Arrythmogenic Right Ventricular Dysplasia Cardiomyoptahy (Risk Factors…
Arrythmogenic Right Ventricular Dysplasia Cardiomyoptahy
Nonpharmacological treatment, including catheter ablation and implantable cardioverter–defibrillator use, is usually reserved for patients with ventricular arrhythmias in whom drug therapy is ineffective
If these methods of treatment are also ineffective, the patient may be eligible for a
A healthy, disease-free, donor heart is taken from a deceased patient and swapped with the diseased heart, in order for the patient to survive.
This isn't believed to be necessary for many ARVD patients.Most people who die from Arrhthmogenic Right Ventricular Dysplasia do so because they are not diagnosed, monitored, and treated correctly.
Most patients who are monitored and treated appropriately have a relatively good outcome.
This is a small device that's placed in the chest or abdomen which is used to help treat arrhythmias
It works by detecting irregular heart rhythms and sending electrical pulses to correct them. The device has wires that connect to parts of the heart and carry electrical signals back to the implantable cardioverter-defibrillator where the heart rhythms are monitored.
This procedure uses thin and flexible wires (catheters) to selectively destroy parts of the heart that are responsible for causing heart rhythm problems. The wires are usually inserted into a vein in the neck or groin regions, and are then threaded up and into the heart muscle.
Management of each patient suffering from ARVD is different and personalized depending on what their body responds to.
There are two main goals of treating ARVD
Reduce the frequency and severity of ventricular arrhythmias
Prevent or limit the worsening of ventricular function and heart failure
Pharmacologic treatment is usually the first choice.
Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C) is a heart muscle disease characterized by peculiar right and left ventricular involvement and electrical instability that causes life-threatening ventricular arrhythmias and potentially sudden death.
ARVD results in fibrofatty replacement of the right ventricle, as well as the subepicardial region of the left ventricle.
Originally it was believed that ARVD begins in the right ventricle and progresses to biventricular involvement and potentially heart failure. However, amination of the heart in autopsy cases have established that most patients with ARVC have biventricular involvement, and have found that left ventricular involvement is typical from the onset of the disease.
The left ventricular involvement is typically subepicardial, and the changes are related more to fibrosis than to fat or fibrofatty infiltration, although at times the changes are probably similar to those seen in the right ventricle.
Due to the right ventricle being relatively thin, the subepicardial nature of disease is difficult to appreciate or determine in imaging studies and has only infrequently been investigated at autopsy. Atrial involvement appears not to have been adequately investigated.
This usually leads to progressive right ventricular dilatation and dysfunction.
This type of cardiomyopathy is often familial. Thirty to fifty percent of all cases have a family history of the disease.
Although an autosomal-recessive pattern has also been reported, the most common pattern of inheritance is autosomal-dominant. Linkage analysis has located the genetic abnormality on chromosomes 1, 2, 3, and 14 for the dominant form and on chromosome 17 for the recessive variant of the disease.
Other causes of ARVD are unknown and are described as 'idiopathic'.
Sporadic cases are the most common.
Research teams are still investigating other causes.
There's conflicting evidence on other causes of ARVD.
Diagnosing Arrhythmogenic Right Ventricular Dysplasia (ARVD) at early stages is described as a 'clinical challenge' as it is difficult in patients with minimal right ventricle abnormalities at echocardiographic or angiographic examinations. It can be difficult to determine the difference between ARVD and Right Ventricular outflow-tract tachycardia, which is a usually benign and nonfamilial arrhythmic condition.
Can be diagnosed through past medical history, family medical history, physical examination, and tests.
These tests can include:
Cardiac CT scan
is an ultrasound test that is turned into moving pictures of the heart. The test uses high-pitched sound waves that are sent through a device called a transducer. The device picks up echoes of the sound waves as they bounce off the different parts of the heart, and it turns the results into a moving video of the heart working.
Cardiac CT scan
is performed on patients with heart problems as it uses Xrays to create detailed images of the heart and blood vessels.
is used to get detailed pictures of the structures within the heart from different angles. It is used to detect or monitor cardiac disease and to evaluate the heart’s anatomy and function within diseased patients. This is vital in diagnosing ARVD as it shows a clear view of the heart, and if it has fibrofatty replacement of the right and left ventricle.
Endomyocardial biopsy is an invasive tool used to evaluate dialted cardiomyopathies.
It is also difficult as some patients may not be suspected to be suffering from ARVD, as they will present asymptomatic.
The average age for diagnosis regarding ARVD is 31 years of age
It is almost never suspected or diagnosed in young children and infants
A diagnosis of ARVD should usually only be made if other diseases that could cause Ventricular Tachycardia from the Right Ventricle have been ruled out.
ARVD will be suspected if the patient has these characteristics:
Abnormal function of the right ventricle (RV)
Fatty or fibrous-fatty infiltrating the right ventricle heart muscle (myocardium)
An abnormal ECG reading
Arrhythmias (especially looking for ventricular tachycardia, supraventricular tachycardia, or ventricular fibrillation, especially with exercise)
Family history of ARVD
Patients can (and are often reported as) presenting asymptomatic.
Sudden death could be first symptom for some patients
This is due to the fact that ARVD is commonly not diagnosed (or misdiagnosed), and without any treatment, patients do not have a good life expectancy.
Arrhythmias of right ventricular region
ECG depolarization/re-polarization changes
Dizziness, light headed, fainting
Paramedics should be suspicious of electrocardiographic abnormalities such as
inverted T waves
premature ventricular complex
as these are frequently signs of Arrhythmogenic Right Ventricular Dysplasia.
These signs and symptoms reflect something going wrong in the heart and circulatory system and thus should immediately spark a response from paramedics and doctors.
Patients may complain of chest pain/discomfort.
They may also have hypotension/hypertension, diaphoresis, tachy or bradycardia, and may potentially be in respiratory distress.
ARVD patients are usually diagnosed around 30 years of age, and typically present symptoms between the second and fourth decade of life.
Typically, one of the first recorded signs of ARVD is Ventricular Tachycardia originating from the right ventricle.
Management of ARVD patients focuses on adequate circulation and respiration rate.
Adrenaline (Epipinephrine), Beta Blockers and other drugs may be required in extreme cases.
Potential treatment options may include one or more of the following options:
IPPV / CPAP
Notify and transport to hospital as necessary
Doctors will reassess and determin the best long-term treatment option for the patient.
This type of cardiomyopathy is believed to have a strong influence by genetics
Previous heart problems
The link between exercise/physical exertion and ARVD is not clear, however in some cases, exercise can be an immediate trigger for arrhythmias.
Studies have found that young men who are avid athletes often are more susceptible to ARVD.
However, most male athletes who have suffered from ARVD have acquired it through genetic mutations. Therefore, exercise may not be the cause of someones ARVD, it may just aggravate it. However, the link between exercise and ARVD is unclear.
Stimulants have been found to exacerbate ARVD symptoms and arrhythmias. Stimulants that could cause harm in ARVD patients include; nicotine, caffine, and some pharmacological stimulants found in cold and flu mediciine.
Drug therapy is an effective treatment option for some ARVD patients. Usually, the drugs which are used include:
β-blockers work by blocking the effects of epinephrine (adrenaline) to slow down the patients heart rate.
According to the Queensland Ambulance Service, Amiodarone reduces conduction across cardiac tissue which is especially useful when treating ARVD. It also prolongs the duration of the action potential and therefore also prolongs the refractory period of atrial, ventricular, and nodal tissues.
Flecainide is an antiarrhythmic. It is used for treating and preventing various types of irregular heartbeat that lead to life-threatening heart rhythm disturbances. It works by stabilizing the heart rhythm when the heart is beating too fast or in an irregular rhythm.
Medicines used to treat ARVD include beta blockers or antiarrhythmic agents which are used to help lessen the frequency and severity of arrhythmias.
The prevalence and incidence of Arrhythmogenic Right Ventricular Dysplasia is uncertain
However, with 80% of suffers being aged under 40 years old,it is described as a younger people disease.
Most sufferes are diagnosed around 31 years of age.
ARVD is almost never suspected or diagnosed in infancy or childhood. It is described as a 'clinical challange' to diagnose.
After hypertrophic heart disease, ARVD is the leading cause of sudden cardiac death in young people.
ARVD has a high prevalence rate in young competetive athletes. It has been found that approximately 5-10% of competitive and Top Level Athletes (TLA) suffer from ARVD.
The prevalence in the general population is belived to be between 1:2000 to 1:5000.
Although, this could be higher due to the number of undiagnosed or misdiagnosed cases.
Although both men and women suffer from ARVD, the ratio of men to women ARVD patients is about 3:1.