arrhythmogenic biventricular cardiomyopathy

Arrhythmogenic biventricular cardiomyopathy (ABVC) is a rare and serious heart condition that affects both ventricles, the lower chambers of the heart. It is characterized by a replacement of the normal myocardial tissue with fibrofatty tissue, leading to a disruption in the electrical conduction system of the heart.

Key Features:

• Biventricular Involvement: ABVC affects both the right and left ventricles, making it distinct from other forms of arrhythmogenic cardiomyopathy that primarily affect one ventricle.
• Fibrofatty Replacement: The normal myocardial tissue is replaced by fibrofatty tissue, leading to a disruption in the heart's electrical conduction system.
• Increased Risk of Arrhythmic Events: ABVC increases the risk of life-threatening arrhythmias, particularly in young patients and athletes.

Causes and Risk Factors:

• Genetic Defects: ABVC is caused by genetic defects that affect the desmosomes, areas on the surface of muscle cells that link them together.
• Intense Endurance Athletics: ABVC can also develop in intense endurance athletes, particularly those participating in sports that involve repetitive stress on the heart.

Symptoms and Complications:

• Sudden Cardiac Death: ABVC increases the risk of sudden cardiac death, particularly in young patients and athletes.
• Arrhythmic Events: Patients with ABVC are at an increased risk of life-threatening arrhythmias.
• Heart Failure: In advanced cases, ABVC can lead to heart failure.

References:

• [6] Arrhythmogenic cardiomyopathy is an inherited heart muscle disorder, predisposing to sudden cardiac death, particularly in young patients and athletes. Pathological features include loss of myocytes and fibrofatty replacement of right ventricular myocardium; biventricular involvement is often observed.
• [13] Arrhythmogenic cardiomyopathy (ACM) is a heart disease characterized by a fibrotic replacement of myocardial tissue and a consequent predisposition to ventricular arrhythmic events, especially in the young. ... With a multidisciplinary approach that includes the ...
• [15] Arrhythmogenic cardiomyopathy is an inherited heart muscle disorder, predisposing to sudden cardiac death, particularly in young patients and athletes. Pathological features include loss of myocytes and fibrofatty replacement of right ventricular myocardium; biventricular involvement is often observed.

Note: The above information is based on the search results provided and may not be an exhaustive description of arrhythmogenic biventricular cardiomyopathy.

Arrhythmogenic biventricular cardiomyopathy (ABC) is a rare heart condition that affects both ventricles, leading to abnormal heart rhythms and potentially life-threatening complications. The signs and symptoms of ABC can vary in severity and may include:

• Abnormal heart rhythms: Arrhythmias such as atrial fibrillation, premature ventricular contraction, ventricular tachycardia, and cardiac arrest are common in patients with ABC [1][2].
• Palpitations: Patients may experience occasional or rapid palpitations, which can be associated with lightheadedness and dizziness [3].
• Syncope: Fainting or a feeling of passing out due to reduced blood flow to the brain is another symptom of ABC [4].
• Shortness of breath: Difficulty breathing during physical activity or even at rest can indicate right ventricular failure, a complication of ABC [5].
• Chest pain: Chest pain, especially after physical activity or heavy meals, may be experienced by patients with ABC [6].
• Sudden cardiac death: This is the most severe and potentially life-threatening symptom of ABC, often occurring in young patients and athletes [7].

It's essential to note that symptoms may start subtly and progress over time. If you're experiencing any of these symptoms or have concerns about your heart health, consult a medical professional for proper evaluation and care.

References: [1] Arrhythmogenic Cardiomyopathy Symptoms · Arrhythmia, such as atrial fibrillation, premature ventricular contraction, ventricular tachycardia and ventricular ... [2] VT can cause weakness, nausea, vomiting and lightheadedness, as well as feelings of a racing or skipping heart. Syncope– Also referred to as fainting or a ... [3] occasional or rapid palpitations which may be associated with lightheadedness and dizziness · syncope (... [4] VT can cause weakness, nausea, vomiting and lightheadedness, as well as feelings of a racing or skipping heart. Syncope– Also referred to as fainting or a ... [5] Feb 21, 2024 — Shortness of breath or trouble breathing with activity or even at rest. · Chest pain, especially after physical activity or heavy meals. [6] Sep 15, 2017 — Arrhythmogenic cardiomyopathy is an inherited heart muscle disorder, predisposing to sudden cardiac death, particularly in young patients and athletes. [7] Symptoms include palpitations, syncope, and cardiac arrest, and with worsening disease, manifestations of right ventricular failure. Diagnosis includes ECG, ...

Arrhythmogenic biventricular cardiomyopathy (ABC) is a rare heart condition characterized by the replacement of myocardium with fibrofatty tissue, leading to ventricular tachycardia and sudden cardiac death. Diagnostic tests for ABC are crucial for early detection and management.

Diagnostic Criteria

The diagnosis of ABC is based on consensus criteria involving clinical and electrocardiographic factors, cardiac imaging, and genetic testing [5]. The proposed Padua diagnostic criteria encompass dominant-right, biventricular, and dominant-left ACM [8].

Cardiac Testing

Cardiac testing for ABC includes:

• Electrocardiogram (ECG): An ECG is a crucial initial test to identify arrhythmias and conduction abnormalities associated with ABC [2].
• Cardiac Imaging: Cardiac imaging using echocardiograms or cardiac magnetic resonance imaging (MRI) is essential for visualizing the extent of myocardial replacement and fibrofatty infiltration [2].
• 24-hour ECG: A 24-hour ECG can help identify arrhythmias and conduction abnormalities associated with ABC [2].

Histologic Diagnosis

The histologic diagnosis of AC can be made from endomyocardial biopsy during cardiac catheterization, at the time of ventricular assist device implantation, or as a standalone procedure [3]. This is particularly useful in cases where imaging findings are inconclusive.

Noninvasive Imaging

Noninvasive imaging is crucial for diagnosing and managing ABC. Despite advanced multimodality imaging tools, the diagnosis of ACM remains challenging due to the lack of a single sensitive and specific test [6].

Challenges in Diagnosis

The diagnosis of ABC is challenging due to the lack of a single gold-standard test and the need for a combination of criteria [7]. A 2019 International Expert report appraised these previous criteria, finding good accuracy for diagnosis of ARVC but a lack of sensitivity for identification of ACM [4].

References

[1] by D Corrado · 2021 · Cited by 110 — Criteria for diagnosis of arrhythmogenic cardiomyopathy (ACM) were first proposed in 1994 and revised in 2010 by a Task Force.

[2] Cardiac testing includes an electrocardiogram (ECG), cardiac imaging using echocardiograms or cardiac magnetic resonance imaging (MRI), and a 24-hour ECG ...

[3] by HJ Tadros · 2023 · Cited by 3 — The histologic diagnosis of AC can be made from endomyocardial biopsy during cardiac catheterization, at the time of ventricular assist device ...

[4] by D Corrado · 2024 · Cited by 43 — A 2019 International Expert report appraised these previous criteria, finding good accuracy for diagnosis of ARVC but a lack of sensitivity for identification ...

[5] Diagnosis is based on consensus criteria involving clinical and electrocardiographic factors, cardiac imaging, and genetic testing. First-degree family members ...

[6] by M Kodali · 2024 · Cited by 1 — Noninvasive imaging is crucial for diagnosing and managing arrhythmogenic cardiomyopathy. Despite advanced multimodality imaging tools, ...

[7] by F Graziano · 2022 · Cited by 34 — The diagnosis of ACM is challenging due to the lack of a single sensitive and specific test.

[8] by D Corrado · 2020 · Cited by 438 — The proposed Padua diagnostic criteria encompass dominant-right, biventricular and dominant-left ACM. ... International Experts. Arrhythmogenic ...

[9] by F Graziano · 2024 — Diagnosing ACM is challenging as there is no single gold-standard test and a combination of criteria is required. The first diagnostic criteria ...

Arrhythmogenic biventricular cardiomyopathy (ABC) is a rare and serious heart condition characterized by abnormal heart rhythms and structural changes in the heart muscle. When it comes to drug treatment, there are several options available.

Pharmacological Options

According to various studies [1], [4], pharmacological therapy for ABC includes:

• Antiarrhythmic agents: These medications help regulate abnormal heart rhythms. Examples include amiodarone and sotalol [6], [8].
• β-blockers: Beta-blockers, such as metoprolol or bisoprolol, can be used to slow the heart rate and reduce symptoms [3], [7].
• Heart failure drug therapy: In cases where ABC leads to heart failure, medications like ACE inhibitors or ARBs may be prescribed to improve heart function [1].

Most Effective Drug Therapy

Research suggests that amiodarone alone or in combination with a beta-blocker is the most effective drug therapy for preventing symptomatic ventricular tachycardia in patients with ABC [6]. However, it's essential to note that the use of these medications should be carefully considered and monitored by a healthcare professional due to potential side effects.

Other Treatment Options

In addition to medication, other treatment options for ABC may include:

• Implantable cardioverter-defibrillators (ICDs): ICDs can help regulate abnormal heart rhythms and prevent sudden cardiac death.
• Catheter ablation: This minimally invasive procedure involves using radiofrequency energy or cryoablation to destroy the abnormal electrical pathways in the heart.

It's essential for patients with ABC to work closely with their healthcare provider to determine the best treatment plan, which may involve a combination of these options.

Arrhythmogenic biventricular cardiomyopathy (ABC) is a rare heart condition characterized by the replacement of myocardial tissue with fibrofatty tissue, leading to ventricular arrhythmias and sudden cardiac death. The differential diagnosis of ABC involves considering various conditions that can mimic its phenotype.

Conditions that can mimic ABC:

• Primary arrhythmia conditions: These include conditions such as idiopathic ventricular tachycardia, Brugada syndrome, and long QT syndrome, which can present with similar electrocardiographic (ECG) abnormalities [7].
• Dilated cardiomyopathy (DCM): DCM is the most common condition requiring a differential diagnosis with ABC due to overlapping phenotypic features such as left ventricular systolic dysfunction and arrhythmias [9].
• Arrhythmogenic right ventricular cardiomyopathy (ARVC): ARVC can be misdiagnosed, especially in children, and can often be mistaken for myocarditis caused by a viral infection or ventricular tachycardia [3].
• Myocarditis: Myocarditis can present with similar symptoms to ABC, including ventricular arrhythmias and heart failure [3].

Diagnostic criteria:

The proposed Padua diagnostic criteria encompass dominant-right, biventricular, and dominant-left arrhythmogenic cardiomyopathy (ACM). These criteria include major and minor criteria that help in the diagnosis of ABC [10].

• Major criteria: These include histological evidence of fibrofatty replacement of myocardium, ventricular tachycardia or fibrillation, and a family history of ACM.
• Minor criteria: These include ECG abnormalities such as epsilon waves, T-wave inversion, and QRS prolongation.

References:

[3] Arrhythmogenic cardiomyopathy may be misdiagnosed, especially in children. It can often be mistaken for myocarditis caused by a viral infection, ventricular tachycardia, or other conditions [3].

[7] Conditions that can mimic the ABC phenotype (phenocopies) and enter into differential diagnosis of ABC include primary arrhythmia conditions such as idiopathic ventricular tachycardia, Brugada syndrome, and long QT syndrome [7].

[9] DCM is the most common condition requiring a differential diagnosis with ALVC due to overlapping phenotypic features such as LV systolic dysfunction and arrhythmias [9].

[10] The proposed Padua diagnostic criteria encompass dominant-right, biventricular, and dominant-left ACM. Abstract. The original designation of “arrhythmogenic right ventricular cardiomyopathy” was changed to arrhythmogenic cardiomyopathy (ACM) in 2017 [10].