Departments of Cardiology, Seattle Children's Hospital, University of Washington, Seattle, WA, USA.
Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
Pediatr Cardiol. 2021 Aug;42(6):1372-1378. doi: 10.1007/s00246-021-02621-1. Epub 2021 May 4.
Interstage mortality (IM) remains high for patients with single-ventricle congenital heart disease (SVCHD) in the period between Stage 1 Palliation (S1P) and Glenn operation. We sought to characterize IM.
This was a descriptive analysis of 2184 patients with SVCHD discharged home after S1P from 60 National Pediatric Cardiology Quality Improvement Collaborative sites between 2008 and 2015. Patients underwent S1P with right ventricle-pulmonary artery conduit (RVPAC), modified Blalock-Taussig-Thomas shunt (BTT), or Hybrid; transplants were excluded.
IM occurred in 153 (7%) patients (median gestational age 38 weeks, 54% male, 77% white), at 88 (IQR 60,136) days of life, and 39 (IQR 17,84) days after hospital discharge; 13 (8.6%) occurred ≤ 30 days after S1P. The mortality rate for RVPAC was lower (5.2%; 59/1138) than BTT (9.1%; 65/712) and Hybrid (20.1%; 27/134). More than half of deaths occurred at home (20%) or in the emergency department (33%). The remainder occurred while inpatient at center of S1P (cardiac intensive care unit 36%, inpatient ward 5%) or at a different center (5%). Fussiness and breathing problems were most often cited as harbingers of death; distance to surgical center was the biggest barrier cited to seeking care. Cause of death was unknown in 44% of cases overall; in the subset of patients who underwent post-mortem autopsy, the cause of death remained unknown in 30% of patients, with the most common diagnosis being low cardiac output.
Most IM occurred in the outpatient setting, with non-specific preceding symptoms and unknown cause of death. These data indicate the need for research to identify occult causes of death, including arrhythmia.
在一期姑息治疗（S1P）和 Glenn 手术之间的阶段，患有单心室先天性心脏病（SVCHD）的患者的中期死亡率（IM）仍然很高。我们试图描述 IM。
这是对 2008 年至 2015 年期间，60 个国家儿科心脏病学质量改进合作机构中出院回家接受 S1P 治疗的 2184 例 SVCHD 患者的描述性分析。患者接受了右心室肺动脉导管（RVPAC）、改良的 Blalock-Taussig-Thomas 分流术（BTT）或 Hybrid 治疗的 S1P。排除了移植。
153 例（7%）患者发生 IM（中位胎龄 38 周，54%为男性，77%为白人），发生在住院后 88 天（IQR 60，136），住院后 39 天（IQR 17，84）；13 例（8.6%）发生在 S1P 后≤30 天。RVPAC 的死亡率较低（5.2%，59/1138），而 BTT（9.1%，65/712）和 Hybrid（20.1%，27/134）较高。超过一半的死亡发生在家庭（20%）或急诊室（33%）。其余的发生在 S1P 中心的住院病房（心脏重症监护病房 36%，住院病房 5%）或不同的中心（5%）。最常提到的死亡先兆是烦躁不安和呼吸问题；到手术中心的距离是寻求护理的最大障碍。在总体的 44%的病例中，死因不明；在接受尸检的患者亚组中，30%的患者死因仍不清楚，最常见的诊断是低心输出量。
大多数 IM 发生在门诊环境中，有非特异性的前驱症状和不明原因的死亡。这些数据表明需要研究来确定隐匿性死亡原因，包括心律失常。
