致心律失常性右室心肌病(ARVC)临床和病理观察(附3例报告)

Clinical and pathological observation of arrhythmogenic right ventricular cardiomyopathy

目的 通过所遇的病例 ,学习有关文献 ,提高对ARVC的认识 ,特别是对中青年的不明原因心律失常 ,早期识别、预防猝死。讨论 病因 ,目前普遍认为是常染色体遗传病 ,可为显性、不完全外显及隐性。也提出心肌被脂肪组织所替代是慢性心肌炎引起的后天性损伤 (炎症、坏死 )和修复过程演进的结果。诊断 主要依据心慌、头晕、昏厥等症状 ,结合心电图有典型的室性早搏、短阵室速、室颤 ,QRS时限≥ 110ms,进一步可对其心脏形态及功能进行评价。组织学检查为诊断ARVC的金标准 ,但心内膜活检须注意取材部位 ,预防室壁穿孔等并发症。治疗 目前主张一般情况用药物抗心律失常 ,严重者可植入除颤器 。

Objective By analysing cases of ARVC we encountered and reviewed related literaturts, to deepen the understanding of ARVC, especially early recoganizing arrhythmia with unknown aetiology in young and middleaged and prevention of sudden death. Discussion At present, ARVC is generally believed a autosomal hereditary disease and may be dominance, partial dominance or larval. It is also suggested that cardiac muscle replaced by fatty tissue is the result of acquired injury (inflammation, necrosis) caused by chronic myocarditis and repairing process.Diagnosis Diagnosis is mainly based upon clinical symptoms such as palpilation, dizziness, syncope, combined with typical electrocardiogram of ventricular premature beats, paroxysmal tachyarrhythmia, ventricular fibrillation, QRS time limit ≥110ms, Furthmore, evaluation of the shape and function of the heart should be performed. Histological examination is the gold standard for diagnosis of ARVC, but must pay attention to the site of drawing material during endocardial biopsy to avoid ventricular wall perforation. Treatment ARVC is usually treated with antiarrhythmic drug. severe cases could be treated by implanting defibrillator or undergoing heart transplantation.