Dept of Cardiology, St Antonius Hospital, Nieuwegein, The Netherlands
Dept of Cardiology, St Antonius Hospital, Nieuwegein, The Netherlands.
Eur Respir J. 2016 Jun;47(6):1750-7. doi: 10.1183/13993003.01588-2015. Epub 2016 Mar 10.
Pulmonary arteriovenous malformations (PAVMs) are associated with severe neurological complications in hereditary haemorrhagic telangiectasia (HHT). Transthoracic contrast echocardiography (TTCE) is recommended for screening of pulmonary right-to-left shunts (RLS). Although growth of PAVMs is shown in two small studies, no studies on follow-up with TTCE exist.All HHT patients underwent a second TTCE 5 years after initial screening. Patients with a history of PAVM embolisation were excluded. Pulmonary RLS grade on TTCE after 5 years was compared to the grade at screening.200 patients (53.5% female, mean±sd age at screening 44.7±14.1 years) were included. Increase in RLS grade occurred in 36 (18%) patients, of whom six (17%) underwent embolisation. The change in grade between screening and follow-up was not more than one grade. Of patients with nontreatable pulmonary RLS at screening (n=113), 14 (12.4%) underwent embolisation. In patients without pulmonary RLS at initial screening (n=87), no treatable PAVMs developed during follow-up.Within 5 years, no treatable PAVMs developed in HHT patients without pulmonary RLS at initial screening. Increase in pulmonary RLS grade occurred in 18% of patients, and never increased by more than one grade. Of patients with nontreatable pulmonary RLS at initial screening, 12% underwent embolisation.
肺动静脉畸形（PAVMs）与遗传性出血性毛细血管扩张症（HHT）的严重神经系统并发症有关。经胸超声心动图对比造影（TTCE）被推荐用于筛查肺右向左分流（RLS）。虽然有两项小型研究显示 PAVMs 有生长，但尚无关于 TTCE 随访的研究。所有 HHT 患者在初次筛查后 5 年内进行第二次 TTCE。排除有 PAVM 栓塞史的患者。比较 5 年后 TTCE 的 PAVM 分级与筛查时的分级。200 例患者（53.5%为女性，筛查时的平均年龄±标准差为 44.7±14.1 岁）纳入研究。36 例（18%）患者 RLS 分级增加，其中 6 例（17%）患者接受了栓塞治疗。筛查和随访时的分级变化不超过 1 级。在筛查时有不可治疗性 RLS 的患者中（n=113），有 14 例（12.4%）接受了栓塞治疗。在初次筛查时无 RLS 的患者中（n=87），随访期间未发生可治疗性 PAVMs。在初次筛查时无 RLS 的 HHT 患者中，5 年内未发生可治疗性 PAVMs。18%的患者 RLS 分级增加，且从未增加超过 1 级。在初次筛查时有不可治疗性 RLS 的患者中，有 12%接受了栓塞治疗。
