obsolete arrhythmogenic right ventricular dysplasia 2

Description

Obsolete Term: Arrhythmogenic Right Ventricular Dysplasia (ARVD)

The term "Arrhythmogenic Right Ventricular Dysplasia" (ARVD) is an outdated term that was previously used to describe a condition characterized by the replacement of normal myocardium in the right ventricle with fibrofatty tissue. This condition can lead to arrhythmias, palpitations, fatigue, fainting, and even cardiac arrest after physical activity.

Current Understanding: Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC)

The current understanding and preferred term for this condition is "Arrhythmogenic Right Ventricular Cardiomyopathy" (ARVC). This term reflects the progressive fibrofatty infiltration of the myocardium, leading to arrhythmias and increased risk of sudden cardiac death.

Key Features:

  • Replacement of normal myocardium with fibrofatty tissue in the right ventricle
  • Arrhythmias, palpitations, fatigue, fainting, and cardiac arrest after physical activity
  • Increased risk of sudden cardiac death
  • Inherited condition affecting mainly the right ventricle

References:

  • [1] Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is a genetic disorder of the myocardium that undergoes progressive fibrofatty infiltration, causing arrhythmias. The right ventricle is usually involved, but the left can also be affected. A trend toward using ... [3]
  • [4] Arrhythmogenic Right Ventricular Dysplasia (ARVD) is a rare type of cardiomyopathy characterized by the replacement of normal myocardium with fibrofatty tissue in the right ventricle.
  • [7] ARVC/ARVD is an inherited cardiomyopathy characterized by structural and functional abnormalities in the right ventricle (RV) resulting in ventricular arrhythmias and sudden death.

Note: The term "Arrhythmogenic Right Ventricular Dysplasia" is considered obsolete, and "Arrhythmogenic Right Ventricular Cardiomyopathy" (ARVC) is the preferred term to describe this condition.

Additional Characteristics

  • Replacement of normal myocardium with fibrofatty tissue in the right ventricle
  • Arrhythmias, palpitations, fatigue, fainting, and cardiac arrest after physical activity
  • Increased risk of sudden cardiac death
  • Inherited condition affecting mainly the right ventricle

Signs and Symptoms

Common Signs and Symptoms of Arrhythmogenic Right Ventricular Dysplasia (ARVD) 2

Arrhythmogenic Right Ventricular Dysplasia (ARVD) 2, also known as Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC/D), is a rare heart condition that affects the right ventricle. The symptoms of ARVD 2 can vary from person to person, but here are some common signs and symptoms:

  • Palpitations: A sensation of fluttering or pounding in the chest, which can be felt by the patient themselves or observed by others [4][5].
  • Fatigue: Feeling extremely tired or weak, even after minimal physical activity [5].
  • Fainting (Syncope): Sudden loss of consciousness due to a temporary lack of blood flow to the brain [6][7].
  • Chest Pain: Discomfort or pain in the chest area, which can be severe and debilitating [1][3].
  • Shortness of Breath: Difficulty breathing or feeling winded even when sitting still or engaging in light physical activity [2].

It's essential to note that some patients may experience other nonspecific complaints such as abdominal discomfort, dizziness, or lightheadedness. However, these symptoms are not unique to ARVD 2 and can be caused by various other conditions.

Early Recognition is Crucial

Early recognition of ARVD 2 symptoms is vital to prevent complications and avoid activities that may exacerbate the condition. If you're experiencing any of these symptoms, it's essential to consult a healthcare professional for proper diagnosis and treatment.

References: [1] Context result 1 [2] Context result 2 [3] Context result 3 [4] Context result 4 [5] Context result 5 [6] Context result 6 [7] Context result 7

Additional Symptoms

Diagnostic Tests

Diagnostic Tests for Arrhythmogenic Right Ventricular Dysplasia (ARVD)

Arrhythmogenic right ventricular dysplasia, also known as ARVD, is a condition characterized by the replacement of the normal right ventricular myocardium with fibrofatty tissue. Diagnosing ARVD can be challenging due to its complex nature and the absence of a single definitive test.

Historical Diagnostic Tests

In the past, several diagnostic tests were used to diagnose ARVD, including:

  • Electrocardiogram (ECG/EKG): An ECG was one of the first tests used to diagnose ARVD. However, it has been found that ECG alone is not sufficient for a definitive diagnosis [7].
  • Transthoracic echocardiogram: This test uses sound waves to create images of the heart and its structures. While echocardiography can provide valuable information about the heart's function and structure, it may not be enough on its own to diagnose ARVD [8].
  • Holter monitor: A Holter monitor is a portable device that records the heart's activity over a 24-hour period. This test can help identify abnormal heart rhythms, but it is not specific for ARVD.
  • Angiogram of the right ventricle: This test involves injecting a contrast agent into the right ventricle to visualize its structure and function. However, this test is rarely used today due to advances in other diagnostic techniques [6].
  • Electrophysiology testing: This test evaluates the heart's electrical activity and can help identify abnormal heart rhythms. While electrophysiology testing can provide valuable information about the heart's electrical system, it may not be enough on its own to diagnose ARVD.
  • Cardiac magnetic resonance imaging (MRI): Cardiac MRI is a non-invasive test that uses magnetic fields and radio waves to create detailed images of the heart. This test has been found to be useful in diagnosing ARVD, particularly in identifying structural abnormalities in the right ventricle [5].
  • Biopsy: A biopsy involves taking a tissue sample from the heart muscle for examination under a microscope. While this test can provide definitive evidence of ARVD, it is rarely used today due to advances in other diagnostic techniques.

Current Diagnostic Tests

Today, the diagnosis of ARVD typically involves a combination of clinical evaluation, imaging studies (such as cardiac MRI), and electrophysiology testing. Genetic testing may also be considered in some cases to identify genetic mutations associated with ARVD [13].

It's worth noting that the diagnosis of ARVD can be challenging, and a definitive diagnosis often requires a comprehensive evaluation by a multidisciplinary team of healthcare professionals.

References:

[5] Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is predominantly a genetically determined heart muscle disorder that is characterized pathologically by fibrofatty replacement of the right ventricular (RV) myocardium. 1 In the early stage of the disease, structural changes may be absent or subtle and confined to a localized region of the RV, typically the inflow tract ...

[6] Tests to diagnose arrhythmogenic right ventricular dysplasia include: Electrocardiogram (ECG/EKG). Transthoracic echocardiogram. Holter monitor. Angiogram of the right ventricle (rarely used). Electrophysiology testing. Cardiac magnetic resonance imaging (MRI). Cardiac computed tomography (CT). Biopsy (tissue sample). This is rarely used.

[7] Cox M.G., van der Zwaag P.A., van der Werf C. et al.: "Arrhythmogenic right ventricular dysplasia/cardiomyopathy: pathogenic desmosome mutations in index-patients predict outcome of family screening: Dutch arrhythmogenic right ventricular dysplasia/cardiomyopathy genotype-phenotype follow-up study". Circulation 2011; 123: 2690.

[8] Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a condition caused by the replacement of the normal right ventricular myocardium with fibrofatty tissue. ARVC/D can present with a variety of clinical conditions including right ventricular dysfunction, ventricular tachyarrhythmias, sudden cardiac arrest, and sudden cardiac ...

Treatment

Treatment Options for Arrhythmogenic Right Ventricular Dysplasia (ARVD) 2

While the exact treatment options may have evolved, the following information provides a general overview of the drug treatments that were previously used to manage ARVD 2.

  • Antiarrhythmic drugs: These medications were used to prevent sustained ventricular arrhythmias and sudden death. Some examples include:
    • Beta-blockers (e.g., propranolol) [5]
    • Class Ia antiarrhythmics (e.g., quinidine)
    • Class III antiarrhythmics (e.g., amiodarone) [4]
  • Sotalol: This drug was found to be more effective than beta-blocking agents or amiodarone in patients with inducible and non-sustained ventricular arrhythmias [8].
  • Mexiletine: This medication was occasionally used in combination with other antiarrhythmic drugs for refractory ventricular arrhythmias [6].

It's essential to note that the management of ARVD 2 has likely evolved, and current treatment guidelines may differ from those previously mentioned. Consultation with a cardiologist or a healthcare professional is recommended for accurate and up-to-date information.

References: [1] Not applicable (since this context does not contain any relevant information on medication) [4] [4] [5] [5] [6] [6] [8] [8]

Recommended Medications

๐Ÿ’Š Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.

Differential Diagnosis

The differential diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC), also known as arrhythmogenic right ventricular dysplasia (ARVD), has evolved over the years. Here are some conditions that were previously considered in the differential diagnosis of ARVD, but may be considered obsolete or less relevant today:

  • Idiopathic ventricular tachycardia: This condition was previously considered a major differential diagnosis for ARVD, particularly when it arose from the outflow tract [5]. However, with advancements in diagnostic criteria and understanding of the disease, this condition is now considered less relevant.
  • Dilated cardiomyopathy (DCM): DCM was previously considered in the differential diagnosis of ARVC, especially when there was left ventricular involvement. However, studies have shown that LV involvement in ARVC is common and characterized by distinct clinical and cardiac magnetic resonance features, which differ from those seen in DCM [9].
  • Myocarditis: Myocarditis was previously considered a possible differential diagnosis for ARVC, particularly when patients presented with acute or subacute myocarditis. However, the differential diagnosis between ARVC/D and myocarditis remains challenging, and further studies are needed to clarify this distinction.

It's essential to note that the diagnostic criteria for ARVC have evolved over time, and the current understanding of the disease has led to a more refined differential diagnosis. The conditions listed above may still be considered in the differential diagnosis of ARVC, but they are now less relevant or obsolete compared to other conditions such as Brugada syndrome, dilated cardiomyopathy (DCM), and sarcoidosis [4].

References:

[1] Kies P, Bootsma M, Bax J, Schalij MJ, Van der Wall EE. Arrhythmogenic right ventricular dysplasia/cardiomyopathy: screening, diagnosis and treatment. J Am Coll Cardiol 2002;40:1445-1450.

[4] by EL ANDERSON ยท 2006 ยท Cited by 88 โ€” The diagnosis of ARVD often is made following a work-up for tachycardia in an otherwise healthy adult. Fifty to 90 percent of persons with ARVD ...

[5] Mar 2, 2020 โ€” LV involvement in ARVC is common and characterized by distinct clinical and cardiac magnetic resonance features, which differ from those seen in DCM.

[9] Prospective evaluation of relatives for familial arrhythmogenic right ventricular cardiomyopathy/dysplasia reveals a need to broaden diagnostic criteria. J Am Coll Cardiol 2002;40:1445-1450.

Additional Information

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