Hypertrophic cardiomyopathy

[Phloston]

Sorry for the simple question, but what is the actual cause of death due to this disease?

The mitral (yes, mitral) valvular obstruction of the left ventricular outflow tract is obviously exacerbated with decreased intra-ventricular volume (e.g. during strenuous exercise), but how does that cause death?

Is it due to arrhythmia?

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[thinker]

eMedicine:

Sudden cardiac death
This is the most devastating presenting manifestation of HCM. It has the highest incidence in preadolescent and adolescent children and is particularly related to extreme exertion. The risk of sudden death in children is as high as 6% per year.

In more than 80% of cases, the arrhythmia that causes sudden death is ventricular fibrillation. Many of these cases degenerate into ventricular fibrillation from rapid atrial arrhythmias, such as fibrillation, supraventricular tachycardia, or Wolff-Parkinson-White syndrome, while others result from ventricular tachycardia and low cardiac output hemodynamic collapse.

 

[thehundredthone]

As a rule of thumb, sudden cardiac death = arrhythmia. MI, HOCM, WPW, Long QT, etc. all deteriorate into arrhythmias.

 

[busy08]

yup ventricular fibrillation

 

[Jonari]

Pholston do you know which autosomal dominant condition where HC is the most common cause of death?

 

[Phloston]

Pholston do you know which autosomal dominant condition where HC is the most common cause of death?

The first thing that comes to mind is Friedreich's ataxia --> chromosome 9 --> intronic GAA TNR. However, unlike Huntington's, this TNR expansion is autosomal recessive, not dominant.

I don't know, what?

 

[Jonari]

The first thing that comes to mind is Friedreich's ataxia --> chromosome 9 --> intronic GAA TNR. However, unlike Huntington's, this TNR expansion is autosomal recessive, not dominant.

I don't know, what?

Nice homie.

I'm glad you brought Fried's ataxia up with being recessive. Some sources site it as dominant whereas Kaplan has it as recessive. It's kind of a pita.