (scarring) and disorganised, chaotic myocytes

What are the causes/reasons for arrhythmogenicity in HOCM?

Systolic anterior motion of the mitral valve (SAM) is when the mitral valve moves into the left ventricular outflow tract.

This causes both left ventricular outflow tract obstruction AND mitral regurgitation.

What is systolic anterior motion of the mitral valve?

Chest pain - myocardial bridging of the coronary arteries and a large LV mean that there is a low &#039;coronary reserve&#039;
Dyspnoea - due to diastolic dysfunction 
Palpitations - due to atrial fibrillation/ventricular tachycardia
Syncope - due to LVOTO/arrhythmia

What are some of the presenting features of HCM and why do they arise?

An ejection systolic murmur at the lower-left sternal border 

The murmur can be augmented using the valsalva manoeuvre

What is the typical murmur of HOCM?

How can you differentiate it from other murmurs?

Family history of sudden cardiac death

Unexplained syncope 

Aberrant exercise response (hypotension) 

Runs of non-sustained ventricular tachycardia (&lt;30 seconds) 

Massive LV Hypertrophy (&gt;3cm)

What are some indications for primary prevention insertion of an implantable cardioverter defibrillator?

Reducing contractility and increasing diastolic filling time.

This is done by using beta-blockers and calcium channel blockers (centrally acting, non-dihydropyridine class) 

Eventually if people develop DECREASED systolic function, then you can institute the usual drugs (ACEi, ARBs, Spironolactone etc)

What is the principle of treatment of heart failure in HOCM and how is it acheived?

Very low threshhold.

CHADSVAS is not validated in AF.

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Hypertrophic Cardiomyopathy Flashcards

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