hypertrophic cardiomyopathy 8

Hypertrophic Cardiomyopathy (HCM) Description

Hypertrophic cardiomyopathy (HCM) is a genetic disorder that is characterized by left ventricular hypertrophy unexplained by secondary causes. This condition involves the thickening of the heart muscle, which can lead to obstruction of blood flow out of the heart.

Key Features:

• Left ventricular hypertrophy
• Thickening of the heart muscle
• Obstruction of blood flow out of the heart in approximately 60% of patients
• Asymmetric left ventricular hypertrophy, with enlarged myocytes in disarray

Prevalence: HCM affects approximately 1 in every 500 persons worldwide and about 750,000 Americans.

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Common Signs and Symptoms of Hypertrophic Cardiomyopathy

Hypertrophic cardiomyopathy (HCM) can cause a range of symptoms, which may vary in severity and frequency from person to person. According to various medical sources [8][9], some common signs and symptoms of HCM include:

• Difficulty breathing (shortness of breath): This is one of the most common complaints among people with HCM, especially during exertion or physical activity.
• Fatigue: Feeling tired or exhausted, even after minimal physical activity, can be a symptom of HCM.

It's essential to note that not everyone with HCM will experience symptoms, and some may only develop them over time [2]. If you're experiencing any of these symptoms, it's crucial to consult a healthcare provider for proper evaluation and treatment.

Diagnostic Tests for Hypertrophic Cardiomyopathy

Hypertrophic cardiomyopathy (HCM) can be diagnosed using various tests, including:

• Echocardiogram: This is the most common test used to diagnose HCM, as it will usually show the thickening of your heart walls [5].
• Electrocardiogram (ECG): An ECG may show abnormal electrical activity in the heart, which can be a clue to the possible diagnosis of HCM [7].
• Magnetic Resonance Imaging (MRI): MRI can provide detailed images of the heart and help diagnose HCM.
• Genetic testing: Genetic testing can identify genetic mutations that cause HCM. It is important for two reasons: patients can find out more about their own prognosis, and they learn if family members are also affected [6].
• Exercise test: An exercise test may be used to assess the heart's function during physical activity.
• Blood tests: Blood tests may be used to rule out other conditions that may be causing symptoms similar to HCM.

It is worth noting that a diagnosis of HCM is typically made based on a combination of these tests, as well as a thorough medical history and physical exam [4].

Medications for Hypertrophic Cardiomyopathy (HCM)

According to various sources, commonly prescribed medications for HCM include:

• Beta blockers [5]
• Disopyramide [7]
• Calcium channel blockers [5]
• Heart rhythm medications [5]
• Anticoagulants [5]

These medications can help manage symptoms and improve heart function in people with HCM. However, it's essential to note that the effectiveness of these medications may vary from person to person.

New Investigational Medications

Recently, a new investigational cardiac medication called mavacamten has been approved by the FDA for the treatment of obstructive hypertrophic cardiomyopathy (oHCM) [6, 8]. This medication has shown promise in improving heart function and reducing symptoms in people with HCM.

Other Treatment Options

In some cases, patients may require more invasive procedures such as heart surgery or catheter-based interventions if their obstruction is severe and not amenable to treatment with medications [11].

References:

[5] Commonly prescribed medications for HCM include beta blockers, disopyramide, calcium channel blockers, heart rhythm medications, and anticoagulants. [6] Research Highlights: Mavacamten, a new investigational cardiac medication, may improve heart function for people with thickened heart muscle leading to obstructed blood flow through the heart, a condition known as obstructive hypertrophic cardiomyopathy (oHCM). [7] Soor GS, Luk A, Ahn E, et al. Hypertrophic cardiomyopathy: current understanding and treatment objectives. J Clin Pathol. 2009 Mar. 62(3):226-35. [8] FDA has approved Camzyos (mavacamten) capsules to treat adults with symptomatic New York Heart Association (NYHA) class II-III obstructive hypertrophic cardiomyopathy (oHCM) to improve exercise capacity and symptoms. [11] If the obstruction is more significant and not amenable to treatment with medications, patients may need heart surgery or a catheter-based procedure.

Differential Diagnosis of Hypertrophic Cardiomyopathy

Hypertrophic cardiomyopathy (HCM) can be challenging to diagnose due to its complex symptomatology and potential for devastating consequences. When considering the differential diagnosis, it's essential to exclude other conditions that may cause similar symptoms.

According to various medical sources [8][10], the most common causes of acquired left ventricular hypertrophy that should be excluded when HCM is suspected are:

• Systemic hypertension: High blood pressure can lead to thickening of the heart muscle.
• Aortic stenosis: A narrowing of the aortic valve can cause the heart to work harder, leading to hypertrophy.

Additionally, other conditions such as physiologic remodeling due to fitness [10] and certain syndromes (e.g., Fabry disease) should also be considered in the differential diagnosis.

It's crucial for healthcare professionals to carefully evaluate patients with suspected HCM and rule out these potential causes to ensure accurate diagnosis and treatment.