肺动静脉畸形(PAVMs)最常见于遗传性出血性毛细血管扩张症(HHT)。PAVM患者可出现严重的并发症,包括中风,短暂性脑缺血发作(TIA),脑脓肿.PAVM在非HHT患者中很少见,对该患者人群知之甚少。这项回顾性研究的目的是更好地了解仅在非HHT患者中发生的PAVM的临床表现和结果。
对2000年1月1日至2018年12月31日在梅奥诊所-罗切斯特的非HHTPAVM患者进行了回顾。库拉索评分>1的患者被排除在外。人口统计,成像特性,神经系统并发症,并对随访影像进行分析。
确定了77例PAVM患者。诊断时平均年龄为48.2±18.3岁,女性占优势(59.7%)。大多数PAVM以下叶为主(66.7%),在75.3%和89.6%的病例中简单和单一,分别。大多数患者无症状(46.8%),呼吸困难是最常见的症状(28.6%)。19.5%的患者发生神经系统并发症。大多数PAVM是特发性的(61%)。30例患者(39%)有一个或多个可能的危险因素,包括以前的胸外科手术(23.4%),先天性心脏病(19.5%),胸部创伤(10.4%)。37例(48.1%)患者进行了栓塞治疗,只有4例(5.2%)进行了手术切除。
非HHTPAVM更常见于女性,最常见的是简单和单一的,并且具有下叶优势和神经系统并发症的高发生率。在约40%的病例中发现了潜在的诱发风险因素。临床医生应该意识到有胸部创伤史的患者发生PAVM的风险,先天性心脏病,肺部感染/脓肿,还有胸外科.
Pulmonary arteriovenous malformations (PAVMs) are most commonly associated with hereditary hemorrhagic telangiectasia (HHT). Patients with PAVMs can present with serious complications including stroke, transient ischemic attack (TIA), and brain abscess. PAVMs are rare in non-HHT patients and little is known about this patient population. The aim of this retrospective
study is to better understand clinical presentation and outcomes of PAVMs occurring exclusively in non-HHT patients.
Non-HHT patients with PAVMs at the Mayo Clinic-Rochester between 01/01/2000 and 12/31/2018 were reviewed. Patients with Curacao score > 1 were excluded. Demographics, imaging characteristics, neurological complications, and follow-up imaging were analyzed.
Seventy-seven patients with PAVMs were identified. The mean age at diagnosis was 48.2 ± 18.3 years with female preponderance (59.7%). The majority of PAVMs had lower lobe predominance (66.7%) and were simple and single in 75.3% and 89.6% of cases, respectively. Most patients were asymptomatic (46.8%) with dyspnea being the most common symptom (28.6%). Neurologic complications occurred in 19.5% of patients. The majority of PAVMs were idiopathic (61%). Thirty patients (39%) had one or more possible risk factors including previous thoracic surgery (23.4%), congenital heart disease (19.5%), and chest trauma (10.4%). Embolization was performed in 37 (48.1%) patients and only 4 (5.2%) underwent surgical resection.
Non-HHT PAVMs occur more commonly in females, are most commonly simple and single, and have lower lobe predominance and a high rate of neurologic complications. Potential predisposing risk factors were identified in about 40% of the cases. Clinicians should be aware of the risk of
PAVM development in patients with history of chest trauma, congenital heart disease, lung infection/abscess, and thoracic surgery.