Understanding Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) – Symptoms, Causes, and Treatment

ABSTRACT

Arrhythmogenic right ventricular cardiomyopathy is a form of heart disease that usually appears in adulthood. Heart disease includes many heart problems which means it includes heart and blood vessel disease. The heart is a muscular organ which pumps blood through the blood vessels of the circulatory system. In humans, the size of the heart is approximately the size of a fist which is located between the lungs in the middle compartment of the chest. Usually, the heart is divided into four chambers: upper left and right atria and lower left and right ventricles.

In a healthy heart, blood flows one way through the heart due to heart valves which prevent backflow. The walls of the heart are made up of three layers epicardium, myocardium and endocardium. The deoxygenated blood enters the heart through the right atrium from the superior and inferior vena cava and further passes to the right ventricle. Cardiovascular diseases are the most common cause of death. It is situated in the mediastinum at the level of T5-T8, a double membrane sc known as pericardium surrounds the heart and attaches to the mediastinum. An adult heart has a mass of 250-350 gm. The fibrous cardiac skeleton gives the structure to the heart and the cardiac skeleton provides the boundary in the heart’s electrical conduction system. In this article, we are going to discuss the causes, symptoms and ayurvedic management of ARVC (arrhythmogenic right ventricular cardiomyopathy).

INTRODUCTION

ARVC (Arrhythmogenic right ventricular cardiomyopathy) is a disorder of the myocardium which is the muscular layer of the heart. This condition causes the part of the myocardium to break down and increases the risk of abnormal heartbeat (arrhythmia) and causes sudden death. It is known as Arrhythmogenic cardiomyopathy (ACM), Arrhythmogenic right ventricular dysplasia (ARVD), which is inherited heart disease. It can be found in association with diffuse palmoplantar keratoderma and wooly hair which is an autosomal recessive condition known as Naxos disease. It is predominantly seen in males, 30-50% have familial distribution. The main clinical hallmark of this disease is ventricular arrhythmias which is arising from the right ventricle. The fatty fibrous tissue replaces the normal heart muscle which interrupts the normal signals in the heart and causes an irregular and life-threatening heart rhythm. Usually begins in the right ventricle (as the right ventricle stretches out and becomes thin and poorly contracts)  but after some time affects the left ventricle, so there is irregular heart pumping so blood backs up in the circulatory system which causes the fluid to build up in the soft tissues and sometimes in the lungs which further causes oedema and shortness of breath and results in heart failure. ARVC is a rare genetic disease which shows symptoms between the age of 10-50 years of age.

ARRHYTHMOGENIC RIGHT VENTRICULAR CARDIOMYOPATHY

PATHOGENESIS

The cause is unknown, but apoptosis (cell death) plays an important role. This disease starts in the subepicardial region and moves towards the endocardial surface which leads to transmural involvement.  There are 2 pathological patterns which are seen in ACM/ARVC- Fatty infiltration and fibro-fatty filtration.

  1. Fatty infiltration-
  2. It is confined to the right ventricle and involves a partial and near complete substitution of the myocardium with fatty tissue without thinning of the walls.
  3. There is evidence of myocyte degeneration and death is seen in 50% of fatty infiltration.
  4. Fibro Fatty Infiltration-
  5. It is the replacement of myocytes with fibrofatty tissues.
  6. Myocardial atrophy is due to injury and apoptosis which leads to thinning of the RV free wall.
  7. These areas are prone to aneurysm formation

STAGES OF ARRHYTHMOGENIC RIGHT VENTRICULAR DYSPLASIA

There are 3 stages of ARVC (Arrhythmogenic right ventricular dysplasia)-

  1. Concealed Stage- There are no symptoms of abnormal heart rhythm while exercising.
  2. Electrical Stage- There is a high risk of ventricular arrhythmias and risk of sudden cardiac death, ECG shows abnormal heart rhythm issues.
  3. Structural Stage- They are at even higher risk of abnormal ventricular rhythm and sudden cardiac death.  

CAUSES

  1. It results from a genetic defect. 60% of people have genetic mutations. Due to mutations in the desmosomes, genes impair the functions of desmosomes, without normal desmosomes, cells of the myocardium detach and die so heart muscles come under pressure. The damaged myocardium gets replaced with fat and scar tissue. As the abnormal build-up walls of the ventricle get stretched out it prevents the flow and leads to changes in arrhythmia.
  2. There are 2 patterns of inheritance for ARVC-
  3. Autosomal Dominant- when one of the parents has a genetic mutation, it is more prevalent in Italy and other geographic areas
  4. Autosomal Recessive- When both the parents have genetic mutations but show no symptoms. It can be seen in Naxos disease which causes the thickening of the skin.
  5. Viral and inflammatory myocarditis
  6. Birth abnormalities which affect the right ventricle

SYMPTOMS

  1. ARVC causes chest pain which seems like a heart attack
  2. Ventricular arrhythmias- The irregular heart rhythms start in the ventricles or the lower chamber of the heart, the most common rhythm is ventricular tachycardia. Almost 77% of people are in this condition.  
  3. Supraventricular arrhythmias are most often in atrial fibrillation
  4. Heart Palpitations- that is the fluttering of the chest which forms abnormal rhythms of the heart.
  5. Chest pain
  6. Sudden cardiac death
  7. Shortness of breath
  8.  Swelling in the legs, ankles and abdomen
  9.  Fainting
  10. Lightheadedness

DIAGNOSIS

  1. Electrocardiogram (ECG/EKG)
  2. A Signal averaged ECG (SAECG)
  3. Cardiac MRI
  4. Holter monitor
  5. Cardiac magnetic resonance imaging (MRI)
  6. Biopsy
  7. Cardiac Computed Tomography
  8. Transthoracic echocardiogram

DIFFERENTIAL DIAGNOSIS

  1. Congenital heart disease-
  2. Partial anomalous venous return
  3. Atrial septal defect
  4. Repaired tetralogy of Fallot
  5. Acquired heart disease-
  6. Tricuspid valve disease
  7. Pulmonary hypertension
  8. Right ventricular infarction
  9. Miscellaneous-
  10. Sarcoidosis
  11. Idiopathic RVOT tachycardia  

TREATMENT

  1. Antiarrhythmic drugs (amiodarone
  2. Blood pressure medications such as Beta blockers and diuretics
  3. Anticoagulant (Warfarin)
  4. Radiofrequency (catheter ablation)
  5. Implantable cardioverter defibrillator (ICD)
  6. Heart Transplant

AYURVEDIC MANAGEMENT

In ARVC (Arrhythmogenic right ventricular cardiomyopathy) this is due to the consumption of causative agents, the doshas get vitiated. These doshas contaminate the rasa dhatus (nutritional fluid in the circulation) and then enter the hridya (heart). On entering the heart through rasa dhatus, the doshas get troubled and cause hrid roga.

  1.  Due to imbalance  Vata doshas cause irregular heartbeat.
  2. As the pitta doshas get aggravated that is responsible for an increased heartbeat.
  3.  As the Kapha dosha gets imbalanced it results in a slow down of heartbeat.

NIDANA

  1. Consumption of too hot foods
  2. Excessive consumption of sour, bitter foods
  3. Excessive thinking, stress
  4. Excessive physical activity
  5. Administration of a strong enema
  6. Excessive indulgence in sexual activity
  7. Frequent vomiting
  8. Excessive formation of ama (toxins)
  9. Forcibly withholding the natural urges
  10. Fear
  11. Panic
  12. Emaciation

TYPES OF HRID ROGAS

Hrid rogas is of 5 types

  1. Vataja Hrid Rogas- heart disease is caused by vitiation of Vata
  2. Pittaja Hrid Rogas- Heart disease is caused by vitiation of pitta
  3. Kaphaja Hrid Rogas- Heart disease due to vitiation of Kapha
  4. Tridoshaja Hrid Rogas- This is caused by the vitiation of three doshas (Vata, Pitta, Kapha)
  5. Krimija Hrid Rogas- Heart disease is caused by worms, bacteria (infections)

1. Vataja Hridrogas – It causes

  • Expansion of heart
  • Churning type of pain
  • Tearing type of pain
  • Cutting type of pain

2. Pittaja Hridrogas – It causes

  • Severe thirst, sucking pain
  • Excessive tiredness
  • Excessive sweating
  • Dryness in the oral cavity
  • Sucking pain

3. Kaphaja Hridrogas – It causes

  • Heaviness in the body
  • Loss of appetite
  • Stiffness
  • Rigidity
  • Excessive salivation

4. Tridosha HridRogas

Whole doshas will be seen in tridosha hridrogas

5. Krimija Hrid Rogas

This is seen in patients who consume apathya ahara vihara, which manifests kleda. Experience the toda (pricking type of pain) in the heart region.

  • Nausea
  • Vomiting
  • Tastelessness
  • Excessive dryness in the body     

HERBS USEFUL IN ARVC (Arrhythmogenic right ventricular cardiomyopathy)

1. ARJUNA

Arjuna (Terminalia arjuna) helps to balance pitta and Kapha doshas and has a great effect on cardiac diseases so known as a cardiac tonic (strengthens the heart and is useful in angina and in high blood pressure). It is used in the treatment of aconite poisoning. Because its kshaya ras is useful in the healing of fractures. Helps to reduce fat and cholesterol levels, useful in chest injuries and in many bleeding disorders./p>

2. BRAHMI

Brahmi (Bacopa monnieri) helps to improve intelligence, acts as an excellent anti-ageing herb and has antioxidant properties. It acts as a nerve tonic which promotes the growth of the brain and helps to improve the coordination between the tongue and limbs. Useful in many psychiatric disorders which improve the quality of life and in many spleen disorders. It is also good for heart disorders so known for its cardiac tone property.

3. SHANKHPUSHPI

Shankhpushpi (Convolvulus pluricaulis) helps to improve intelligence and memory.  It is known for its natural detoxification property, improves strength, induces sleep and helps to improve the quality of hair and promotes hair growth. Shankhpushpi is known for its rasayan property. It helps to improve the complexion of the skin and is used in many skin disorders.

4. VACHA

Vacha (Acorus calamus) helps to balance Vata and Kapha doshas. Vacha helps to relieve the ama dosha so helpful in the digestion process and useful against many psychological imbalances and disorders. It is extensively used in the treatment of obesity which relieves syphilis and in Vata doshas imbalances. Helps to induce uterine contraction so it is useful in dysmenorrhea. Improves speech, memory and intelligence.

HERBAL REMEDIES BY PLANET AYURVEDA FOR ARVC (Arrhythmogenic right ventricular cardiomyopathy)

Planet ayurveda is an ayurvedic manufacturing company that makes all the formulations from its natural resources. They have many branches overseas. Planet ayurveda is a GMP certified company, all products made are free from any preservatives, colors and additional resins. Ingredients are free from any adulterations. Products cause no side effects but provide many health benefits to patients.

PRODUCTS LIST

  1. ARJUNA CAPSULES
  2. ARJUN TEA
  3. TOTAL HEART SUPPORT
  4. HRIDAYARNAVA RASA
  5. BRAHMI VATI 

PRODUCTS DESCRIPTION

1. Arjuna Capsules

Arjuna Capsules are single herbal capsules which are made by planet ayurveda using a single standardized extract of Arjuna (Terminalia arjuna). These capsules help to balance the tridoshas in the body, Help to support the healthy circulation of the heartwhich maintains healthy blood cells and healthy metabolism. Taking these capsules supports the healthy cholesterol level in the body.

Dosage-2 capsules twice daily with plain water after meals

2. ARJUN TEA

Arjuna Tea is a herbal tea formulation which is made by planet ayurveda using various ingredients such as Arjuna (Terminalia arjuna), Dalchini (Cinnamomum zeylanicum), Chhoti Elaichi (Elettaria cardamomum) and many more. This herbal tea is an effective remedy for maintaining heart and blood pressure. It is also helpful in high cholesterol, congestive heart failure and many heart problems. This tea is rich in antioxidants and provides support to the vascular system.

Dosage– 2 cups twice daily

3. Total Heart Support

Total heart support are polyherbal capsules made by planet Ayurveda using ingredients such as Arjuna (Terminalia arjuna), Shankhpushpi (Convolvulus pluricaulis) and many more. This capsule is helpful in balancing Vata and pitta doshas. Helpful in heart disease in the treatment of high cholesterol levels and in congestive heart failure. Due to weakness in heart muscles, there is breathlessness so it is an effective remedy for heart disorders.

Dosage– 2 capsules twice daily with plain water after meals.

4. Hridayarnava Rasa

Hridayarnava Rasa is a tablet formulation which is prepared by planet Ayurveda using ingredients such as Shuddh Parad (Purified mercury), Tamra Bhasma, Shuddh Gandhak and many more. This tablet is helpful in cardiac disorders which further cause breathlessness. Heaviness in the chest and in many heart-related problems. This helps to balance Kapha doshas.

Dosage– 1 tablet twice daily.

5. Brahmi Vati

Brahmi Vati is a classical ayurvedic formulation which is made by planet ayurveda in the form of tablet using ingredients such as Brahmi (Bacopa monnieri), Vacha (Acorus calamus), Swarna Makshika bhasma and many more. These tablets are useful in panic attacks and in maintaining normal blood pressure. It is used in sleeplessness, in chronic fever and anxiety.

Dosage- 1 tablet twice daily with lukewarm water.

CONCLUSION

Arrhythmogenic right ventricular cardiomyopathy is a form of heart disease that usually appears in adulthood. ARVC (Arrhythmogenic right ventricular cardiomyopathy) is a disorder of the myocardium which is the muscular layer of the heart. This condition causes the part of the myocardium to break down and increases the risk of abnormal heartbeat (arrhythmia) and causes sudden death.   ARVC is a rare genetic disease which shows symptoms between the age of 10-50 years of age. On entering the heart through rasa dhatus, the doshas get troubled and cause hrid roga. We discussed many herbal remedies by planet ayurveda which are really effective and cause no side effects.

Dr. Vikram Chauhan

http://www.drvikram.com

Dr. Vikram Chauhan (MD - Ayurveda) is the CEO and Founder of Planet Ayurveda Pvt. Ltd. He is Author of the Book "Ayurveda – God’s Manual For Healing". He is an Ayurveda Expert Serving People worldwide through all the Possible Mediums, Operating from Main Branch in Mohali, India. With his Vast Experience in Herbs and their Applied Uses, he is successfully treating Numerous Patients suffering from Various Ailments with the help of Purest Herbal Supplements, Diet, and Lifestyle, according to the Principles of Ayurveda. For More Details, visit www.PlanetAyurveda.com.

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