General anesthesia in children with idiopathic pulmonary arterial hypertension (PAH) carries an increased risk of peri-operative cardiorespiratory complications though risk stratifying individual children pre-operatively remains difficult. We report the incidence and echocardiographic risk factors for adverse events in children with PAH undergoing general anesthesia for cardiac catheterization. Echocardiographic, hemodynamic, and adverse event data from consecutive PAH patients are reported. A multivariable predictive model was developed from echocardiographic variables identified by Bayesian univariable logistic regression. Model performance was reported by area under the curve for receiver operating characteristics (AUC_roc) and precision/recall (AUC_pr) and a pre-operative scoring system derived (0–100). Ninety-three children underwent 158 cardiac catheterizations with mean age 8.8 ± 4.6 years. Adverse events (n = 42) occurred in 15 patients (16%) during 16 catheterizations (10%) including cardiopulmonary resuscitation (n = 5, 3%), electrocardiographic changes (n = 3, 2%), significant hypotension (n = 2, 1%), stridor (n = 1, 1%), and death (n = 2, 1%). A multivariable model (age, right ventricular dysfunction, and dilatation, pulmonary and tricuspid regurgitation severity, and maximal velocity) was highly predictive of adverse events (AUC_roc 0.86, 95% CI 0.75 to 1.00; AUC_pr 0.68, 95% CI 0.50 to 0.91; baseline AUC_pr 0.10). Pre-operative risk scores were higher in those who had a subsequent adverse event (median 47, IQR 43 to 53) than in those who did not (median 23, IQR 15 to 33). Pre-operative echocardiography informs the risk of peri-operative adverse events and may therefore be useful both for consent and multi-disciplinary care planning.

尽管在术前对个别儿童进行风险分层仍然很困难，但特发性肺动脉高压（PAH）儿童的全身麻醉会增加围手术期心肺并发症的风险。我们报告了接受全身麻醉心导管检查的 PAH 儿童不良事件的发生率和超声心动图危险因素。报告连续 PAH 患者的超声心动图、血流动力学和不良事件数据。根据贝叶斯单变量逻辑回归确定的超声心动图变量开发了多变量预测模型。模型性能通过受试者操作特征 (AUC _roc ) 和精确度/召回率 (AUC _pr )的曲线下面积以及导出的术前评分系统 (0–100) 来报告。 93 名儿童接受了 158 次心导管检查，平均年龄为 8.8 ± 4.6 岁。 16 次导管插入术 (10%) 期间，15 名患者 (16%) 发生不良事件 ( n = 42)，包括心肺复苏 ( n  = 5, 3%)、心电图变化 ( n  = 3, 2%)、显着低血压 ( n  = 2, 1%)、喘鸣 ( n  = 1, 1%) 和死亡 ( n  = 2, 1%)。多变量模型（年龄、右心室功能障碍和扩张、肺和三尖瓣反流严重程度以及最大流速）可以高度预测不良事件（AUC _roc 0.86，95% CI 0.75 至 1.00；AUC _pr 0.68，95% CI 0.50 至1.00）。 0.91；基线 AUC _pr 0.10）。随后发生不良事件的患者（中位数 47，IQR 43 至 53）的术前风险评分高于未发生不良事件的患者（中位数 23，IQR 15 至 33）。术前超声心动图可告知围手术期不良事件的风险，因此可能对知情同意和多学科护理计划有用。