Chick Jeffrey Forris Beecham, Reddy Shilpa N, Pyeritz Reed E, Trerotola Scott O
Division of Interventional Radiology, Department of Radiology, Perelman School of Medicine, Hospital of the University of Pennsylvania, 1 Silverstein, 3400 Spruce Street, Philadelphia, PA, 19104, USA.
Division of Vascular and Interventional Radiology, Department of Radiology, University of Michigan Medical Center, 1500 East Medical Center Drive, Ann Arbor, MI, 48109, USA.
Cardiovasc Intervent Radiol. 2017 Jul;40(7):1003-1009. doi: 10.1007/s00270-017-1604-6. Epub 2017 Feb 10.
To determine current practice regarding screening, management, and follow-up of pulmonary arteriovenous malformations (PAVMs), particularly those with feeding arteries <3 mm in diameter, in those patients with hereditary hemorrhagic telangiectasia (HHT) at HHT Centers of Excellence.
A 32-question survey focusing on PAVM screening, management, and follow-up was sent to 147 participants at HHT Centers worldwide.
Thirty-three responses were received (22%); 60% from interventional radiologists and 20% from pulmonologists; 67% from the USA, 21% European, and 6% Asian. In HHT patients with initial negative screening echocardiogram, 60% recommended repeat screening contrast echocardiogram in 5 years, while 15% felt that no additional screening was necessary in these patients. Fifty-nine percentage reported never having seen a patient with initial negative contrast echocardiogram become positive. In patients with grade 1 echocardiograms, 41% recommended contrast-enhanced CT as the next step in management, 22% recommended non-contrast CT, and 22 and 3% recommended repeat echocardiogram in 5 and 10 years, respectively. In patients with grade 1 echocardiograms but no visible PAVMs, opinions regarding the use of bubble filters were evenly divided, while 79% recommended prophylactic antibiotics prior to invasive procedures. Eighty percentages of respondents treat PAVMs with feeding artery less than 3 mm, and 52% treat feeders less than 2 mm. Regarding follow-up, 71% reported seeing PAVM recurrence at the site of a previously completely treated PAVM.
There is inconsistency in practice regarding PAVM screening, management, and follow-up, suggesting a need for further research and guideline development.
确定在卓越遗传性出血性毛细血管扩张症（HHT）中心，针对遗传性出血性毛细血管扩张症（HHT）患者肺动静脉畸形（PAVM）的筛查、管理及随访的当前实践情况，尤其是那些供血动脉直径<3毫米的肺动静脉畸形。
向全球HHT中心的147名参与者发送了一份包含32个问题的调查问卷，重点关注PAVM的筛查、管理及随访。
共收到33份回复（22%）；其中60%来自介入放射科医生，20%来自肺科医生；67%来自美国，21%来自欧洲，6%来自亚洲。在初始经胸超声心动图筛查为阴性的HHT患者中，60%建议在5年后重复进行对比增强超声心动图筛查，而15%认为这些患者无需进一步筛查。59%的受访者表示从未见过初始对比增强超声心动图为阴性的患者转为阳性。在超声心动图1级的患者中，41%建议下一步进行对比增强CT检查，22%建议进行非对比CT检查，22%和3%分别建议在5年和10年后重复超声心动图检查。在超声心动图1级但未见明显PAVM的患者中，对于使用气泡过滤器的意见分歧较大，而79%建议在侵入性操作前使用预防性抗生素。80%的受访者会治疗供血动脉小于3毫米的PAVM，52%会治疗小于2毫米的供血动脉。关于随访，71%的受访者报告在先前已完全治疗的PAVM部位出现复发。
在PAVM的筛查、管理及随访方面存在实践差异，这表明需要进一步研究并制定指南。
