Hypertrophic Cardiomyopathy

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1.    Introduction

Hypertrophic obstructive cardiomyopathy (HCM) is a serious medical condition in which a portion of the heart becomes thickened thus that affects the normal pumping ability of the heart (Maron, Bonow, Cannon III, Leon, & Epstein, 1987). The muscles of the heart becomes abnormally thick thus heart there is great pressure on the heart to pump blood to the entire body.


2.    Risk Factors

The following are the risk factors more closely associated with hypertrophic cardiomyopathy (Maron & Maron, 2013) are as follows:

  • Familial history of cardiomyopathy
  • Diabetes or other metabolic diseases
  • A disease that could lead to cardiomyopathy such as coronary heart disease
  • Unexplained syncope
  • Maximum left ventricular wall thickness

 


3.    Familial Hypertrophic Cardiomyopathy

It is an inherited heart condition in which muscular thickening occurs thus separating the right and the left ventricle of the heart. That obstructs normal blood flow thus affecting the pumping efficiency of the heart (Niimura et al., 1998). It is caused by mutations in several genes that includes MYBPC3, TNNT2, and TNNI3 genes.


4.    Symptoms

Signs and symptoms of cardiomyopathy includes either just one or more of the following:

  • Chest pain
  • Fainting
  • Shortness of breath
  • Palpitations
  • Irregular heartbeats


5.    Treatment and Prevention

Treatment for HCM (Wigle, Rakowski, Kimball, & Williams, 1995) includes;

  • Septal Myectomy

It is a surgical procedure in which small amount of the septal wall which got thickened is being removed by the surgeon to widen the tract for outflow from the left ventricle to the aorta and reduces pressure on the heart (Kim et al., 2016).

  • Ethanol Ablation

It is for those who can not be treated with septal myectomy and is carried out in a cardiac catheterization lab. For this purpose, a catheter is being inserted to inflate the artery and the thickened septal wall causing blockade is located by a contrast agent. After locating that point, alcohol is injected through a the catheter that kills the cell in contact (Talreja et al., 2004) thus causing the shrinkage of the septal wall back to the normal size and widening the passage for the normal flow of blood.

  • Implantable Cardioverter Defibrillators

ICD is recommended to the patients who are at risk of sudden cardiac arrest. In this procedure, a defibrillator is a small device which is connected to wires and then threaded to the heart through a vein (Maron et al., 2000). It monitors the rhythm of the heart and detects any abnormality thus then energy is delivered to the corresponding heart muscle to make the rhythm normal again.


By: Zoha Khan


References

 

  1. Kim, L. K., Swaminathan, R. V., Looser, P., Minutello, R. M., Wong, S. C., Bergman, G., . . . Singh, H. S. (2016). Hospital volume outcomes after septal myectomy and alcohol septal ablation for treatment of obstructive hypertrophic cardiomyopathy: US Nationwide Inpatient Database, 2003-2011. JAMA cardiology, 1(3), 324-332.
  2. Maron, B. J., Bonow, R. O., Cannon III, R. O., Leon, M. B., & Epstein, S. E. (1987). Hypertrophic cardiomyopathy. New England Journal of Medicine, 316(14), 844-852.
  3. Maron, B. J., & Maron, M. S. (2013). Hypertrophic cardiomyopathy. The Lancet, 381(9862), 242-255.
  4. Maron, B. J., Shen, W.-K., Link, M. S., Epstein, A. E., Almquist, A. K., Daubert, J. P., . . . Boriani, G. (2000). Efficacy of implantable cardioverter-defibrillators for the prevention of sudden death in patients with hypertrophic cardiomyopathy. New England Journal of Medicine, 342(6), 365-373.
  5. Niimura, H., Bachinski, L. L., Sangwatanaroj, S., Watkins, H., Chudley, A. E., McKenna, W., . . . Maron, B. J. (1998). Mutations in the gene for cardiac myosin-binding protein C and late-onset familial hypertrophic cardiomyopathy. New England Journal of Medicine, 338(18), 1248-1257.
  6. Talreja, D. R., Nishimura, R. A., Edwards, W. D., Valeti, U. S., Ommen, S. R., Tajik, A. J.,. . . Holmes, D. R. (2004). Alcohol septal ablation versus surgical septal myectomy: comparison of effects on atrioventricular conduction tissue. Journal of the American College of Cardiology, 44(12), 2329-2332.
  7. Wigle, E. D., Rakowski, H., Kimball, B. P., & Williams, W. G. (1995). Hypertrophic cardiomyopathy: clinical spectrum and treatment. Circulation, 92(7), 1680-1692.


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