Frustaci A, Chimenti C, Bellocci F, Morgante E, Russo M A, Maseri A
Istituto di Cardiologia, Università Cattolica del S. Cuore, and the Dipartimento di Medicina Sperimentale e Patologia, Rome, Italy.
Circulation. 1997 Aug 19;96(4):1180-4. doi: 10.1161/01.cir.96.4.1180.
Lone atrial fibrillation (LAF) is a common clinical syndrome, but its origin remains unknown.
We performed endomyocardial biopsies of the right atrial septum (2 to 3 per patient; mean, 2.8) and of the two ventricles (6 per patient) in 12 patients (10 men, 2 women; mean age, 32 years) with paroxysmal LAF refractory to conventional antiarrhythmic treatment. As controls, we used endomyocardial biopsies (3 to 5 per patient; mean, 4.4) from the right atrial septum of 11 patients with Wolff-Parkinson-White syndrome (WPW) undergoing resection of the abnormal AV pathway. The weight of the biopsies ranged from 2.8 to 4.5 mg. Biopsy samples were processed for histology and electron microscopy and were read by a pathologist blinded to clinical data. All patients underwent two-dimensional Doppler echocardiography; cardiac catheterization; coronary angiography; and hormonal, virologic, and electrophysiological studies. All tests and WPW biopsies were normal, but all LAF atrial biopsy specimens (average, 2.8 per patient) showed abnormalities (P<.0001). The type of abnormalities varied: Two patients had a severe hypertrophy with vacuolar degeneration of the atrial myocytes and ultrastructural evidence of fibrillolysis occupying >50% of the areas assessed morphometrically (P=.50), 8 had lymphomononuclear infiltrates with necrosis of the adjacent myocytes (5 with fibrosis and 3 without; P<.003), and 2 had only nonspecific patchy fibrosis (P=.50). Biventricular biopsies were abnormal in only 3 patients and showed inflammatory infiltrates similar to those found in atrial biopsies.
Abnormal atrial histology was uniformly found in multiple biopsy specimens in all patients with LAF. It was compatible with a diagnosis of myocarditis in 66% of patients (active in 25%) and of noninflammatory localized cardiomyopathy in 17% and was represented by patchy fibrosis in 17%. The cause of the pathological changes, which were found only in atrial septal biopsies but not in biventricular biopsies, in 75% of patients remains unknown.
孤立性心房颤动（LAF）是一种常见的临床综合征，但其起源尚不清楚。
我们对12例（10例男性，2例女性；平均年龄32岁）阵发性LAF患者进行了右心房隔（每位患者2至3块；平均2.8块）和两个心室（每位患者6块）的心内膜活检，这些患者对传统抗心律失常治疗无效。作为对照，我们使用了11例接受异常房室通路切除术的预激综合征（WPW）患者右心房隔的心内膜活检（每位患者3至5块；平均4.4块）。活检组织重量在2.8至4.5毫克之间。活检样本进行组织学和电子显微镜检查，并由一位对临床数据不知情的病理学家解读。所有患者均接受二维多普勒超声心动图、心导管检查、冠状动脉造影以及激素、病毒学和电生理研究。所有检查及WPW活检均正常，但所有LAF患者的心房活检标本（平均每位患者2.8块）均显示异常（P<0.0001）。异常类型各异：2例患者出现严重肥大，伴有心房肌细胞空泡变性，且超微结构显示纤颤溶解占据经形态计量评估区域的>50%（P=0.50）；8例患者有淋巴细胞单核细胞浸润及相邻心肌细胞坏死（5例伴有纤维化，3例无纤维化；P<0.003）；2例患者仅有非特异性斑片状纤维化（P=0.50）。仅3例患者的双心室活检异常，且显示出与心房活检中相似的炎性浸润。
在所有LAF患者的多个活检标本中均一致发现心房组织学异常。66%的患者符合心肌炎诊断（25%为活动性心肌炎），17%符合非炎性局限性心肌病诊断，17%表现为斑片状纤维化。75%患者的病理改变仅在右心房隔活检中发现，而双心室活检未发现，其病因仍不清楚。
