Arrhythmogenic Right Ventricular Dysplasia ; Humans

Arrhythmogenic Right Ventricular Dysplasia ; diagnosis ; Humans ; Male

Arrhythmogenic Right Ventricular Dysplasia ; diagnosis ; Electrocardiography ; Humans ; Male ; Middle Aged

Arrhythmogenic Right Ventricular Dysplasia/genetics* ; Genotype ; Humans ; Phenotype ; Risk Assessment

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a kind of inherited cardio-myopathy, which is characterized by fibro-fatty replacement of right ventricular myocardium, leading to ventricular arrhythmia. However, rapid atrial arrhythmias are also common, including atrial fibrillation, atrial flutter and atrial tachycardia. Long term rapid atrial arrhythmia can lead to further deterioration of cardiac function. This case is a 51-year-old male. He was admitted to Department of Cardiology, Peking University Third Hospital with palpitation and fatigue after exercise. Electrocardiogram showed incessant atrial tachycardia. Echocardiography revealed dilation of all his four chambers, especially the right ventricle, with the left ventricular ejection fraction of 40% and the right ventricular hypokinesis. Cardiac magnetic resonance imaging found that the right ventricle was significantly enlarged, and the right ventricular aneurysm had formed; the right ventricular ejection fraction was as low as 8%, and the left ventricular ejection fraction was 35%. The patients met the diagnostic criteria of ARVC, and both left and right ventricles were involved. His physical activities were restricted, and metoprolol, digoxin, spironolactone and ramipril were given. Rivaroxaban was also given because atrial tachycardia could cause left atrial thrombosis and embolism. His atrial tachycardia converted spontaneously to normal sinus rhythm after these treatments. Since the patient had severe right ventricular dysfunction, frequent premature ventricular beats and non-sustained ventricular tachycardia on Holter monitoring, indicating a high risk of sudden death, implantable cardioverter defibrillator (ICD) was implanted. After discharge from hospital, physical activity restriction and the above medicines were continued. As rapid atrial arrhythmia could lead to inappropriate ICD shocks, amiodarone was added to prevent the recurrence of atrial tachycardia, and also control ventricular arrhythmia. After 6 months, echocardiography was repeated and showed that the left ventricle diameter was reduced significantly, and the left ventricular ejection fraction increased to 60%, while the size of right ventricle and right atrium decreased slightly. According to the clinical manifestations and outcomes, he was diagnosed with ARVC associated with arrhythmia induced cardiomyopathy. According to the results of his cardiac magnetic resonance imaging, the patient had left ventricular involvement caused by ARVC, and the persistent atrial tachycardia led to left ventricular systolic dysfunction.
Arrhythmogenic Right Ventricular Dysplasia/complications* ; Atrial Fibrillation ; Humans ; Male ; Middle Aged ; Stroke Volume ; Ventricular Function, Left ; Ventricular Function, Right

Arrhythmogenic right ventricular dysplasia is a rare but important cause of ventricular tachycardia in young patioents, which is of a left bundle branch block configuration, The common abnormality on routine ECG is inverted T-waves on precordial leads and magnetic resonance image shows the fatty replacement and/or extreme thinning of the myocardium of right ventricle. Histologically, this disease is characterized by patch replacement of right ventricular myocardium by adipose and fibrous tissue. We expierienced a case of arrythmogenic right ventricular dysplasia with ventricular tachycardia which was ablated successfully by radiofrequency catheter ablation.
Arrhythmogenic Right Ventricular Dysplasia ; Bundle-Branch Block ; Catheter Ablation ; Electrocardiography ; Heart Ventricles ; Myocardium ; Tachycardia, Ventricular

Arrhythmogenic right ventricular dysplasia is a rare heart muscle disorder of unknown cause that primarily involves the right ventricle. It is characterized pathologically by fibrofatty replacement of the right ventricular myocardium. Clinical manifestations include structural and functional abnormalities of the right ventricle, electrocardiographic depolarization/repolarization changes, and presentation with sudden death or ventricular arrhythmias of right ventricular origin. It is one of the important causes of the ventricular arrhythmia or sudden death among apparently healthy young people. We report a case of arrhythmogenic right ventricular dysplasia with the review of the literature.
Arrhythmias, Cardiac ; Arrhythmogenic Right Ventricular Dysplasia* ; Death, Sudden ; Electrocardiography ; Heart Ventricles ; Myocardium

The clinical diagnosis of right ventricular (RV) cardiomyopathies is often challenging. It is difficult to differentiate the isolated left ventricular (LV) noncompaction cardiomyopathy (NC) from biventricular NC or from coexisting arrhythmogenic ventricular cardiomyopathy (AC). There are currently few established morphologic criteria for the diagnosis other than RV dilation and presence of excessive regional trabeculation. The gross and microscopic changes suggest pathological similarities between, or coexistence of, RV-NC and AC. Therefore, the term arrhythmogenic right ventricular cardiomyopathy is somewhat misleading as isolated LV or biventricular involvement may be present and thus a broader term such as AC should be preferred. We describe an unusual case of AC associated with a NC in a 27-year-old man who had a history of permanent pacemaker 7 years ago due to second-degree atrioventricular block.
Adult ; Arrhythmogenic Right Ventricular Dysplasia ; Atrioventricular Block ; Cardiomyopathies* ; Diagnosis ; Echocardiography* ; Humans

Adult ; Arrhythmogenic Right Ventricular Dysplasia ; physiopathology ; Electrocardiography ; Humans ; Male ; Middle Aged

Arrhythmogenic Right Ventricular Dysplasia ; diagnosis ; Death, Sudden ; Humans ; Male ; Middle Aged