ABSTRACT
Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is predominantly an inherited cardiomyopathy with typical histopathological characteristics of fibro-fatty infiltration mainly involving the right ventricular (RV) inflow tract, RV outflow tract, and RV apex in the majority of patients. The above pathologic evolution frequently brings patients with ARVD/C to medical attention owing to the manifestation of syncope, sudden cardiac death (SCD), ventricular arrhythmogenesis, or heart failure. To prevent future or recurrent SCD, an implantable cardiac defibrillator (ICD) is highly desirable in patients with ARVD/C who had experienced unexplained syncope, hemodynamically intolerable ventricular tachycardia (VT), ventricular fibrillation, and/or aborted SCD. Notably, the management of frequent ventricular tachyarrhythmias in ARVD/C is challenging, and the use of antiarrhythmic drugs could be unsatisfactory or limited by the unfavorable side effects. Therefore, radiofrequency catheter ablation (RFCA) has been implemented to treat the drug-refractory VT in ARVD/C for decades. However, the initial understanding of the link between fibro-fatty pathogenesis and ventricular arrhythmogenesis in ARVD/C is scarce, the efficacy and prognosis of endocardial RFCA alone were limited and disappointing. The electrophysiologists had broken through this frontier after better illustration of epicardial substrates and broadly application of epicardial approaches in ARVD/C. In recent works of literature, the application of epicardial ablation also successfully results in higher procedural success and decreases VT recurrences in patients with ARVD/C who are refractory to the endocardial approach during long-term follow-up. In this article, we review the important evolution on the delineation of arrhythmogenic substrates, ablation strategies, and ablation outcome of VT in patients with ARVD/C.
Subject(s)
Humans , Anti-Arrhythmia Agents , Arrhythmogenic Right Ventricular Dysplasia , Cardiomyopathies , Catheter Ablation , Catheters , Death, Sudden, Cardiac , Defibrillators , Epicardial Mapping , Follow-Up Studies , Heart Failure , Prognosis , Recurrence , Syncope , Tachycardia , Tachycardia, Ventricular , Ventricular FibrillationABSTRACT
Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is characterized by progressive fibrofatty replacement of the myocardium that predisposes to ventricular tachycardia and sudden death in young individuals and athletes. The prevalence of ARVD/C is estimated as 1:1000 to 1:1250 in the general population. Although it is a relatively uncommon cause of sudden cardiac death, it accounts for up to one fifth of sudden cardiac death in people less than 35 years of age. Clinical presentation of ARVD/C usually consists of arrhythmias of right ventricular origin that include premature ventricular beats, sustained ventricular tachycardia and ventricular fibrillation that can lead to sudden death. The authors present a case of a 26 year old young male, carpenter by occupation, previously healthy, with sudden death. The internal and external postmortem findings were normal except for mild right ventricular hypertrophy. But histo-pathological examination of heart tissues revealed replacement of myocardial tissue with mature fibrofatty tissue. The following medico-legal autopsy case is being reported for its rarity and the importance of histopathology to find out the cause of death.
Subject(s)
Adult , Arrhythmogenic Right Ventricular Dysplasia/complications , Arrhythmogenic Right Ventricular Dysplasia/epidemiology , Arrhythmogenic Right Ventricular Dysplasia/mortality , Autopsy , Cause of Death , Death, Sudden, Cardiac/etiology , Forensic Pathology/methods , Humans , MaleABSTRACT
Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is characterized histologically by massive infilteration of right ventricular wall by fat tissue with surviving strands of cardiomyocytes bordered by or embedded in fibrosis. ARVC has been recognized as a cause of sudden death, especially in the young. We report an autopy case of ARVD/C in a 35-year-old female. She was found dead in her house under apparently natural circumstances. The autopsy revealed a dilated 340-gram heart with a fibrofatty replacement of the right ventricular myocardium. On the review of her past medical history, she had taken medical examination for prolonged general weakness about 1 year prior to death. At that time the echocardiogram revealed dilatation of right ventricular cavity size and moderately decreased left ventricular systolic function, the electrocardiogram revealed R>S at V1 lead and T-wave inversion at V1-V3 leads. To the best of our knowledge, this is the second autopsy case of ARVD/C, reported in the literature of Korea.
Subject(s)
Adult , Female , Humans , Autopsy , Death, Sudden , Dilatation , Electrocardiography , Fibrosis , Heart , Korea , Myocardium , Myocytes, CardiacABSTRACT
A 35-year-old man was admitted with a 20 day history of generalized edema and muscular weakness of the lower extremities. He was alert with a pale puffy face and an ejection murmur was heard at the cardiac apex. The electrocardiogram disclosed low voltage, first degree atrioventricular block, and a right bundle branch block. During the hospitalization an intractable diastolic hypotension developed, which measured 0 mmHg at the lowest point. At that time the echocardiogram revealed a dilated, akinetic right ventricle. Eventually a multiorgan failure developed and an autopsy following his death presented a fibrofatty replacement of the right ventricular myocardium. This might be a case of an arrhythmogenic right ventricular dysplasia/cardiomyopathy, which is usually characterized clinically by a ventricular tachycardia and may cause a sudden death in young adults.