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Arrhythmogenic Left Ventricular Cardiomyopathy

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Arrhythmogenic left ventricular cardiomyopathy is a genetic syndrome that can cause sudden cardiac death.

Medically reviewed by

Dr. Yash Kathuria

Published At November 27, 2023
Reviewed AtNovember 27, 2023

Introduction

Globally, the disease that causes a significant health burden is heart disease. Among cardiovascular diseases, cardiomyopathies are a diverse group of heart disorders. Arrhythmogenic left ventricular cardiomyopathy is one subtype of cardiomyopathy. The person with the condition is more susceptible to sudden cardiac death. Heart muscle disease affects athletes, adolescents, and young populations. Around 87 % of people are examined with the heart condition, out of which 70 percent are diagnosed with arrhythmogenic right ventricular cardiomyopathy and 17 % with arrhythmogenic left ventricular cardiomyopathy. The condition can be due to genetic mutations. Imaging modalities can help the healthcare provider in diagnosing the condition. The condition is rare, but some cases have not been reported due to its rarity. The article discusses arrhythmogenic left ventricular cardiomyopathy, its causes, symptoms, diagnosis, and treatment.

What Is Arrhythmogenic Cardiomyopathy?

Arrhythmogenic cardiomyopathy is a heart disease that affects the heart muscles. The heart has two ventricles and two articles. The lower chambers are ventricles that are primarily affected. When the fibrous or fatty tissue replaces the affected heart muscle, the situation will disrupt the heart's electrical system. This will lead to abnormal heart rhythm. The other symptoms include fainting, dizziness, and palpitations.

What Is Arrhythmogenic Left Ventricular Cardiomyopathy?

Arrhythmogenic left ventricular cardiomyopathy (ALVC) is a subtype of arrhythmogenic cardiomyopathy. The left ventricle is the heart's left chamber, which is affected by the condition. The normal electrical heart system gets disrupted due to the replacement of the heart muscle with fatty tissue. This will lead to several heart symptoms, which may cause life-threatening conditions.

What Is the Difference Between Arrhythmogenic Left and Right Ventricular Cardiomyopathy?

The main difference between arrhythmogenic left and right ventricular cardiomyopathy is the involvement of the side of the ventricle. In ALVC, the affected ventricle is the left one, and the heart muscle changes lead to an abnormal electrical system on the left ventricle. In arrhythmogenic right ventricular cardiomyopathy (ARVC) the affected ventricle is the right one, leading to disruption of the electrical system on the right ventricle. Both conditions are challenging to diagnose, and specific tests are required to detect the conditions.

What Are the Causes of Arrhythmogenic Left Ventricular Cardiomyopathy?

The arrhythmogenic left ventricular cardiomyopathy is a hereditary condition that tends to pass from one family member to another. The inherited gene mutation is the leading cause of the development of the condition. There is a fifty percent chance of an individual inheriting one copy of gene mutation from the affected parent. The condition will affect the genes involved in cell adhesion in the heart muscle. These cells maintain the electrical system of the heart.

Gene mutations can disrupt the cell-to-cell connection within the heart. Due to disruption, fatty tissue replaces the normal or healthy heart muscle. The other causes of the condition are inflammation, viral infection, and environmental factors. The point to be noted is that not all individuals with gene mutations linked to ALVC will show symptoms. There is a need for further research to know the exact cause of the condition.

What Are the Signs and Symptoms of Arrhythmogenic Left Ventricular Cardiomyopathy?

The person with the condition may not show any symptoms. Sometimes, the symptoms are discovered on regular screening due to other medical conditions. If an individual experiences symptoms, then they include:

  • An individual with the condition may experience an abnormal or irregular heartbeat, which might be rapid or slow. The condition is called palpitations.

  • The second symptom includes fainting or loss of consciousness. This can be when the blood flow to the brain gets affected due to arrhythmia. The condition is called syncope.

  • The affected individual may feel a dull pain in the chest region or pressure in the chest area.

  • When an individual is in a lying flat position, the symptom includes difficulty breathing or shortness of breath. The symptoms can also be experienced during any physical activity.

  • There can be swelling in the feet, legs, or ankles. The condition is called edema. This will happen when more fluid is accumulated in the affected area.

  • The affected person will feel persistent tiredness and lack of energy. The condition is called fatigue.

  • Discomfort.

  • The patient will also experience abnormal heart rhythms. The other symptoms include ventricular tachycardia and ventricular fibrillation.

  • The other symptoms include dizziness, weakness, nausea, vomiting, heart failure, and sudden cardiac death.

How Is Arrhythmogenic Left Ventricular Cardiomyopathy Diagnosed?

Due to the challenge in diagnosing ALVC, a multi-step process is required to diagnose the condition. The steps include clinical assessment, genetic testing, imaging tests, and family history. The diagnostic tests are as follows:

  • Clinical Evaluation: The healthcare provider will take a detailed medical history and physically examine the patient. They will also ask the patient about the symptoms, any family history of cardiac conditions, or other medical conditions from which the patient is suffering.

  • Electrocardiography (ECG): The healthcare provider may advise an ECG to check electrical heart activity and irregular heart rhythms.

  • Echocardiography: The doctor will suggest this test to see the heart's structure and function. The affected ventricle shape and size will be visualized.

  • Genetic Testing: The affected gene mutations responsible for the development of ALVC will be detected using genetic testing.

  • Cardiac Magnetic Resonance Testing (MRI): The test will determine the myocardial tissue and fibrofatty tissue replacement.

How Is Arrhythmogenic Left Ventricular Cardiomyopathy Treated?

The treatment plan that will reduce the condition severity and associated risk factors is as follows:

  • Lifestyle Changes: The patient should adopt a healthy lifestyle to manage the condition. The changes include regular physical activity, such as walking, jogging, and cycling. The other changes include maintaining a healthy diet, managing stress, and limiting alcohol consumption.

  • Medications: The healthcare provider may prescribe antiarrhythmic drugs, beta-blockers, and angiotensin-converting enzyme inhibitors, which will help treat ALVC.

  • Heart Failure Management: Diuretics and aldosterone antagonists can manage the condition's symptoms. The doctor may advise heart transplantation in severe cases.

  • Genetic Counseling: The patient and family members suffering from the condition should be counseled because the condition is transferred from parents to their children.

Conclusion:

In conclusion, the hereditary condition in which the left ventricle is affected leads to palpitations, syncope, unconscious state, and dull pain in the chest region. Clinical assessment, imaging tests, and genetic testing will diagnose the condition. The management strategies include lifestyle changes and medications. Regular monitoring of the patient is required to adjust treatment plans. The advancement in the research of ALVC is efficient for better management.

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Dr. Yash Kathuria
Dr. Yash Kathuria

Family Physician

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