Department of Surgery, University of Texas Southwestern Medical Center, Dallas, TX, USA.
, Los Angeles, CA, USA.
Sleep Breath. 2023 May;27(2):505-510. doi: 10.1007/s11325-022-02632-z. Epub 2022 May 13.
Patients with congenital central hypoventilation syndrome (CCHS) have autonomic dysfunction and lack ventilatory responses to hypoxemia and hypercarbia and thus are prone to adverse events during general anesthesia. The objective of this study was to describe the perioperative outcomes of patients with CCHS who were undergoing diaphragm pacer (DP) implantation surgeries under general anesthesia.
A retrospective cohort study was conducted on patients with CCHS who underwent DP implantation surgeries at CHLA between January 2000 and May 2016. Charts were reviewed for demographics, PHOX2B genotype, ventilatory support, comorbidities, anesthesia administered, and perioperative courses.
Of 19 patients with CCHS (58% female) mean age at surgeries was 8.6 ± 5.8 years. Seventeen patients were ventilator-dependent during sleep only; two were ventilator dependent 24 h per day. Mean surgery duration was 3.1 ± 0.5 h. Seventeen patients were extubated to PPV via tracheostomy in the OR. Two patients were extubated to NPPV on postoperative day (POD) 1. Mean transition time to home ventilator or NPPV was 3.0 ± 2.2 days, and mean hospital stay was 5.0 ± 2.1 days. One patient premedicated without ventilatory support developed hypoxemia and hypoventilation. Ten patients (52%) had intraoperative events such as bradycardia, hypotension, significant hypoxemia, and bronchospasm. Fifteen patients had postoperative events. Hypoxemia, pneumonia, and atelectasis accounted for most of perioperative complications. One patient experienced seizure on POD 2 due to hypercarbia.
Patients with CCHS are vulnerable to the cardiorespiratory effects of sedative and anesthetic agents. Therefore, they require vigilant monitoring and optimal ventilatory support in the perioperative period.
先天性中枢性肺泡换气不足综合征（CCHS）患者存在自主神经功能障碍，对低氧血症和高碳酸血症无通气反应，因此在全身麻醉期间易发生不良事件。本研究旨在描述在全身麻醉下接受膈肌起搏器（DP）植入术的 CCHS 患者的围手术期结果。
对 2000 年 1 月至 2016 年 5 月期间在 CHLA 接受 DP 植入术的 CCHS 患者进行回顾性队列研究。对患者的人口统计学资料、PHOX2B 基因型、通气支持、合并症、麻醉方式和围手术期过程进行了回顾。
19 例 CCHS 患者（58%为女性），手术时的平均年龄为 8.6±5.8 岁。17 例患者仅在睡眠时需要呼吸机支持；2 例患者 24 小时需要呼吸机支持。手术平均持续时间为 3.1±0.5 小时。17 例患者在手术室通过气管造口行 PPV 拔管。2 例患者在术后第 1 天行 NPPV 拔管。患者转回家用呼吸机或 NPPV 的平均过渡时间为 3.0±2.2 天，平均住院时间为 5.0±2.1 天。1 例未行通气支持的患者预用药后出现低氧血症和通气不足。10 例（52%）患者术中出现心动过缓、低血压、显著低氧血症和支气管痉挛等事件。15 例患者术后出现并发症。低氧血症、肺炎和肺不张是围手术期并发症的主要原因。1 例患者术后第 2 天因高碳酸血症发生癫痫。
CCHS 患者易受到镇静和麻醉药物的心肺影响。因此，他们在围手术期需要进行严密监测和最佳的通气支持。
