双向Glenn术后发生肺动-静脉畸变的研究

Glenn手术是治疗青紫型先心病常用的姑息性手术方法,术后部分患者可出现肺动-静脉畸变的病理改变,表现为青紫进行性加重.这种病变与体内多种机制的变化有关,其中因手术后肝静脉血流改道,来源于肝血流中的某些调控血管增生和调节血管张力的物质不再进入肺循环而缺乏,是导致肺血管异常增生、扩张和畸变的重要原因.     

双向Glenn术后发生肺动-静脉畸变的研究

Glenn手术是治疗青紫型先心病常用的姑息性手术方法,术后部分患者可出现肺动-静脉畸变的病理改变,表现为青紫进行性加重。这种病变与体内多种机制的变化有关,其中因手术后肝静脉血流改道,来源于肝血流中的某些调控血管增生和调节血管张力的物质不再进入肺循环而缺乏,是导致肺血管异常增生、扩张和畸变的重要原因。     

介入治疗肺动静脉畸形一例

肺动静脉畸形（PAVM）是一种临床少见的肺内血管畸形,近期我院收治1例,报道如下. 1临床资料 患者,男性,60岁,鼻出血数十年.曾考虑鼻中隔偏曲行手术,手术成功,但术后仍反复鼻出血.     

肺动静脉畸形的介入治疗

肺动静脉畸形(pulmonary arteriovenous malformations PAVMs)为先天性肺血管畸形。是罕见的肺部疾病，迄今为止国外文献报道仅数百例．国内报道很少。现将介人治疗PVAMs报告如下。     

38例原发性肝癌动-静脉瘘介入治疗的疗效分析

目的回顾性评价超选择性动脉插管栓塞原发性肝癌合并动-静脉瘘的疗效,以期提高患者的生存质量和延长生存期。方法本院近2年实施经动脉插管栓塞化疗的原发性肝癌425例,其中合并动-静脉瘘38例,采用超选择性动脉插管分别对动-静脉瘘以无水乙醇或无水乙醇＋明胶海绵颗粒、对肿瘤病灶以无水乙醇＋碘油（1：1）或碘油＋2～3种化疗药物实施栓塞。观察术后的临床表现,并随访复发率与生存率。结果425例原发性肝癌合并动脉-门静脉瘘38例（8．9％）,其中,高流量型21例（55％）,低流量型17例（45％）,同时伴有肝动脉-肝静脉瘘8例（21％）。38例均成功实施动-静脉瘘及肿瘤病灶的超选择性动脉插管栓塞术,其中,35例经过2～3次治疗。术后肝功能好转23例（61％）;腹水消失13例（34％）,腹水明显减少20例（53％）;6个月生存率84％（32例）,1年生存率61％（23例）。结论超选择性动脉插管栓塞术治疗肝癌伴动-静脉瘘,能缓解其导致的门静脉高压及其严重后果,疗效肯定,并且为同期实施的肿瘤病灶栓塞治疗奠定了基础,该方法可明显提高患者的生存质量,延长生存期。     

CT诊断肺血栓栓塞症-深静脉血栓形成的临床应用进展

临床实践证明CT诊断肺血栓栓塞症 (PTE)和深静脉血栓形成 (DVT)的敏感性及特异性较高 ,本文综述了CT肺动脉造影 (CTPA)和间接性CT静脉造影 (CTV)的临床应用、比较影像学及发展前景。CTPA CTV可作为诊断PTE DVT的首选检查方法。CT肺血流灌注技术 ,可以在微血管水平定量研究肺血流灌注 ,评价肺血流灌注功能 ,用于指导治疗、评价疗效     

肺移植围手术期ECMO动脉-静脉与静脉-静脉转流应用效果对比观察

目的 对比观察肺移植围手术期体外膜肺氧合（ECMO）动脉-静脉与静脉-静脉转流的应用效果。方法回顾221例肺移植患者临床资料，根据ECMO转流方式不同分为动脉-静脉转流147例（VA组）、静脉-静脉转流74例（VV组）。比较两组围手术期相关临床资料，包括术前左心室射血分数、平均肺动脉压、心功能分级，手术方式、手术时间、术中出血量和输血量，术后立即撤除ECMO比例、ECMO转流时间以及肺部感染、原发性移植物失功（PGD）、肾功能不全、持续性肾脏替代治疗、二次开胸止血等不良事件。结果 两组术前左心室射血分数、心功能分级比较差异均无统计学意义（P均>0.05）,VA组术前平均肺动脉压显著高于VV组（P<0.05）。两组手术方式、手术时间、术中出血量和输血量比较差异均无统计学意义（P均>0.05）。VA组术后立即撤除ECMO比例显著高于VV组，ECMO转流时间显著低于VV组（P均<0.05）。两组术后肺部感染发生率、二次开胸止血比例比较差异均无统计学意义（P均>0.05）,VA组术后PGD、肾功能不全、持续性肾脏替代治疗比例均显著低于VV组（P均<0.05）...     

肺血栓栓塞症CT肺动脉造影检查技术及优化策略

肺血栓栓塞症（PTE）是临床常见的危、急重症,CT肺动脉造影（CTPA）是PTE患者的首选检查方法。规范的 CTPA检查技术及图像后处理方法对PTE患者的诊断和治疗具有十分重要的临床意义,文章将阐述PTE的CTPA检 查技术及优化策略。     

脑动静脉畸形的介入治疗

脑动静脉畸形是一种先天性脑血管发育异常,是导致自发性颅内出血的常见原因之一。随着血管内介入治疗技术和材料的发展,脑动静脉畸形的栓塞治疗越来越广泛地应用于临床。因此,有关脑动静脉畸形血管内栓塞治疗的适应证、栓塞技术和材料以及治疗的并发症更加引起人们关注。     

TACE术中应用聚乙烯醇微粒栓塞治疗肝细胞癌并发肝动-静脉分流患者疗效

目的 探讨在TACE过程中应用聚乙烯醇微粒（PVA）栓塞治疗肝细胞癌（HCC）并发肝动-静脉分流（AVS）患者的效果。 方法 2013年1月~2015年2月我院诊治的HCC并发AVS患者156例,根据经动脉注入造影剂到达门静脉或者肝静脉显影时间的长短,将患者分为A组（慢速型）43例、B组（中速型）61例和C组（快速型）52例,在TACE治疗过程中分别给予300~500μm、500~710μm和710~1000μmPVA对AVS进行栓塞治疗。 结果 快速型、中速型和慢速型患者在Child-Pugh分级、甲胎蛋白水平和肿瘤大小等方面比较差异无统计学意义（P>0.05）;在治疗后3月,A组、B组和C组客观缓解率分别为27.9%、26.2%和28.8%,差异无统计学意义（P>0.05）;三组不良反应发生率无统计学差异（P<0.05）;A组 1a和2a生存率分别为67.7%和34.9%,B组分别为65.6%和36.1%,C组分别为65.4%和32.7%,均无统计学差异（P>0.05）。 结论 应用PVA栓塞治疗HCC并发AVS患者疗效确实,可能有助于提高TACE治疗的疗效。     

Management of pancreatic arteriovenous malformation

Pancreatic arteriovenous malformations (AVM), while extremely rare, are frequently complicated by gastrointestinal bleeding. The elimination of pancreatic AVM is difficult once portal hypertension has developed. We describe herein a patient with congenital AVM of the pancreatic head presenting with recurrent episodes of melena, in whom pylorus-preserving pancreatoduodenectomy provided a means of definitive management. We also review the literature and focus on the diagnostic and therapeutic approaches. Angiography is always necessary to facilitate tactics of treatment, even if diagnosis has been established by non-invasive imaging modalities. To obtain complete regression, total extirpation of the affected organ, or at least the involved portion, should be performed before this disease leads to the lethal complications of gastrointestinal bleeding and portal hypertension. Transcatheter arterial embolization is the only alternative treatment for the control of hemorrhage.     

应用X刀治疗脑动静脉畸形87例

目的探讨立体定向放射外科治疗脑动静脉畸形的临床效果。方法对87例脑动静脉畸形患者采用X刀治疗，动静脉畸形团大小为5～40mm。87例中单独接受X刀治疗55例，联合血管内栓塞治疗32例。治疗周边剂量17～30Gy，平均22．6Gy，等剂量曲线70％～90％。随访时间6～96个月，平均53．4个月。结果单独接受X刀治疗的脑动静脉畸形患者，41例完全闭塞，9例部分闭塞，5例无变化；联合血管内栓塞治疗的患者，26例完全闭塞，3例部分闭塞，3例无变化。两组患者完全闭塞67例(77．01％)，癫痫完全缓解7例(26．17％)，头痛、头昏症状缓解18例(38．30％)，再出血2例，症状性放射性脑水肿6例。经治疗后，完全缓解2例，遗留神经功能障碍4例。结论立体定向放射外科治疗是脑动静脉畸形的安全而有效的治疗方法之一。     

Colonoscopic removal of a polypoid arteriovenous malformation

A 53-year-old male presenting with a 3-month history of intermittent mild rectal bleeding was found, on double contrast barium enema, to have a large polyp on a long stalk in the sigmoid colon. Large bowel endoscopy confirmed the presence of a 2 cm pedunculated polyp which was removed using a diathermic snare, with slight bleeding following the procedure that did not require endoscopic haemostasis. Only after histologic examination was the polyp shown to be a colonic arteriovenous malformation. Endoscopically, arteriovenous malformations generally appear as flat or elevated bright red lesions. A pedunculated polypoid appearance is extremely uncommon. In this case, no gastrointestinal bleeding or polypoid recurrence was observed during the 12 months of clinical and endoscopic follow-up.     

大型或巨大型脑动静脉畸形的显微外科治疗25例

目的 总结25例大型或巨大型脑动静脉畸形(AVM)患者的显微外科治疗经验,探讨降低手术死亡率和致残率的有效方法。 方法 25例AVM患者,男17例,女8例,平均年龄26.8岁,均经数字减影全脑血管造影确诊。病变大小为5-6 cm者12例,>6 cm者13例;按照国内史玉泉分级法:3级15例,3-4级10例;位于幕下2例。全部患者行显微外科手术治疗,病灶完全摘除,其中8例行病灶血管内栓塞加手术切除。 结果 完全切除畸形血管团,24例痊愈,1例重残(1年后恢复),无手术死亡,1例术中出现正常脑灌注压突破;17例术后复查DSA,均未见残留畸形血管团。结论 显微外科手术切除畸形血管团,仍然是治疗脑AVM的有效手段,血管内栓塞加手术切除是治疗巨大型AVM的理想方法。     

Asymptomatic arteriovenous malformation of the pancreas

Summary We reported a case of asymptomatic pancreatic AVM, incidentally found on routine ultrasonography and diagnosed noninvasively by means of Doppler ultrasonography and MRI. The diagnosis was confirmed by angiography. This is the first report to demonstrate the characteristics of pancreatic AVM by these two imaging techniques. They proved to be useful in identifying the vascular nature of AVM without the use of a contrast material, which is a definite advantage over DSA and CT. The quality of the image obtained was equivalent or superior to that obtained by DSA and contrast-enhanced CT. Doppler ultrasonography and MRI may serve as the primary imaging techniques of choice in suspected cases of AVM.     

Pancreatic arteriovenous malformation with portal hypertension

A 45-year-old man with recurrent episodes of hematemesis caused by extensive varices in the esophagus and stomach was admitted. He had a history of liver cirrhosis with hepatitis C virus infection. Computed tomography revealed a conglomeration of small strong nodular stains in the pancreatic head. Angiography revealed a racemose vascular network at the same site and early appearance of the portal venous system in the arterial phase. With a diagnosis of pancreatic arteriovenous malformation with portal hypertension, he underwent pylorus-preserving pancreaticoduodenectomy, preceded, 2 days earlier, by transcatheter arterial embolization of some of the feeding arteries. The varices observed preoperatively in the esophagus and stomach disappeared, and he has been well for 6 years after the operation. We reviewed 47 cases of pancreatic arteriovenous malformation previously reported in the English-language literature, with a focus on the clinical manifestations, treatment approaches, and etiological relationship with portal hypertension and liver cirrhosis.     

Pancreatic arteriovenous malformation with duodenal ulcer

Summary We report the color Doppler ultrasonography features of arteriovenous malformation (AVM) of the pancreas, a very rare disease. The patient was a 52-year-old man with congenital AVM of the pancreas and a duodenal ulcer that had been resistant to medication. Endoscopic color Doppler ultrasonography (color Doppler EUS) revealed many abnormal color signals showing pulsatile wave form at the portion of the duodenal wall involving the duodenal ulcer. Extracorporeal color Doppler ultrasonography revealed a mosaic-like color signal, caused by turbulent flow, in the portal trunk. Angiography demonstrated a vascular network with extensive proliferation at the pancreatic head and early portal filling. It is possible that the pancreatic AVM had caused the duodenal ulcer. Color Doppler EUS can be a useful modality for detection of vessel abnormalities of the gastrointestinal tract.     

3D打印技术在颅内动静脉畸形血管内介入治疗中的初步应用

目的探讨3D打印技术在指导颅内动静脉畸形(AVM)血管内介入治疗中的临床应用价值。方法贯序交替选取2015年2月至5月广东省人民医院神经外科进行血管内介入治疗的AVM患者10例,其中5例为对照组,5例为进行3D打印模型试验组。对试验组5例AVM患者采用256层螺旋CT薄层增强扫描或3D-DSA旋转成像,提取检查结果的DICOM原始数据,应用薄层数据扫描获取5例AVM患者三维数据,通过Mimics 14.01软件进行数字化数据提取和重建,并按1∶1比例进行3D打印,获得实体模型。应用AVM实体模型指导与患者及其家属的术前谈话及手术方案设计,并与对照组结果进行对比。结果 3D打印模型试验组术前谈话时间(10.2±0.8)min较对照组术前谈话时间(15.0±1.3)min显著缩短,差异有统计学意义(P0.01);试验组患者及其家属满意度评分显著高于对照组[(9.3±0.6)分比(8.1±0.3)分,P0.01],试验组术中造影后至手术实施时间较对照组显著缩短[(5.7±0.4)min比(10.5±1.6)min,P0.01]。无一例手术相关并发症。结论 3D打印模型可以帮助术者更加直观地了解畸形团空间构筑学特征,指导术者进行手术方案的制定,同时应用3D打印实体模型可提高与AVM患者术前谈话效率及患者满意度。     

BMPR2 gene mutation in pulmonary arteriovenous malformation and pulmonary hypertension: A case report

The transforming growth factor-β superfamily signaling pathway is thought to be involved in the pathogenesis of pulmonary arteriovenous malformation (PAVM). However, the association between bone morphogenetic protein receptor type 2 (BMPR2) gene mutations and PAVM remains unclear. We present a case of concurrent PAVM and pulmonary arterial hypertension (PAH), with a deletion mutation in exon 6 and exon 7 of the BMPR2 gene. Drug treatment for PAH improved the patient's hemodynamics and exercise capacity, but worsened oxygenation. This case suggests that BMPR2 gene mutation may be associated with the complex presentation of PAVM combined with PAH.