Turrini Pietro, Corrado Domenico, Basso Cristina, Nava Andrea, Thiene Gaetano
Department of Pathology, University of Padua Medical School, Padova, Italy.
Ann Noninvasive Electrocardiol. 2003 Apr;8(2):161-9. doi: 10.1046/j.1542-474x.2003.08212.x.
The natural history of arrhythmogenic right ventricular cardiomyopathy is determined by the electrical instability of the dystrophic myocardium, which can precipitate arrhythmic cardiac arrest any time during the course of the disease and by the progressive myocardial loss that results in ventricular dysfunction and heart failure. Sudden death accounts for the majority of the fatal events but its occurrence is mostly unpredictable. There are no prospective and controlled studies assessing clinical markers that can predict the occurrence of life-threatening ventricular arrhythmias. However, the noninvasive risk profile, which emerges from retrospective analysis of clinical and pathologic series, is characterized by history of syncope, physical exercise, spontaneous ventricular tachycardia or ventricular fibrillation, right ventricular dysfunction, left ventricular involvement, right precordial negative T wave, right bundle branch block, QT-QRS dispersion, right precordial ST-segment elevation and late potentials. At present only QRS dispersion, history of syncope and right and/or left ventricular abnormalities at radionuclide angiography proved to be independent noninvasive predictors of sudden death.
致心律失常性右室心肌病的自然病史由营养不良心肌的电不稳定性决定，这种电不稳定性可在疾病过程中的任何时候引发心律失常性心脏骤停，同时也由导致心室功能障碍和心力衰竭的进行性心肌丧失所决定。猝死占大多数致命事件，但它的发生大多不可预测。目前尚无前瞻性对照研究评估可预测危及生命的室性心律失常发生的临床标志物。然而，通过对临床和病理系列进行回顾性分析得出的无创风险特征包括晕厥史、体育锻炼、自发性室性心动过速或心室颤动、右心室功能障碍、左心室受累、右胸前导联T波倒置、右束支传导阻滞、QT-QRS离散度、右胸前导联ST段抬高和晚电位。目前，只有QRS离散度、晕厥史以及放射性核素血管造影显示的右心室和/或左心室异常被证明是猝死的独立无创预测指标。
