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
    尽管栓塞现在被认为是PAVM的首选治疗方法,如果畸形涉及大血管,可以考虑手术干预。
    Despite embolization being now considered the preferred treatment for PAVM, surgical intervention may be considered if the malformation involves large vessels.
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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: Journal Article
    目的:为了评估安全性,聚氨酯覆膜支架的技术成功和短期有效性(PKPapyrus,BioTronik,柏林)用于治疗不适合栓塞治疗的肺动静脉畸形(PAVMs)。
    方法:在此IRB批准的,回顾性研究,我们分析了接受聚氨酯覆盖支架排除PAVM的患者的数据.该研究包括5名患者(所有女性),中位年龄为40岁(范围25-60)。患者出现低氧血症,TIA,和/或鼻出血;4例被证实患有HHT。所有人都在胸部CT上诊断出多个PAVM,并且除了聚氨酯覆盖的支架外,还使用其他设备进行了栓塞。在所有情况下,支架置入的指征是短和/或曲折的供血动脉。通过即时或短期并发症评估安全性,例如,迁移,支架内血栓形成,和骨折。技术成功被定义为将支架准确地放置在预期位置的能力。有效性定义为成功排除PAVM,无AVM灌注。
    结果:支架置入的技术成功率为100%。单支架展开后AVM排除率为80%;在不完全排除的情况下,使用重叠支架完全覆盖第二条供血动脉取得了成功.在5个月的中位随访期间(范围2-10),所有支架仍然专利,排除AVM,无其他并发症。
    结论:用聚氨酯覆盖的支架排除PAVM在技术上是可行的,安全,当传统的栓塞技术无法实现时,对于具有短/曲折的供血动脉的PAVM显示出短期有效性。
    OBJECTIVE: To evaluate the safety, technical success and short-term effectiveness of polyurethane-covered stent (PK Papyrus, BioTronik, Berlin) in the treatment of pulmonary arteriovenous malformations (PAVMs) that are not amenable to embolotherapy.
    METHODS: In this IRB-approved, retrospective study, data from patients who received polyurethane-covered stents for exclusion of PAVMs were analyzed. The study included 5 patients (all women) with a median age of 40 years (range 25-60). Patients presented with hypoxemia, TIAs, and/or epistaxis; 4 were confirmed to have HHT. All had multiple PAVMs diagnosed on chest CT and underwent embolization with other devices in addition to the polyurethane-covered stent. The indication for stent placement in all cases was a short and/or tortuous feeding artery. Safety was assessed by immediate or short-term complications, e.g., migration, stent thrombosis, and fracture. Technical success was defined as the ability to accurately place the stent at the intended location. Effectiveness was defined as successful exclusion of PAVM with no perfusion across the AVM.
    RESULTS: Technical success of stent placement was 100%. AVM exclusion rate was 80% after single stent deployment; in the case of incomplete exclusion, success was achieved using an overlapping stent to completely cover a second feeding artery. During the median follow-up period of 5 months (range 2-10), all stents remained patent, and AVMs were excluded without other complications.
    CONCLUSIONS: Exclusion of PAVMs with polyurethane-covered stents is technically feasible, safe, and shows short-term effectiveness for PAVMs with a short/tortuous feeding artery when traditional embolization techniques are not possible.
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  • 文章类型: Journal Article
    目的:肺动静脉畸形(PAVMs)可引起脑脓肿复发。主要目的是确定脑脓肿幸存者中PAVM的患病率。事后还评估了心脏从右到左分流的比例。
    方法:这是一项针对丹麦2007年至2016年成人(≥18岁)隐源性细菌性脑脓肿幸存者的横断面研究。患者被邀请进行气泡超声心动图检查,以检测血管从右到左分流,如果异常,随后的胸部计算机断层扫描诊断PAVM。数据表示为n/N(%)或具有四分位距的中值(IQR)。
    结果:47/157(30%)的合格患者接受了研究参与,其中两名患者未出现预定的气泡超声心动图检查。参与者的平均年龄为54岁(IQR45-62),19/57(33%)为女性,而59岁(IQR48-68,p=0.05)和41/85女性(48%,p=0.22)在非参与者中。10/45(22%)参与者的气泡超声心动图提示分流,随后通过计算机断层扫描确认了一名1级分流患者的PAVM。在所有被检查的参与者中,PAVM的相应患病率为2%(95%置信区间0.06-11.8)。另有9/45(20%)诊断为持续性卵圆孔未闭(n=8)或房间隔缺损(n=1),这与丹麦背景人群中成人25%的总体患病率相当。
    结论:在隐源性细菌性脑脓肿的成年幸存者中,未确诊的PAVM是罕见的,但在部分患者中可以考虑。脑脓肿患者中心脏从右到左分流的患病率与普通人群的患病率相对应。
    OBJECTIVE: Pulmonary arteriovenous malformations (PAVMs) may cause recurrent brain abscess. The primary aim was to determine the prevalence of PAVM amongst survivors of brain abscess. The proportion with cardiac right-to-left shunts was also assessed post hoc.
    METHODS: This was a cross-sectional population-based study of adult (≥18 years) survivors of cryptogenic bacterial brain abscess in Denmark from 2007 through 2016. Patients were invited for bubble-echocardiography to detect vascular right-to-left shunting and, if abnormal, subsequent computed tomography thorax for diagnosis of PAVM. Data are presented as n/N (%) or median with interquartile range (IQR).
    RESULTS: Study participation was accepted by 47/157 (30%) eligible patients amongst whom two did not appear for scheduled bubble-echocardiography. The median age of participants was 54 years (IQR 45-62) and 19/57 (33%) were females compared with 59 years (IQR 48-68, p = 0.05) and 41/85 females (48%, p = 0.22) in non-participants. Bubble-echocardiography was suggestive of shunt in 10/45 (22%) participants and PAVM was subsequently confirmed by computed tomography in one patient with grade 1 shunting. The corresponding prevalence of PAVM was 2% (95% confidence interval 0.06-11.8) amongst all examined participants. Another 9/45 (20%) were diagnosed with patent in persistent foramen ovale (n = 8) or atrial septum defect (n = 1), which is comparable with the overall prevalence of 25% amongst adults in the Danish background population.
    CONCLUSIONS: Undiagnosed PAVM amongst adult survivors of cryptogenic bacterial brain abscess is rare but may be considered in select patients. The prevalence of cardiac right-to-left shunts amongst brain abscess patients corresponds to the prevalence in the general population.
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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
    目的:确定栓塞后全身经络继发咯血的患病率,并评估复发率和治疗结果。
    方法:930名PAVM患者(801名已知或可能患有HHT)的记录,在1996年7月2日至2021年7月22日期间,我们从一个HHT中心搜索了一次因栓塞后全身侧支继发的终生咯血.用永久性颗粒或明胶浆液进行栓塞。对已确定患者的临床特征和治疗结果进行了回顾。
    结果:对9例PAVM栓塞后全身动脉侧支再灌注患者进行了28次栓塞手术。这包括8名已知HHT的患者。5例使用永久性颗粒,19例使用明胶浆液。由于咯血复发,四名患者每人需要四次栓塞,2例患者需要3次栓塞,2例患者需要2次栓塞.5例患者出现慢性无法解决的咯血,4例需要入住ICU的大咯血。HHT患者全身动脉再灌注继发咯血的终生患病率和发生率估计为1.0%和0.05%,分别。支气管动脉起源最常见(8例)。在这个中心治疗的第一个病人,导致心肌梗死和卒中的主要不良事件发生在使用300-500微米永久性颗粒时.据推测,这是由于左向右分流和随后的全身性栓塞所致。随后患者接受明胶海绵浆液治疗,无不良事件发生。该患者最终因大量咯血而死亡,在双侧弥漫性PAVM的情况下。第二例患者,有儿童支气管动脉弹簧圈栓塞史,在等待肺叶切除术期间因大量咯血而过期。在两种情况下,患者接受了手术,包括一次肺叶切除术和一次全肺切除术,用于复发性咯血(需要至少5次住院)。其余5例患者仅通过血管内治疗即可长期缓解咯血。
    结论:栓塞后全身络脉继发于PAVM的咯血的终生患病率很少,但复发率很高。在这个有限的系列中,用明胶海绵浆液栓塞似乎是安全的,尽管难治性和多灶性疾病最终可能需要手术切除。
    OBJECTIVE: To determine the prevalence of hemoptysis secondary to post-embolization systemic collaterals and review the recurrence rate and treatment outcomes.
    METHODS: The records of 930 patients with PAVM (801 with known or possible HHT), from a single HHT center between July 2, 1996 and July 22, 2021, were searched for a single lifetime episode of hemoptysis secondary to post-embolization systemic collaterals. Embolization was performed with permanent particles or gelatin slurry. Clinical features and treatment outcomes of identified patients were reviewed.
    RESULTS: Twenty-eight embolization procedures have been performed in 9 patients with post-PAVM embolization systemic artery collateral reperfusion. This included 8 patients with known HHT. Permanent particles were used in 5 cases and gelatine slurry was used in 19 cases. Due to the recurrence of hemoptysis, four patients required four embolizations each, two patients required three embolizations and two patients required two embolizations. Chronic unresolving hemoptysis was the presentation in 5 patients and massive hemoptysis requiring ICU admission in 4. The lifetime prevalence and incidence of hemoptysis secondary to systemic artery reperfusion in HHT patients was estimated to be 1.0% and 0.05%, respectively. Bronchial artery origin was most common (8 patients). In the first patient treated at this center, a major adverse event resulting in myocardial infarct and stroke occurred with the use of 300-500-micron permanent particles. This was presumed to be due to left-to-right shunting and subsequent systemic embolization. Subsequent patients were treated with gelatin sponge slurry without adverse events. This patient ultimately expired due to large volume hemoptysis, in the setting of bilateral diffuse PAVMs. A second patient, with history of childhood bronchial artery coil embolization, expired from large volume hemoptysis while awaiting lobectomy. In two cases, patients underwent surgery, including one lobectomy and one pneumonectomy, for recurrent hemoptysis (requiring at least five hospital admissions). The remaining five patients achieved prolonged resolution of hemoptysis with endovascular treatment alone.
    CONCLUSIONS: Lifetime prevalence of hemoptysis secondary to PAVM post-embolization systemic collaterals is rare, but recurrence is high. In this limited series, embolization with gelatin sponge slurry appeared safe, although surgical resection may ultimately be required in refractory and multifocal disease.
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  • 文章类型: Journal Article
    肺动静脉畸形(PAVMs)并不常见,肺动脉和肺静脉之间主要是先天性直接瘘管连接,导致从右到左分流.PAVM患者通常无症状,在进行放射学成像以用于其他适应症时偶然发现病变。在这次审查中,我们讨论了PAVM的分类和放射学特征,以及它们的治疗和随访方案,特别关注经皮血管内技术和可用治疗设备的发展。
    Pulmonary arteriovenous malformations (PAVMs) are uncommon, predominantly congenital direct fistulous connections between the pulmonary arteries and pulmonary veins, resulting in a right to left shunt. Patients with PAVMs are usually asymptomatic with lesions detected incidentally when radiological imaging is performed for other indications. In this review, we discuss the classification and radiological features of PAVMs as well as their treatment and follow-up options, with a particular focus on percutaneous endovascular techniques and the evolution of the available equipment for treatment.
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  • 文章类型: Case Reports
    肺动静脉畸形(PAVMs)可能表现为出血或栓塞事件,需要进行干预。经肺动脉(PA)的经导管线圈栓塞是一种既定的方法。尽管有许多PA线圈栓塞,我们还是提出了一例PAVM复发的病例。PAVM闭塞是通过经中隔和肺静脉入路放置堵塞物来实现的。(难度等级:高级。).
    Pulmonary arteriovenous malformations (PAVMs) may manifest with bleeding or embolic events necessitating intervention. Transcatheter coil embolization through the pulmonary artery (PA) is an established approach. We present a case of recurrent PAVMs despite numerous PA coil embolizations. PAVM occlusion was achieved through plug placement by a transseptal and pulmonary venous approach. (Level of Difficulty: Advanced.).
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