Hypertrophic cardiomyopathy (HCM) disease (Sudden cardiac death)



Last reviewed on: 16th October, 2020

Hypertrophic cardiomyopathy (HCM) is a prime disease of the myocardium (the muscle of the heart) in which a portion of the myocardium is thickened without any noticeable cause, making functional weakening of the cardiac muscle. It is a foremost cause of sudden cardiac death in young athletes. The occurrence of hypertrophic cardiomyopathy is a significant cause of sudden unpredicted cardiac death in any age group and as a cause of deactivating cardiac symptoms. Younger people are more severe form of hypertrophic cardiomyopathy than older. 

Hypertrophic cardiomyopathy is normally symptomless till abrupt cardiac death, and for this reason some recommend routinely screening certain populations for this disease 

A cardiomyopathy is a disease that upsets the muscle of the heart. The cardiac contractile cells (myocytes) in the heart increase in size, which evolve in the thickening of the heart muscle. The normal arrangement of muscle cells is disrupted, a phenomenon known as myocardial disarray. HCM also causes interruptions of the electrical functions of the heart. 

HCM is normally due to alteration in one of nine sarcomeric genes that results in a mutated protein in the sarcomere, the primary component of the myocyte. These are mostly single-point missense mutations in the genes for beta-myosin heavy chain (MHC), myosin-binding protein C, cardiac troponinT, or tropomyosin. These genes transformations cause myofibril and myocyte structural deformities and possible deficits in force generation. 

SYMPTOMS AND SIGNS
Signs and symptoms of hypertrophic cardiomyopathy may comprise one or more of the following:
  • Shortness of breath, specifically during exercise
  • Chest pain, specifically during exercise
  • Collapsing, especially during or just after exercise or exertion
  • Feeling of rapid, fluttering or pounding heartbeats
  • Low sound of the heart, which a doctor might detect while listening to your heart
When to see a doctor ?
Numeral conditions can cause shortness of breath and heart palpitations. It's essential to get a quick, accurate diagnosis and appropriate care. See your doctor if you experience any symptoms related with hypertrophic cardiomyopathy. Checkout the following symptoms; 
  • Fast or uneven heartbeat
  • Difficulty inhalation
  • Chest discomfort
CAUSES
Hypertrophic cardiomyopathy is typically triggered by abnormal genes that cause the heart muscle to develop abnormally thick. People with hypertrophic cardiomyopathy also have an abnormal organization of heart muscle cells, a disorder known as myofiber disarray. This disarray can result to arrhythmia in some people. 

The severity of hypertrophic cardiomyopathy differs widely. Most people with hypertrophic cardiomyopathy have a form of the disease in which the wall (septum) among the two bottom chambers of the heart (ventricles) becomes inflamed and hinders blood flow out of the heart. This disorder is sometimes called obstructive hypertrophic cardiomyopathy. 

Occasionally hypertrophic cardiomyopathy arises without significant blocking of blood flow. Though, the heart's main pumping chamber (left ventricle) may become stiff, decreasing the amount of blood the ventricle can confine and the amount pumped out to the body with each heartbeat. This situation is sometimes called non-obstructive hypertrophic cardiomyopathy. 

Risk factors
Hypertrophic cardiomyopathy is normally inherited. Children of a close relative with hypertrophic cardiomyopathy will inherit the genetic Hypertrophic cardiomyopathy is usually triggered by abnormal genes (gene mutations) transformation for the disease. Close relatives (parents, children or siblings) of a person with hypertrophic cardiomyopathy should ask their doctors about diagnosing for the disease. 

TREATMENT 
Diagnosis
HCM is detected based on medical history (symptoms and family history), a physical examination, and echocardiogram results. Extra tests may comprise blood tests, electrocardiogram, chest X-ray, exercise stress test, cardiac catheterization, CT scan, and MRI. 

Treatment
Treatment of HCM depends on whether there is thinning in the path that blood takes to leave the heart (outflow tract); how the heart is running; and if arrhythmias are present. Treatment is targeted at avoiding symptoms and difficulties and comprises risk identification and regular follow-up, lifestyle changes, medications, and procedures as needed. 

PREVENTION
Since hypertrophic cardiomyopathy is inherited, it cannot be prevented. But it is essential to detect the condition as early as possible to guide treatment and avoid complications. 

Preventing sudden death
Implantation of a cardioverter-defibrillator has been revealed to help avoid sudden cardiac death, which arises in about 1 percent of people with hypertrophic cardiomyopathy. 

Since many people with hypertrophic cardiomyopathy don't recognize their condition, there are cases where the first sign of a problem is sudden cardiac death. These cases can occur in apparently healthy young people, including high school athletes and other young, active adults. News of these types of deaths generates comprehensible attention as they are so unexpected, but parents should be aware that these deaths are quite rare. 

Experts in heart abnormalities normally recommend that people with hypertrophic cardiomyopathy not take part in most competitive sports with the possible exclusion of some low intensity sports. Discuss precise recommendations with your cardiologist. 

The following are some players who have died from Hypertrophic cardiomyopathy (HCM);
  • Marc Vivian Foe (1st May, 1975 - 26th June, 2003)
  • Reggie Lewiss (21st November, 1965 - 27th July, 1993)
  • Benjamin Daniel Breedlove (8th August, 1993 - 25th December, 2011)
  • Mitchell James Cole (6th October, 1985 – 1st December, 2012)
  • Alexei Andreyevich Cherepanov (15th January, 1989 – 13th October, 2008) 

Posted by: Lusubilo A. Mwaijengo

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