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

  • Sleep Breathing Physiology and Disorders • Original Article
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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.

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Data availability

The data that support the findings of the study are not openly available due to patient confidentiality.

Code availability

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Authors and Affiliations

  1. Department of Surgery, University of Texas Southwestern Medical Center, Dallas, TX, USA

    Gloria Y. Chang

  2. Los Angeles, CA, USA

    Tate Salazar

  3. Keck School of Medicine of University of Southern California, Los Angeles, CA, USA

    Abhishek Karnwal, Cathy E. Shin, J. Gordon McComb, Thomas G. Keens & Iris A. Perez

  4. Department of Anesthesia and Critical Care Medicine, Children’s Hospital Los Angeles, Los Angeles, CA, USA

    Abhishek Karnwal

  5. Division of Pediatric Pulmonology and Sleep Medicine, Children’s Hospital Los Angeles, MS 83, 4650 Sunset Blvd, Los Angeles, CA, 900217, USA

    Sheila S. Kun, Josephine Ellashek, Thomas G. Keens & Iris A. Perez

  6. Department of Surgery, Children’s Hospital Los Angeles, Los Angeles, CA, USA

    Cathy E. Shin

  7. Department of Surgery/Division of Neurosurgery, Children’s Hospital Los Angeles, Los Angeles, CA, USA

    J. Gordon McComb

Authors
  1. Gloria Y. Chang
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  2. Tate Salazar
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Contributions

Gloria Y. Chang, Tate Salazar, Abhishek Karnwal, Sheila S. Kun, Josephine Ellashek, Cathy Shin, J. Gordon McComb, Thomas G. Keens, and Iris A. Perez contributed to the study conception and design. Material preparation, data collection, and analysis were performed by Gloria Y. Chang, Tate Salazar, Abhishek Karnwal, Sheila S. Kun, Josephine Ellashek, Cathy Shin, J. Gordon McComb, Thomas G. Keens, and Iris A. Perez. The first draft of the manuscript was written by Gloria Y. Chang, Tate Salazar, Thomas G. Keens, and Iris A. Perez, and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.

Corresponding author

Correspondence to Iris A. Perez.

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The study was approved by the Institutional Review Board (IRB) of Children’s Hospital Los Angeles (IRB# CHLA-16–00249).

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The authors declare no competing interests.

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Chang, G.Y., Salazar, T., Karnwal, A. et al. Perioperative outcomes and the effects of anesthesia in congenital central hypoventilation patients. Sleep Breath 27, 505–510 (2023). https://doi.org/10.1007/s11325-022-02632-z

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  • DOI: https://doi.org/10.1007/s11325-022-02632-z

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