Shovlin Claire L, Buscarini Elisabetta, Hughes J Michael B, Allison David J, Jackson James E
NHLI Vascular Science, Imperial College London, London, UK.
Respiratory Medicine, and VASCERN HHT European Reference Centre, Hammersmith Hospital, Imperial College Healthcare NHS Trust, London, UK.
BMJ Open Respir Res. 2017 Oct 13;4(1):e000198. doi: 10.1136/bmjresp-2017-000198. eCollection 2017.
Pulmonary arteriovenous malformations (PAVMs) may not be amenable to treatment by embolisation or surgical resection, and many patients are left with significant hypoxaemia. Lung transplantation has been undertaken. There is no guidance on selection criteria.
To guide transplantation listing assessments, the outcomes of the six patients who had been considered for transplantation were compared with a similarly hypoxaemic patient group recruited prospectively between 2005 and 2016 at the same UK institution.
Six patients had been formally considered for lung transplantation purely for PAVMs. One underwent a single lung transplantation for diffuse PAVMs and died within 4 weeks of surgery. The other five were not transplanted, in four cases at the patients' request. Their current survival ranges from 16 to 27 (median 21) years post-transplant assessment. Of 444 consecutive patients with PAVMs recruited between 2005 and 2016, 42 were similarly hypoxaemic to the 'transplant-considered' cohort (SaO <86.5%). Hypoxaemic cohorts maintained arterial oxygen content (CaO) through secondary erythrocytosis and higher haemoglobin. The 'transplant-considered' cohort had similar CaO to the hypoxaemic comparator group, but higher Medical Research Council (MRC) dyspnoea scores (p=0.023), higher rates of cerebral abscesses (p=0.0043) and higher rates of venous thromboemboli (p=0.0009) that were evident before and after the decision to list for transplantation.
The non-transplanted patients demonstrated marked longevity. Symptoms and comorbidities were better predictors of health than oxygen measurements. While a case-by-case decision, weighing survival estimates and quality of life will help patients in their decision making, the data suggest a very strong case must be made before lung transplantation is considered.
肺动静脉畸形（PAVM）可能无法通过栓塞或手术切除进行治疗，许多患者会出现严重低氧血症。已开展肺移植手术。目前尚无关于选择标准的指导意见。
为指导移植评估，将6例曾被考虑进行移植的患者的结局与2005年至2016年在同一英国机构前瞻性招募的低氧血症程度相似的患者组进行比较。
6例患者曾因PAVM被正式考虑进行肺移植。1例因弥漫性PAVM接受了单肺移植，术后4周内死亡。另外5例未进行移植，其中4例是应患者要求。自移植评估后，他们目前的生存时间为16至27年（中位值21年）。在2005年至2016年连续招募的444例PAVM患者中，42例与“考虑移植”队列的低氧血症程度相似（动脉血氧饱和度<86.5%）。低氧血症队列通过继发性红细胞增多症和较高的血红蛋白水平维持动脉血氧含量（CaO）。“考虑移植”队列的CaO与低氧血症对照队列相似，但医学研究委员会（MRC）呼吸困难评分更高（p=0.023），脑脓肿发生率更高（p=0.0043），静脉血栓栓塞发生率更高（p=0.0009），这些在决定列入移植名单之前和之后均很明显。
未进行移植的患者显示出显著的长寿。症状和合并症比氧测量更能预测健康状况。虽然需逐案决策，权衡生存估计和生活质量将有助于患者做出决策，但数据表明在考虑肺移植之前必须有充分的理由。
