HCM is due to hereditary mutation of myocyte sarcomere .The molecular defects are located in myosin, tropomyosin, titin .Depending upon the protein involved the hypertrophy can be regional or localised.
Non obstructive types most often involve tropomyocin mutations. Obstructive types predominate with myosin mutations.
- Simple ASH
- Apical HCM
- Mid ventricular
Apical HCM can still be prognostically and practically important even though there is no hemodynamic impact.
- Arrhythmic risk persist(Any focal hypertrophy can be substrate for reentry due to slow conduction)
- More importantly apical HCM is the commonest myocardial condition mistaken for unstable angina and they wrongly enter the ACS protocol and might land up in cath lab tables as well !
Always remember high voltage qrs with deep T wave inversion (90 out of 100 times) is due to myocardial pathology not ischemic.
- Reassurance (First advice is, not to search for more information from the internet ! It may confuse them ! )
- Just follow up with yearly echocardiogram .Follow up the siblings too.
- Marriage counseling . (Not contraindicated )
- Holter monitoring or extended loop recording may be done to detect any sub-clinical of arrhythmias.
- Beta blockers are generally not indicated routinely may be given if family history of sudden death .