. 2023 May;27(2):505-510.

doi: 10.1007/s11325-022-02632-z. Epub 2022 May 13.

Perioperative outcomes and the effects of anesthesia in congenital central hypoventilation patients

Gloria Y Chang¹, Tate Salazar², Abhishek Karnwal^{3

4}, Sheila S Kun⁵, Josephine Ellashek⁵, Cathy E Shin^{3

6}, J Gordon McComb^{3

7}, Thomas G Keens^{3

5}, Iris A Perez^{8

9}

Affiliations

¹ Department of Surgery, University of Texas Southwestern Medical Center, Dallas, TX, USA.
² , Los Angeles, CA, USA.
³ Keck School of Medicine of University of Southern California, Los Angeles, CA, USA.
⁴ Department of Anesthesia and Critical Care Medicine, Children's Hospital Los Angeles, Los Angeles, CA, USA.
⁵ Division of Pediatric Pulmonology and Sleep Medicine, Children's Hospital Los Angeles, MS 83, 4650 Sunset Blvd, Los Angeles, CA, 900217, USA.
⁶ Department of Surgery, Children's Hospital Los Angeles, Los Angeles, CA, USA.
⁷ Department of Surgery/Division of Neurosurgery, Children's Hospital Los Angeles, Los Angeles, CA, USA.
⁸ Keck School of Medicine of University of Southern California, Los Angeles, CA, USA. iaperez@chla.usc.edu.
⁹ Division of Pediatric Pulmonology and Sleep Medicine, Children's Hospital Los Angeles, MS 83, 4650 Sunset Blvd, Los Angeles, CA, 900217, USA. iaperez@chla.usc.edu.

PMID: 35554785
DOI: 10.1007/s11325-022-02632-z

Perioperative outcomes and the effects of anesthesia in congenital central hypoventilation patients

Gloria Y Chang et al. Sleep Breath. 2023 May.

. 2023 May;27(2):505-510.

doi: 10.1007/s11325-022-02632-z. Epub 2022 May 13.

Authors

Gloria Y Chang¹, Tate Salazar², Abhishek Karnwal^{3

4}, Sheila S Kun⁵, Josephine Ellashek⁵, Cathy E Shin^{3

6}, J Gordon McComb^{3

7}, Thomas G Keens^{3

5}, Iris A Perez^{8

9}

Affiliations

¹ Department of Surgery, University of Texas Southwestern Medical Center, Dallas, TX, USA.
² , Los Angeles, CA, USA.
³ Keck School of Medicine of University of Southern California, Los Angeles, CA, USA.
⁴ Department of Anesthesia and Critical Care Medicine, Children's Hospital Los Angeles, Los Angeles, CA, USA.
⁵ Division of Pediatric Pulmonology and Sleep Medicine, Children's Hospital Los Angeles, MS 83, 4650 Sunset Blvd, Los Angeles, CA, 900217, USA.
⁶ Department of Surgery, Children's Hospital Los Angeles, Los Angeles, CA, USA.
⁷ Department of Surgery/Division of Neurosurgery, Children's Hospital Los Angeles, Los Angeles, CA, USA.
⁸ Keck School of Medicine of University of Southern California, Los Angeles, CA, USA. iaperez@chla.usc.edu.
⁹ Division of Pediatric Pulmonology and Sleep Medicine, Children's Hospital Los Angeles, MS 83, 4650 Sunset Blvd, Los Angeles, CA, 900217, USA. iaperez@chla.usc.edu.

PMID: 35554785
DOI: 10.1007/s11325-022-02632-z

Abstract

Purpose: 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.

Methods: 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.

Results: 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.

Conclusion: 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.

Keywords: Anesthesia complications; Congenital central hypoventilation syndrome; Intraoperative; Perioperative; Ventilation.

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Perioperative outcomes and the effects of anesthesia in congenital central hypoventilation patients

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Perioperative outcomes and the effects of anesthesia in congenital central hypoventilation patients

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