NORD - Obstructive Hypertrophic Cardiomyopathy

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What is obstructive hypertrophic cardiomyopathy? Obstructive hypertrophic cardiomyopathy is a genetic disorder where the walls of the heart and the septum between the right and left ventricles become thickened, obstructing blood flow out of the heart.

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Great 👍 But no mention of alcohol septal ablation as an alternative for surgical reduction of septal thickening. Also because the mode if inheritance is autosomal dominance, screening and monitoring of other family members is highly recommended.

PinkCougar
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read about cardiac myosin inhibitors: mavacamten or aficamten for ttt of HoCM

ahmedahm
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Cardiac magnetic resonance (CMR) can also be a useful diagnostic tool as transthoracic echocardiogram (TTE) cannot exclude other causes, e.g. Anderson-Fabry’s disease and cardiac amyloidosis.

Hypertrophic cardiomyopathy (HCM) has morphological markers seen on CMR images.
1. Apical-basal band/apicobasal bundle
2. LV myocardial crypts
3. LV apical aneurysm
4. LV thrombus
5. Right ventricular hypertrophy (RVH)
6. Left ventricular outflow tract (LVOT) obstruction

As for the phenotype of HCM on CMR images, there are also a variety of them.
1. Apical
2. Septal
3. Concentric
4. Mixed
5. Burn-out (Distinguished by late gadolinium enhancement (LGE) on CMR images)
6. Non-contiguous
7. Sigmoid

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