Khan Wahab J, Asif Muhammad, Chaudhry Hammad S, Aslam Sadia, Nadeem Ifrah, Chowdhury Mohammed A
University of South Dakota Sanford School of Medicine, Sioux Falls, SD, USA.
Avera McKennan Hospital and University Health Center, Sioux Falls, SD, USA.
J Community Hosp Intern Med Perspect. 2023 Jun 29;13(4):21-25. doi: 10.55729/2000-9666.1174. eCollection 2023.
Arrhythmogenic cardiomyopathy (ACM) is a myocardium disease characterized by phenotypic features of myocardial scarring due to fibrofatty myocardial replacement often associated with global or regional ventricular dysfunction. For years after arrhythmogenic right ventricular cardiomyopathy (ARVC) was first described, the left ventricle (LV) was generally considered normal or minimally involved. In recent years, however, LV involvement has been recognized. It usually presents with early-on arrhythmias more than heart failure symptoms compared to dilated cardiomyopathy. It can be right ventricular, biventricular, or left ventricular. The underlying pathophysiology involves either desmosomal or non-desmosomal mutations. Phospholamban (PLN) mutation is one of those and is associated with more severe arrhythmias and SCD. Primary prevention with ICD implantation should be considered in these patients, even the ones with an ejection fraction greater than 35%. In addition, if such patients progress to Stage D heart failure, they need to be evaluated for advanced heart failure therapies.
致心律失常性心肌病（ACM）是一种心肌疾病，其特征为心肌纤维脂肪替代导致心肌瘢痕形成的表型特征，常伴有全心或局部心室功能障碍。在致心律失常性右室心肌病（ARVC）首次被描述后的数年里，左心室（LV）通常被认为是正常的或仅有轻微受累。然而，近年来，左心室受累已得到认可。与扩张型心肌病相比，它通常较早出现心律失常而非心力衰竭症状。它可以是右心室、双心室或左心室受累。潜在的病理生理学涉及桥粒或非桥粒突变。磷酸受纳蛋白（PLN）突变就是其中之一，且与更严重的心律失常和心源性猝死（SCD）相关。对于这些患者，即使射血分数大于35%，也应考虑植入植入式心律转复除颤器（ICD）进行一级预防。此外，如果此类患者进展为D期心力衰竭，则需要对其进行晚期心力衰竭治疗评估。
