患有肺动脉闭锁伴完整室间隔的婴儿需要进行球囊房间隔造口术，其 18 个月的死亡率显著升高。
Infants with pulmonary atresia intact ventricular septum who require balloon atrial septostomy have significantly higher 18-month mortality.
机构信息
Department of Medicine, UC San Diego Health, San Diego, CA, USA.
UC San Diego School of Medicine, San Diego, CA, USA.
出版信息
Cardiol Young. 2021 Oct;31(10):1613-1618. doi: 10.1017/S1047951121000640. Epub 2021 Mar 1.
INTRODUCTION
Many newborns with pulmonary atresia/intact ventricular septum require intervention to establish pulmonary flow and sufficient cardiac output. The resulting haemodynamic changes are not well characterised and may have unintended consequences.
METHODS
This is a 30-year (1988-2018) retrospective study of patients with pulmonary atresia intact ventricular septum.
RESULTS
Eighty-nine patients were included, and median follow-up was 8 years. Fifty-five per cent had coronary sinusoids and 27% had right ventricular-dependent coronary circulation. Most patients were managed with surgical aortopulmonary or modified Blalock-Taussig shunt (73%), and 12 patients underwent balloon atrial septostomy before surgical intervention. The remaining patients (27%) underwent only transcatheter interventions; 7 required an atrial septostomy and 17 required ductal stentings. All-cause mortality was 10%, most deaths (89%) occurred before 18 months of age. Of these early deaths, 87% required a balloon atrial septostomy and 85% had right ventricular-dependent coronary sinusoids. Eighteen-month mortality was significantly higher for patients who required a balloon atrial septostomy compared to those who did not (36% versus 1.4% p < 0.0001).
DISCUSSION
Patients with pulmonary atresia/intact ventricular septum who require balloon atrial septostomy in the newborn period have significantly higher 18-month mortality. Quantifying the mortality difference may help guide prognostication and expectation setting. Infants who had septostomy and a surgical shunt in the newborn period fared better than those who only underwent septostomy (even when accompanied by ductal stenting). For infants with right ventricular-dependent circulation, atrial septostomy should only be performed on an urgent or emergent basis and these patients should be considered for early surgical intervention and neonatal transplant.
介绍
许多患有肺动脉闭锁/完整室间隔的新生儿需要介入治疗来建立肺血流和足够的心输出量。由此产生的血流动力学变化尚未得到很好的描述，可能会产生意想不到的后果。
方法
这是一项对 30 年来（1988-2018 年）患有肺动脉闭锁/完整室间隔的患者进行的回顾性研究。
结果
共纳入 89 例患者，中位随访时间为 8 年。55%的患者存在冠状窦，27%的患者存在右室依赖型冠状循环。大多数患者接受了外科体肺或改良的 Blalock-Taussig 分流术（73%）治疗，12 例患者在外科干预前接受了球囊房间隔造口术。其余患者（27%）仅接受了经导管介入治疗；7 例需要房间隔造口术，17 例需要导管支架置入术。全因死亡率为 10%，大多数死亡（89%）发生在 18 个月龄之前。这些早期死亡中，87%需要球囊房间隔造口术，85%存在右室依赖型冠状窦。需要球囊房间隔造口术的患者 18 个月死亡率明显高于无需该术式的患者（36%对 1.4%，p<0.0001）。
讨论
在新生儿期需要球囊房间隔造口术的肺动脉闭锁/完整室间隔患者，18 个月死亡率显著升高。量化死亡率差异可能有助于指导预后和预期设定。在新生儿期接受分流术和外科分流术的婴儿比仅接受分流术的婴儿（即使伴有导管支架置入术）预后更好。对于存在右室依赖型循环的婴儿，仅在紧急或紧急情况下进行房间隔造口术，应考虑这些患者早期进行外科干预和新生儿移植。
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