Kimura Kazumi, Koga Masatoshi, Matsumoto Shoji, Inoue Takeshi, Minematsu Kazuo
Rinsho Shinkeigaku. 2002 Sep;42(9):849-54.
The right-to-left shunt from pulmonary arteriovenous fistula (P-AVF) with Rendu-Osler-Weber (R-O-W) disease can cause paradoxical brain embolism. However, it has remains unclear whether the isolated P-AVF without Rendu-Osler-Weber (R-O-W) disease is associated with ischemic stroke, in particular, paradoxical brain embolism. Our group previously reported a case with paradoxical brain embolism associated with isolated P-AVF without R-O-W disease in 1996. Furthermore, in 1999, we reported that transcranial Doppler (TCD) with saline contrast medium was useful for identifying the presence of P-AVF as a right-to-left shunt. Therefore, when we have an embolic stroke patient with unknown source, we have performed TCD for detecting P-AVF since 1998. The aim of this study was to investigate the frequency of brain infarction associated with isolated P-AVF without R-O-W disease and to evaluate clinical characteristics including a previously reported case, and to elucidate the stroke mechanism.
We studied patients with brain infarction associated with isolated P-AVF without R-O-W disease from 642 ischemic stroke patients admitted to our group within 7 days of stroke onset between August 1998 and May 2002.
Four patients (0.6%) was diagnosed as having brain infarction associated with isolated P-AVF without R-O-W disease. Clinical characteristics of 5 patients were as follows; 1) all patients were middle aged women (mean, 61 year old), 2) they had no evidences of chronic hypoxia such as cyanosis, dyspnea, and erythrocytosis, 3) all strokes occurred in the morning, 4) the diagnosis of brain embolism was made by routine neuroimaging studies, but no potential cardiac nor arterial sources of emboli were detected, 5) the location of brain infarcts on MRI was more frequently in vertebrobasilar circulation (4 cases) than in carotid circulation (1 case), 6) all patients had pulmonary embolism and 4 suffered from deep venous thrombosis, 7) all patients had a history of brain infarction or transient ischemic attack (TIA) before the present attack, 8) all patients had a single P-AVF and its location was right lower lobe in 4 patients and the left lower lobe in the remaining one patient, and 9) all patients could be treated with catheter embolization of P-AVF without recurrent stroke for 37 months in average (range 2-100 months).
The isolated P-AVF without R-O-W disease can cause paradoxical brain embolism. Catheter embolization of P-AVF may be effective in prevention for recurrent stroke. We should not overlooked isolated P-AVF as a right-to-left shunt in embolic stroke patients with unknown etiology.
患有遗传性出血性毛细血管扩张症(Rendu-Osler-Weber,R-O-W)的患者,其肺动静脉瘘(P-AVF)导致的右向左分流可引发反常脑栓塞。然而,尚无Rendu-Osler-Weber(R-O-W)病的孤立性肺动静脉瘘(P-AVF)是否与缺血性卒中相关,尤其是反常脑栓塞,目前仍不清楚。我们团队于1996年曾报道过1例与无R-O-W病的孤立性P-AVF相关的反常脑栓塞病例。此外,在1999年,我们报道经颅多普勒(TCD)联合生理盐水造影剂有助于识别P-AVF作为右向左分流的存在。因此,自1998年起,当我们遇到栓塞性卒中且病因不明的患者时,会进行TCD检查以检测P-AVF。本研究旨在调查与无R-O-W病的孤立性P-AVF相关的脑梗死发生率,评估包括既往报道病例在内的临床特征,并阐明卒中机制。
我们研究了1998年8月至2002年5月期间在卒中发作7天内入院的642例缺血性卒中患者中,与无R-O-W病的孤立性P-AVF相关的脑梗死患者。
4例患者(0.6%)被诊断为与无R-O-W病的孤立性P-AVF相关的脑梗死。5例患者的临床特征如下:1)所有患者均为中年女性(平均61岁);2)无紫绀、呼吸困难和红细胞增多症等慢性缺氧证据;3)所有卒中均发生在早晨;4)通过常规神经影像学检查诊断为脑栓塞,但未检测到潜在的心脏或动脉栓塞源;5)MRI显示脑梗死部位更常见于椎基底动脉循环(4例)而非颈动脉循环(1例);6)所有患者均有肺栓塞,4例有深静脉血栓形成;7)所有患者在本次发作前均有脑梗死或短暂性脑缺血发作(TIA)病史;8)所有患者均有单个P-AVF,4例位于右下叶,其余1例位于左下叶;9)所有患者均接受了P-AVF导管栓塞治疗,平均37个月(范围2 - 100个月)未发生复发性卒中。
无R-O-W病的孤立性P-AVF可导致反常脑栓塞。P-AVF导管栓塞术可能对预防复发性卒中有效。对于病因不明的栓塞性卒中患者,我们不应忽视孤立性P-AVF作为右向左分流的可能性。