Maeda S, Tanaka T, Hayashi T
Department of Medicine, Sakakibara Heart Institute, Tokyo, Japan.
Br Heart J. 1988 Apr;59(4):498-500. doi: 10.1136/hrt.59.4.498.
Three of nine siblings were affected by atrial standstill. Clinical, electrophysiological, and histological findings in two of these cases were studied. Electrophysiological studies showed persistent atrial standstill in one and partial atrial standstill in the other. Biopsy specimens of the right atria showed amyloid deposits, but specimens of the right ventricles and skin did not. Neither patient had peripheral neuropathy, gastrointestinal disorder, or renal dysfunction. Familial atrial standstill is usually associated with peripheral neuropathy. In the two cases studied amyloid deposition mainly affected the atrium, rather than the ventricles or other organs, and caused atrial standstill.
九名兄弟姐妹中有三人患有心房停搏。对其中两例患者的临床、电生理和组织学检查结果进行了研究。电生理研究显示，一例为持续性心房停搏，另一例为部分性心房停搏。右心房活检标本显示有淀粉样沉积物，但右心室和皮肤标本未发现。两名患者均无周围神经病变、胃肠道疾病或肾功能障碍。家族性心房停搏通常与周围神经病变有关。在这两例研究中，淀粉样沉积主要影响心房，而非心室或其他器官，并导致心房停搏。
