What is the difference between Hereditary Chronic Myeloid leukemia (HCL) and Hypertrophic Cardiomyopathy (HCM)?

From the Guidelines

HCOM and HCM are the same condition, referring to Hypertrophic Cardiomyopathy, a genetic heart disease characterized by abnormal thickening of the heart muscle. This condition affects approximately 1 in 500 people and is often inherited as an autosomal dominant trait, meaning a child of an affected parent has a 50% chance of inheriting the condition 1. The management of HCM typically includes lifestyle modifications, such as avoiding intense competitive sports, and medications like beta-blockers or calcium channel blockers to improve heart function 1. In severe cases, procedures such as septal myectomy, alcohol septal ablation, or implantable cardioverter-defibrillator placement may be necessary 1. Regular cardiac monitoring is essential for patients with this condition to track disease progression and adjust treatment as needed 1. Some key points to consider in the management of HCM include:

• The use of genetic testing to identify affected family members and determine the risk of sudden cardiac death 1
• The importance of risk stratification to identify patients at high risk of sudden cardiac death and consider implantable cardioverter-defibrillator placement 1
• The role of lifestyle modifications, such as avoiding intense competitive sports, in reducing the risk of sudden cardiac death 1
• The use of medications, such as beta-blockers or calcium channel blockers, to improve heart function and reduce symptoms 1
• The consideration of procedures, such as septal myectomy or alcohol septal ablation, in patients with severe symptoms or obstructive hypertrophic cardiomyopathy 1.

From the Research

HCM vs HCOM: Understanding the Condition

• Hypertrophic cardiomyopathy (HCM) is a genetic cardiomyopathy that affects the heart muscle, leading to abnormal thickening of the heart wall [(2,3)].
• The condition can cause symptoms such as effort intolerance, heart failure, arrhythmias, and sudden cardiac death [(2,3)].
• HCM is the most common genetic cardiomyopathy, with an estimated prevalence of 0.6% in the general population 3.

Diagnosis and Treatment

• Diagnosis of HCM involves comprehensive and longitudinal assessment with echocardiography, ambulatory electrocardiography, stress testing, and cardiac magnetic resonance imaging 2.
• Treatment options for HCM include lifestyle modifications, familial screening with genetic counseling, pharmacotherapy, sudden cardiac death risk stratification, septal reduction therapy, and heart transplantation 3.
• Medications such as beta blockers and verapamil are commonly used to treat symptoms of HCM, with beta blockers being the first-line therapy 4.

Management and Outcomes

• The goal of management is to encourage and help patients with HCM lead an active, healthy lifestyle, including regular exercise 2.
• Patients with HCM who are treated according to modern guidelines can reasonably be expected to have normal longevity and good quality of life 2.
• Studies have shown that patients with HCM can achieve meaningful symptom relief and low mortality through stepped management, including the use of disopyramide and surgical myectomy 5.