Single-ventricle congenital heart disease (CHD) in pediatric patients with Glenn and Fontan physiology represents a unique physiology requiring the surgical diversion of the systemic venous return from the superior vena cava (Glenn) and then the inferior vena cava (Fontan) directly to the pulmonary arteries. Because many of these patients are on chronic anticoagulation therapy and may have right-to-left shunts, arrhythmias, or lymphatic disorders that predispose them to bleeding and/or clotting, they are at risk of experiencing neurological injury requiring intubation and positive pressure ventilation, which can significantly hamper pulmonary blood flow and cardiac output. The aim of this study was to describe the complex neurological and cardiopulmonary interactions of these pediatric patients after acute central nervous system (CNS) injury.

The authors retrospectively analyzed the records of pediatric patients who had been admitted to a quaternary children’s hospital with CHD palliated to bidirectional Glenn (BDG) or Fontan circulation and acute CNS injury and who had undergone intubation and mechanical ventilation. Patients who had been admitted from 2005 to 2019 were included in the study. Clinical characteristics, surgical outcomes, cardiovascular and pulmonary data, and intracranial pressure data were collected and analyzed.

Nine pediatric single-ventricle patients met the study inclusion criteria. All had undergone the BDG procedure, and the majority (78%) were status post Fontan palliation. The mean age was 7.4 years (range 1.3–17.3 years). At the time of acute CNS injury, which included traumatic brain injury, intracranial hemorrhage, and cerebral infarct, the median time interval from the most recent cardiac surgical procedure was 3 years (range 2 weeks–11 years). Maintaining normocarbia to mild hypercarbia for most patients during intubation periods did not cause neurological deterioration, and hemodynamic profiles were more favorable as compared to periods of hypocarbia. Hypocarbia was associated with unfavorable hemodynamics but was necessary to decrease intracranial hypertension. Most patients were managed using low mean airway pressure (MAWP) in order to minimize the impact on preload and cardiac output.

The authors highlight the complex neurological and cardiopulmonary interactions with respect to partial pressure of arterial CO₂ (PaCO₂) and MAWP when pediatric CHD patients with single-ventricle physiology require mechanical ventilation. The study data demonstrated that tight control of PaCO₂ and minimizing MAWP with the goal of early extubation may be beneficial in this population. A multidisciplinary team of pediatric critical care intensivists, cardiac intensivists and anesthesiologists, and pediatric neurosurgeons and neurologists are recommended to ensure the best possible outcomes.

具有 Glenn 和 Fontan 生理学的儿科患者的单心室先天性心脏病 (CHD) 代表一种独特的生理学，需要手术将全身静脉回流从上腔静脉 (Glenn) 和下腔静脉 (Fontan) 直接转移到肺动脉。由于这些患者中有许多正在接受长期抗凝治疗，并且可能有右向左分流、心律失常或淋巴系统疾病，使他们容易出血和/或凝血，因此他们有需要插管和正压通气的神经损伤风险，这会显着阻碍肺血流量和心输出量。本研究的目的是描述这些儿科患者在急性中枢神经系统 (CNS) 损伤后复杂的神经和心肺相互作用。

作者回顾性分析了因双向 Glenn (BDG) 或 Fontan 循环和急性 CNS 损伤而入院的四级儿童医院 CHD 和接受插管和机械通气的儿科患者的记录。该研究包括了 2005 年至 2019 年入院的患者。收集并分析了临床特征、手术结果、心血管和肺部数据以及颅内压数据。

九名儿科单心室患者符合研究纳入标准。所有人都接受了 BDG 手术，大多数 (78%) 处于 Fontan 姑息治疗后的状态。平均年龄为 7.4 岁（范围 1.3-17.3 岁）。在急性中枢神经系统损伤时，包括外伤性脑损伤、颅内出血和脑梗塞，与最近一次心脏外科手术的中位时间间隔为 3 年（范围 2 周至 11 年）。大多数患者在插管期间维持正常碳酸血症至轻度高碳酸血症不会导致神经功能恶化，并且与低碳酸血症相比，血流动力学特征更有利。低碳酸血症与不利的血流动力学有关，但对于降低颅内高压是必要的。

作者强调了当具有单心室生理学的儿科 CHD 患者需要机械通气时，与动脉 CO ₂ (PaCO ₂ )分压和 MAWP 相关的复杂的神经和心肺相互作用。研究数据表明，以早期拔管为目标，严格控制 PaCO ₂和最小化 MAWP 可能对这一人群有益。建议由儿科重症监护医师、心脏重症监护医师和麻醉师以及儿科神经外科医生和神经科医师组成的多学科团队，以确保获得最佳结果。