PAVM

PAVM
  • 文章类型: Case Reports
    背景:肺动静脉畸形(PAVM),也被称为肺动静脉瘘,是一种罕见的血管发育异常。大多数PAVM病例与遗传性出血性毛细血管扩张症(HHT)有关。与PAVM相关的血胸甚至更罕见,有关这一并发症的管理仍然面临挑战。
    方法:我院收治一名突然出现呼吸困难和胸痛的55岁男性患者。他有鼻出血病史,腹膜内生殖细胞肿瘤和PAVM。胸部未增强CT显示左侧胸腔积液伴部分被动肺不张,间隔6天逐渐增加。诊断性胸腔穿刺术进一步显示出血性积液。CT血管造影(CTA)显示左下肺动脉和PAVM的管腔弯曲扩张,并形成动脉瘤。由于他的家人拒绝手术,患者接受了经导管栓塞治疗.然而,左胸腔积液没有明显减少,即使在介入治疗后血红蛋白值也缓慢下降,表明持续活动性出血的可能性。最终,患者接受了左下叶肺叶切除术,结果令人满意。
    结论:PAVM破裂进入胸膜腔引起的大量血胸可导致致命的结果。CTA可以准确诊断这种病理状况。经导管栓塞术常用于治疗PAVM,但在血胸患者中达到理想的效果可能是具有挑战性的。结合我们的案例和文献回顾,当PAVM并发血胸和大直径的引流静脉时,直接根治性手术可以导致成功的结果。
    BACKGROUND: Pulmonary arteriovenous malformation (PAVM), also known as pulmonary arteriovenous fistula, is a rare vascular developmental anomaly. Most cases of PAVM are associated with hereditary hemorrhagic telangiectasia (HHT). Hemothorax associated with PAVM is even rarer, and management concerning this complication still challenges.
    METHODS: A 55-year-old man with sudden onset of dyspnea and chest pain was admitted to our hospital. He had a medical history of epistaxis, intraperitoneal germ cell tumor and PAVM. Chest unenhanced CT revealed the left-sided pleural effusion together with partial passive atelectasis and gradual increase at the interval of six days. Diagnostic thoracocentesis further revealed hemorrhagic effusion. CT angiography (CTA) showed tortuously dilated lumen of the left lower pulmonary artery and PAVM with the formation of aneurysm. Due to his family\'s refusal of surgery, the patient underwent transcatheter embolization therapy. However, the left pleural effusion did not significantly reduce and there was a slow drop in hemoglobin value even after interventional treatment, indicating the possibility of ongoing active bleeding. Eventually, the patient received lobectomy of the left lower lobe with a satisfactory outcome.
    CONCLUSIONS: Massive hemothorax resulting from PAVM rupture into the pleural space can lead to fatal outcomes. CTA can accurately diagnose this pathologic condition. Transcatheter embolization is frequently used in the treatment of PAVM, but it may be challenging to achieve the desirable effect in patients with hemothorax. Combined with our case and literature review, direct radical surgery can lead to a successful outcome when PAVM complicated with hemothorax and a large diameter of the draining vein.
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  • 文章类型: Case Reports
    肺动静脉畸形,以前被认为是一种罕见的疾病,在过去的20年里,越来越多的无症状患者被发现。通常是先天性的,并伴有遗传性出血性毛细血管扩张症,这些瘘管由于缺乏毛细血管床的肺动脉和静脉的异常连通而导致血液从右到左分流。在成像上确定的存在进食和引流血管的情况下,右向左分流的临床发现证实了诊断,一线治疗是栓塞。本报告重点介绍了在随访10年的患者中偶然发现的大型无症状PAVM的表现和管理,该患者表现为继发于病毒感染的急性低氧性呼吸衰竭,PAVM大小间隔增加。
    Pulmonary arteriovenous malformations, previously considered a rare condition, have been increasingly identified in asymptomatic patients over the past 2 decades. Usually congenital and associated with hereditary hemorrhagic telangiectasia, these fistulae result in right-to-left shunting of blood by abnormal communication of pulmonary arteries and veins lacking capillary beds. Clinical findings of right-to-left shunting in the presence of feeding and draining vessels identified on imaging confirm the diagnosis, for which the first-line therapy is embolization. This report highlights the presentation and management of a large asymptomatic PAVM detected incidentally in a patient who was lost to follow-up for 10 years and represented with acute hypoxic respiratory failure secondary to a viral infection with an interval increase of PAVM size.
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  • 文章类型: Case Reports
    胃肠道动静脉畸形(AVM)是一种罕见的异常,主要是由于先天原因。胰腺AVM患者可以在没有症状的情况下生存。它可以表现为消化道出血或门脉高压,可以通过计算机断层扫描(CT)或血管造影进行诊断。CT表现包括多个离散的胰腺内血管。一名48岁的男子抱怨腹部疼痛,并向背部辐射了一个月,与呼吸急促有关,食欲不振,一个月内体重意外下降33%,无恶心或呕吐。在体检时,腹部松软,上腹部压痛,墨菲征阴性。实验室检查显示高淀粉酶,肝肾功能正常。CT显示胰腺AVM。他接受了部分胰腺切除术和脾切除术。手术后,患者报告症状有所改善。所有后续访问都很顺利。胰腺AVM是一种罕见的疾病,大多数患者最重要的主诉是胃肠道出血。它需要根据体征和症状进行成像。主要的成像模式是CT,用减影血管造影进行确认。在可以接受的情况下,手术治疗是大多数患者的管理标准。此外,早期发现这些罕见的异常可以避免大量胃肠道出血和耐药性门静脉高压症的发展,如果发生出血,可以挽救患者的生命。
    Arteriovenous malformation (AVM) of the gastrointestinal tract is a rare anomaly, mostly due to congenital reasons. Patients with pancreatic AVM can live without experiencing symptoms. It can present with gastrointestinal bleeding or portal hypertension, and diagnosis can be made by computed tomography (CT) or angiography. CT findings include multiple discrete intrapancreatic vessels. A 48-year-old man complained of abdominal pain with a sensation of fullness that radiated to the back for a month, associated with shortness of breath, loss of appetite, and unintentional weight loss of 33% in one month without nausea or vomiting. On physical examination, the abdomen was soft and lax with epigastric tenderness and a negative Murphy sign. Laboratory investigations showed high amylase with normal liver and kidney functions. CT showed pancreatic AVM. He underwent partial pancreatectomy and splenectomy. After the surgery, the patient reported an improvement in symptoms. All follow-up visits were uneventful. Pancreatic AVM is a rare disease, and the most significant chief complaint of most patients is gastrointestinal tract bleeding. It requires imaging depending on the signs and symptoms. The primary imaging modality is CT, with subtraction angiography for confirmation. Surgical treatment is the standard of management for most patients when tolerable. Additionally, early detection of these rare anomalies can avoid massive gastrointestinal tract bleeding and the development of resistance portal hypertension and can save patients\' lives if bleeding occurs.
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  • 文章类型: Case Reports
    肺动静脉畸形(PAVM)是一种罕见的血管异常,其特征是肺动脉和静脉之间的异常连通。这是咯血的罕见原因。计算机断层扫描血管造影(CTA)已成为识别PAVM的首选和可靠的诊断方法。PAVM栓塞是这种情况的主要推荐治疗方法。我们介绍了一例43岁的男性,左下叶有复杂的PAVM,并伴有咯血,经血管内途径治疗。按照程序,病人的症状得到了缓解。
    Pulmonary arteriovenous malformation (PAVM) is a rare vascular anomaly characterized by abnormal communication between the pulmonary artery and vein. It is a rare cause of hemoptysis. Computed tomographic angiography (CTA) has become the preferred and dependable diagnostic approach for identifying PAVM. PAVM embolization is the primary recommended treatment for this condition. We present a case of a 43-year-old male with a complex PAVM in the left lower lobe presenting with hemoptysis treated with an endovascular approach. Following the procedure, the patient\'s symptoms resolved.
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  • 文章类型: Journal Article
    UNASSIGNED: To describe the preliminary experience using Micro Vascular Plug (MVP) for treatment of pulmonary arteriovenous malformations (PAVMs) with small feeding arteries (3 -5 mm) in four patients with Hereditary Haemorrhagic Telangiectasia (HHT).
    UNASSIGNED: One female and three male patients with PAVMs have been treated during 2017. The mean age was 32.5 years; (range: 20-53). All patients underwent contrast echocardiography and computed tomography of the chest to establish the diagnosis.
    UNASSIGNED: Four patients with PAVMs were treated with embolisation using the MVP-3Q and MVP-5Q micro plugs. All MVP were placed without complications and with following immediate occlusion of the PAVMs in all three cases. In one case MVP was placed semi selectively. All cases were with the good clinical outcomes.
    UNASSIGNED: MVP is a new detachable embolisation material which is easy to use. Maximal control during the deployment and immediate occlusion of the target vessels can be achieved.
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