Bauer Anna, Khalil Markus, Schmidt Dorle, Bauer Jürgen, Esmaeili Anoosh, Apitz Christian, Voelkel Norbert F, Schranz Dietmar
1 Justus Liebig University Clinic Giessen, Hessen Pediatric Heart Center, Giessen, Germany.
2 Johann-Wolfgang Goethe University Clinic, Frankfurt, Germany.
Pulm Circ. 2018 Apr-Jun;8(2):2045894018776518. doi: 10.1177/2045894018776518. Epub 2018 Apr 25.
Atrial septostomy (AS) is recommended for pulmonary arterial hypertension (PAH)-associated right ventricular (RV) failure, recurrent syncope, or pulmonary hypertensive crisis (PHC). We aimed to evaluate the feasibility and efficacy of AS to manage PAH from infancy to adulthood. From June 2009 to December 2016, transcatheter atrial communications were created in 11 PAH patients (4 girls/women; median age = 4.3 years; range = 33 days-26 years; median body weight = 14 kg; range = 3-71 kg; NYHA-/Ross class IV; n = 11). PAH was classified as idiopathic (n = 6) or secondary (n = 5). History of syncope was dominant (n = 6); two with patent foramen ovale (PFO) admitted with recurrent PHC, three patients required resuscitation before AS. Three patients had PAH-associated low cardiac output. The average pulmonary arterial pressures (PAP systolic/diastolic) were 101/50 (±34/23); the corresponding systemic arterial pressures (SAP) were 99/54 (±23/11); and the mean ratio of PAPd / SAPd was 0.97 (±0.4). Percutaneous trans-septal puncture was uneventfully performed in nine patients; a PFO was dilated in two patients. There was no procedure-related mortality. The median balloon size was 10 mm (range = 6-14 mm); the mean catheter time was 174.6 ± 48 min; fluoroscopy time was 19.8 (±11) min. Syncope and PHC were successfully treated in all patients. The mean arterial oxygen saturation decreased from 97 ± 2 to 89 ± 11.7. One patient died awaiting lung transplantation, one continues to be listed; two patients received a reverse Potts-shunt, one patient died during follow-up; seven patients are stable with PAH-specific treatment. Percutaneous AS is an effective method palliating PAH-associated syncope, PHCs or right (bi-) ventricular heart failure.
对于肺动脉高压（PAH）相关的右心室（RV）衰竭、反复晕厥或肺动脉高压危象（PHC），推荐进行房间隔造口术（AS）。我们旨在评估AS用于治疗从婴儿期到成年期PAH的可行性和疗效。2009年6月至2016年12月，对11例PAH患者（4例女性；中位年龄 = 4.3岁；范围 = 33天至26岁；中位体重 = 14 kg；范围 = 3至71 kg；纽约心脏协会/罗斯分级IV级；n = 11）进行了经导管房间隔交通建立。PAH分为特发性（n = 6）或继发性（n = 5）。晕厥病史占主导（n = 6）；2例卵圆孔未闭（PFO）患者因反复PHC入院，3例患者在AS术前需要进行复苏。3例患者存在PAH相关的心输出量降低。平均肺动脉压（收缩压/舒张压）为101/50（±34/23）；相应的体动脉压（SAP）为99/54（±23/11）；PAPd / SAPd的平均比值为0.97（±0.4）。9例患者顺利完成经皮经间隔穿刺；2例患者扩张了PFO。无手术相关死亡。球囊中位大小为10 mm（范围 = 6至14 mm）；平均导管操作时间为174.6 ± 48分钟；透视时间为19.8（±11）分钟。所有患者的晕厥和PHC均得到成功治疗。平均动脉血氧饱和度从97 ± 2降至89 ± 11.7。1例患者在等待肺移植时死亡，1例仍在等待名单中；2例患者接受了反向Potts分流术，1例患者在随访期间死亡；7例患者通过PAH特异性治疗病情稳定。经皮AS是缓解PAH相关晕厥、PHC或右（双）心室心力衰竭的有效方法。
