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Background <p>Congenital adrenal cortical hyperplasia (CAH) includes a group of autosomal recessive genetic disorders characterized by impaired synthesis of adrenal corticosteroid hormones. The clinical manifestations associated with CAH commonly include electrolyte imbalance, refractory hypotension, metabolic acidosis, hypoglycemia, and adrenal crisis. The coexistence of endocrine insufficiency and cardiopulmonary abnormalities complicates clinical management in children with CAH undergoing thoracoscopic atrial septal defect (ASD) repair for congenital heart disease. Although thoracoscopic procedures have advantages because of their minimally invasive nature, they also involve risks such as pneumothorax, low-tidal-volume ventilation, and postural changes, which may aggravate hemodynamic fluctuations. These factors considerably increase the difficulty of perioperative management and the risk of complications. Therefore, precise and individualized perioperative anesthesia management is essential. To maintain hemodynamic and respiratory function and ensure surgical safety, comprehensive preoperative assessment, standardized glucocorticoid stress replacement, optimized anesthesia strategies, advanced multi-dimensional monitoring, and careful maintenance of circulatory and internal environmental stability are required.</p> Case presentation <p>This article describes a clinical case of a 21-month-old infant diagnosed with CAH complicated by ASD. After assessment by a multidisciplinary team (MDT), an individualized perioperative anesthesia strategy was developed and applied. Thoracoscopic ASD repair was successfully performed through comprehensive management, including preoperative correction of electrolyte imbalance, intraoperative hormone replacement therapy, continuous monitoring of the internal milieu and hemodynamic stability, and postoperative optimization of pain control and anti-infective therapy. No significant complications occurred during the perioperative period.</p> Conclusion <p>Effective perioperative management should address insufficient and excessive secretion of adrenal cortical hormones, as well as the effects of long-term exogenous hormone therapy in children with CAH undergoing cardiac surgery. In addition, maintenance of endocrine homeostasis and hemodynamic stability is critical for reducing the risk of adverse events. This case further suggests that the clinical value of management lies in integrating endocrine preparation with cardiac anesthesia and postoperative surveillance, rather than in any single isolated intervention.</p>

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Perioperative anesthesia management for a child with congenital adrenal cortical hyperplasia undergoing thoracoscopic repair of atrial septal defect: a case report and literature review

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2026年7月3日

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  • Jiao Wang,
  • Shiqian He,
  • Xiaoyi Pang,
  • Weijie Zhang

摘要

Background

Congenital adrenal cortical hyperplasia (CAH) includes a group of autosomal recessive genetic disorders characterized by impaired synthesis of adrenal corticosteroid hormones. The clinical manifestations associated with CAH commonly include electrolyte imbalance, refractory hypotension, metabolic acidosis, hypoglycemia, and adrenal crisis. The coexistence of endocrine insufficiency and cardiopulmonary abnormalities complicates clinical management in children with CAH undergoing thoracoscopic atrial septal defect (ASD) repair for congenital heart disease. Although thoracoscopic procedures have advantages because of their minimally invasive nature, they also involve risks such as pneumothorax, low-tidal-volume ventilation, and postural changes, which may aggravate hemodynamic fluctuations. These factors considerably increase the difficulty of perioperative management and the risk of complications. Therefore, precise and individualized perioperative anesthesia management is essential. To maintain hemodynamic and respiratory function and ensure surgical safety, comprehensive preoperative assessment, standardized glucocorticoid stress replacement, optimized anesthesia strategies, advanced multi-dimensional monitoring, and careful maintenance of circulatory and internal environmental stability are required.

Case presentation

This article describes a clinical case of a 21-month-old infant diagnosed with CAH complicated by ASD. After assessment by a multidisciplinary team (MDT), an individualized perioperative anesthesia strategy was developed and applied. Thoracoscopic ASD repair was successfully performed through comprehensive management, including preoperative correction of electrolyte imbalance, intraoperative hormone replacement therapy, continuous monitoring of the internal milieu and hemodynamic stability, and postoperative optimization of pain control and anti-infective therapy. No significant complications occurred during the perioperative period.

Conclusion

Effective perioperative management should address insufficient and excessive secretion of adrenal cortical hormones, as well as the effects of long-term exogenous hormone therapy in children with CAH undergoing cardiac surgery. In addition, maintenance of endocrine homeostasis and hemodynamic stability is critical for reducing the risk of adverse events. This case further suggests that the clinical value of management lies in integrating endocrine preparation with cardiac anesthesia and postoperative surveillance, rather than in any single isolated intervention.