de Freitas A F
Postgrad Med J. 1986 Jun;62(728):601-5. doi: 10.1136/pgmj.62.728.601.
A systematic investigation was performed in patients with familial amyloidotic polyneuropathy, Portuguese type (AFp) to assess the pattern and incidence of cardiac involvement. Of 327 patients investigated, ECG abnormalities were present in 285 (87.2%). Low voltage and QS pattern in V1, V2, V3 were found in 51.3% and 35.7% patients respectively. Conduction disturbances were present in 211 (64.5%). Sinus node disease, 1st degree and Wenckebach interventricular blocks were frequent. Complete atrioventricular block was observed in only 2 patients (0.6%). Left anterior hemi-block was present in 30.8%, left bundle branch block in 3.9%, left posterior hemi-block in 2.4% and right bundle branch block in 2.1%. Holter monitoring showed a much higher incidence of conduction disturbances, most of these occurring at night. The mean values of septum and posterior wall thickness and mass evaluated by echocardiography in 72 patients were normal. The systolic and diastolic global and regional functions, determined in 12 patients, analysing the echo by a digitization computer technique, were normal. In 7% a trivial pericardial effusion was observed. In 16 patients with ECG changes and normal echocardiograms the technetium 99m pyrophosphate scanning was negative. We conclude that the ECG is the most precise, sensitive and clinically useful method for detecting cardiac amyloidosis in patients with AFp. In spite of the rarity of congestive and restrictive patterns, the incidence and severity of conduction disturbances does not allow us to consider heart disease in AFp as a benign entity.
对葡萄牙型家族性淀粉样多神经病（AFp）患者进行了一项系统性研究，以评估心脏受累的模式和发生率。在327例接受调查的患者中，285例（87.2%）存在心电图异常。分别有51.3%和35.7%的患者出现V1、V2、V3导联低电压和QS波型。211例（64.5%）存在传导障碍。窦房结疾病、一度和文氏型房室传导阻滞较为常见。仅2例患者（0.6%）观察到完全性房室传导阻滞。左前半阻滞发生率为30.8%，左束支传导阻滞为3.9%，左后半阻滞为2.4%，右束支传导阻滞为2.1%。动态心电图监测显示传导障碍发生率更高，其中大多数发生在夜间。72例患者经超声心动图评估的室间隔和后壁厚度及质量的平均值正常。通过数字化计算机技术分析12例患者的回声确定的收缩和舒张期整体及局部功能正常。7%的患者观察到微量心包积液。16例心电图改变但超声心动图正常的患者，99m锝焦磷酸盐扫描结果为阴性。我们得出结论，心电图是检测AFp患者心脏淀粉样变性最精确、敏感且临床有用的方法。尽管充血性和限制性模式罕见，但传导障碍的发生率和严重程度使我们不能将AFp中的心脏病视为良性疾病。
