Kitano Masataka, Hoashi Takaya, Kakuta Takashi, Fujimoto Kazuto, Miyake Akira, Kurosaki Ken-Ichi, Ichikawa Hazime, Shiraishi Isao
Department of Pediatric Cardiology, National Cerebral and Cardiovascular Center, Suita, Osaka, Japan.
Department of Pediatric Cardiovascular Surgery, National Cerebral and Cardiovascular Center, Suita, Osaka, Japan.
Pediatr Cardiol. 2018 Oct;39(7):1355-1365. doi: 10.1007/s00246-018-1902-z. Epub 2018 May 18.
For neonates with right atrial isomerism (RAI), functional single ventricle (f-SV), and obstructive total anomalous pulmonary venous connection (TAPVC), primary TAPVC repair (TAPVCR) has a poor outcome. At our hospital, the survival rate at 1 year of such neonates undergoing primary TAPVCR between 1999 and 2010 (TAPVCR group) was 30% (3/10). Most deceased cases suffered from capillary leak syndrome and unstable pulmonary resistance after the surgeries. We sought to determine whether less invasive primary draining vein stenting (DVS) improved the outcome of these neonates. We investigated outcomes in consecutive nine such neonates (median gestational age 38 weeks, birth weight 2.8 kg, females 4) who underwent primary DVS with 6-mm-diameter Palmaz® Genesis® stents at our hospital between 2007 and 2017 (DVS group). Eight patients underwent subsequent surgeries to adjust the pulmonary flow after decreased pulmonary resistance. The survival rate at 1 year after the first interventions in the DVS group improved to 77% (7/9), although there was a difference between the interventional eras of the two groups. Of the seven patients who underwent multiple stent redilations with a larger balloon or additional stenting in other sites until the next stage of surgery at a median age of 8 months, four received a bidirectional Glenn (BDG) shunt and TAPVCR and three underwent TAPVCR, with two of those cases reaching BDG. Less invasive primary DVS improved the outcome of neonates with RAI, f-SV, and obstructive TAPVC, with many reaching BDG. Patient selection to advance toward Fontan is thought to further improve the outcome.
对于患有右心房异构（RAI）、功能性单心室（f-SV）和梗阻性完全性肺静脉异位连接（TAPVC）的新生儿，原发性TAPVC修复术（TAPVCR）的预后较差。在我们医院，1999年至2010年间接受原发性TAPVCR的此类新生儿（TAPVCR组）1年生存率为30%（3/10）。大多数死亡病例术后患有毛细血管渗漏综合征和肺阻力不稳定。我们试图确定侵入性较小的原发性引流静脉支架置入术（DVS）是否能改善这些新生儿的预后。我们调查了2007年至2017年间在我们医院接受直径6毫米的Palmaz® Genesis® 支架原发性DVS的连续9例此类新生儿（中位胎龄38周，出生体重2.8千克，女性4例）的预后（DVS组）。8例患者在肺阻力降低后接受了后续手术以调整肺血流量。DVS组首次干预后1年生存率提高到77%（7/9），尽管两组的介入时代有所不同。7例患者在中位年龄8个月时接受了多次使用更大球囊的支架再扩张或在其他部位额外置入支架，直到下一阶段手术，其中4例接受了双向格林（BDG）分流术和TAPVCR，3例接受了TAPVCR，其中2例达到了BDG。侵入性较小的原发性DVS改善了患有RAI、f-SV和梗阻性TAPVC的新生儿的预后，许多患者达到了BDG。选择合适的患者进行Fontan手术被认为可以进一步改善预后。
