Introduction
Arrhythmogenic right ventricular dysplasia or arrhythmogenic right ventricular cardiomyopathy is a rare disease that runs in the family. Arrhythmogenic right ventricular dysplasia or arrhythmogenic right ventricular cardiomyopathy is also referred to as ARVD or ARVC, respectively. Young and healthy individuals experience sudden cardiac arrest that leads to their death. The heart is divided into four different compartments. They are two chambers above and two chambers below. The upper two sections of the heart are called the right and left atrium, and the lower two sections are called the right and left ventricles. This condition arises chiefly from the right ventricle leading to arrhythmias in both ventricles. It has been recorded that approximately 1 in 1250 people are diagnosed with arrhythmogenic right ventricular dysplasia.
What Causes Arrhythmogenic Right Ventricular Dysplasia/ Cardiomyopathy?
Arrhythmogenic right ventricular dysplasia generally harms the right ventricle and may rarely involve the left ventricle. Arrhythmogenic right ventricular dysplasia occurs due to genetic mutation. The genes that code for desmosomal proteins are mutated. Desmosomal proteins help maintain the mechanical unification of the cells. They aid in gluing two cells together. Arrhythmogenic right ventricular dysplasia is more commonly observed in young athletes.
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Very rarely, a viral infection of the right ventricle of the heart or inflammation of the muscles of the heart can also cause arrhythmogenic right ventricular dysplasia/ cardiomyopathy.
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The heart muscles can have fatty deposition and infiltration that may, in turn, lead to arrhythmogenic right ventricular dysplasia/ cardiomyopathy.
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Inflammation of the heart muscles leading to loss of function is another cause of arrhythmogenic right ventricular dysplasia/ cardiomyopathy.
What Are the Signs and Symptoms of Arrhythmogenic Right Ventricular Dysplasia/ Cardiomyopathy?
Arrhythmogenic right ventricular dysplasia or cardiomyopathy is an inherited condition that will, by default, exist in the fetus. Sometimes, viral infections and even unidentified family disorders may lead to the signs and symptoms. This disease has manifestations in healthy and young adults who are active individuals.
The symptoms of arrhythmogenic right ventricular dysplasia are some of the following, as mentioned below.
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Abnormal heartbeat.
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Palpitations.
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Shortness of breath.
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Irregular heartbeats in the lower chambers of the heart.
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Episodes of syncope.
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Weakness.
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Lightheadedness.
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A feeling of a racing heartbeat.
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Dizziness.
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Swelling of the foot and ankles.
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A fluttering feeling in the heart.
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Ascites or fluid accumulation in the abdomen.
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Sudden cardiac death.
How to Diagnose Arrhythmogenic Right Ventricular Dysplasia/ Cardiomyopathy?
Arrhythmogenic right ventricular dysplasia/ cardiomyopathy usually goes undiagnosed. A physical examination, as well as a genetic examination, is very important. Alongside these two, a complete cardiac test is also necessary. An easy way to diagnose arrhythmogenic right ventricular dysplasia/ cardiomyopathy is by studying the patient's electrocardiograph. Any abnormalities or structural changes in the tissues of the heart can be immediately viewed via an electrocardiograph. Learning about the family’s disease history and if there is a presence of other conditions in the family tree is also vital. In severe cases of arrhythmogenic right ventricular dysplasia/ cardiomyopathy, a cardiac biopsy may be required, but this decision will be made after a thorough investigation by the health care professional and team. Below are the diagnostic tools that can help diagnose and determine arrhythmogenic right ventricular dysplasia.
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Electrocardiography.
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Genetic testing.
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Magnetic resonance imaging.
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Treadmill test.
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Stress test.
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Holter monitors.
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Echocardiograms.
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Signal average electrocardiogram.
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Cardiac computed tomography.
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Electrophysiology study.
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Right ventriculogram.
How to Treat Arrhythmogenic Right Ventricular Dysplasia/ Cardiomyopathy?
The approach to managing arrhythmic right ventricular dysplasia/ cardiomyopathy must always be interdisciplinary. There are different treatment modalities for arrhythmogenic right ventricular dysplasia/ cardiomyopathy. They are purely based on the patient’s conditions and clinical manifestations. Knowledge of medical history and any other present genetic mutation that may already be present will further help in prompt treatment. There are three essential management and treatment modalities for a patient suffering from arrhythmogenic right ventricular dysplasia/ cardiomyopathy- catheter ablation, implantable cardioverter defibrillator or ICD, and medication therapy.
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Medication therapy can be used to decrease the episodes and severity of any heart rhythm problem.
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Medications can change the electrical effects of the heart in two ways- directly and indirectly.
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Beta-blockers are safe medications that can decrease the rate of the heart, blood pressure, and adrenaline after-math.
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Implantable cardioverter-defibrillators are generally used for the treatment of patients who need continual heart monitoring.
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An implantable cardioverter-defibrillator is referred to as being “kicked in the chest” by a few patients due to the way they experience it.
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An Implantable cardioverter-defibrillator or ICD can work as a heart pacemaker to correct both slow heart rhythms and fast heart rhythms.
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Catheter ablation is used to treat the zones of the heart that cause irregular heart rhythms. These zones are then cauterized. Catheter ablation is an invasive procedure.
It should be noted that the primary line of treatment is avoiding the incidence of syncope, cardiac arrest, or sudden heart failure. Using medications and necessary treatment modalities, the risk factors and dangerously fatal symptoms must initially be corrected.
Conclusion
ARVC/ ARVD, elongated as arrhythmogenic right ventricular cardiomyopathy/dysplasia, is a form of a heart condition that generally seems to occur in youth and adulthood, especially amongst active, healthy people. It is a disease of the heart muscles or myocardium. This is an inherited condition that leads to the tearing and breaking of the myocardium, which leads to abnormal heart rhythms in the future. In the early stages, there are no symptoms, but the risk of sudden death remains, particularly during challenging exercises. Whenever any cardiovascular symptoms occur, it is wise to get a thorough check immediately. Prompt diagnosis and treatment can help give an excellent prognosis to patients with arrhythmogenic right ventricular dysplasia/ cardiomyopathy. Other than this, patients who have a family history of arrhythmogenic right ventricular dysplasia/ cardiomyopathy must limit vigorous heart exercises and strenuous athletic activities.