PDF(Pubmed)
Abstract：
UNASSIGNED: Exercise stress testing (EST) in pediatric hypertrophic cardiomyopathy (HCM) patients has not well described in a large heterogenous cohort.
UNASSIGNED: The objective of the study was to determine the clinical utility of EST in pediatric HCM.
UNASSIGNED: This was a retrospective single-center analysis of HCM patients younger than 21 years who had EST between January 1, 2000, and January 1, 2019. Clinical, demographic characteristics, and EST data were analyzed, using the last EST during the study or prior to the event in subjects with a primary outcome. The primary composite endpoint included cardiac death, transplant, or arrhythmia requiring implantable cardioverter-defibrillator placement. Outcome analysis was performed using Cox proportional hazard modeling.
UNASSIGNED: The study cohort included 140 patients, 52% with a recognized genetic variant. There were 2 tests aborted due to safety concerns (ST-segment changes, ventricular ectopy). The median age at first EST was 13.6 years. Ninety percent of patients were tested using cycle ergometry, and 44% were on a beta-blocker. The median peak oxygen consumption was 37.1 mL/kg/min (IQR: 12.5 mL/kg/min) or 81.2% predicted, the mean anaerobic threshold was 21.8 Ml (IQR: 8.3 mL), and the median peak power was 2.6 ± 1.1 W/kg or 73.7% predicted. Ectopy during EST was seen in 44% of patients, and 8% had an abnormal blood pressure response to exercise. The endpoint was reached in 12 patients. The presence of any degree of ectopy was a predictor of the composite endpoint (hazard ratio: 5.8; 95% CI: 1.3-26.7).
UNASSIGNED: EST is clinically useful in select pediatric patients with HCM. Ectopy on EST is a risk factor for cardiac death, cardiac transplant, and arrhythmias requiring implantable cardioverter-defibrillator.
UNASSIGNED: The objective of the study was to determine the clinical utility of EST in pediatric HCM.
UNASSIGNED: This was a retrospective single-center analysis of HCM patients younger than 21 years who had EST between January 1, 2000, and January 1, 2019. Clinical, demographic characteristics, and EST data were analyzed, using the last EST during the study or prior to the event in subjects with a primary outcome. The primary composite endpoint included cardiac death, transplant, or arrhythmia requiring implantable cardioverter-defibrillator placement. Outcome analysis was performed using Cox proportional hazard modeling.
UNASSIGNED: The study cohort included 140 patients, 52% with a recognized genetic variant. There were 2 tests aborted due to safety concerns (ST-segment changes, ventricular ectopy). The median age at first EST was 13.6 years. Ninety percent of patients were tested using cycle ergometry, and 44% were on a beta-blocker. The median peak oxygen consumption was 37.1 mL/kg/min (IQR: 12.5 mL/kg/min) or 81.2% predicted, the mean anaerobic threshold was 21.8 Ml (IQR: 8.3 mL), and the median peak power was 2.6 ± 1.1 W/kg or 73.7% predicted. Ectopy during EST was seen in 44% of patients, and 8% had an abnormal blood pressure response to exercise. The endpoint was reached in 12 patients. The presence of any degree of ectopy was a predictor of the composite endpoint (hazard ratio: 5.8; 95% CI: 1.3-26.7).
UNASSIGNED: EST is clinically useful in select pediatric patients with HCM. Ectopy on EST is a risk factor for cardiac death, cardiac transplant, and arrhythmias requiring implantable cardioverter-defibrillator.
摘要：
■小儿肥厚型心肌病（HCM）患者的运动压力测试（EST）在大型异质性队列中尚未得到很好的描述。
■本研究的目的是确定EST在小儿HCM中的临床应用。
■这是对2000年1月1日至2019年1月1日期间患有EST的21岁以下HCM患者的回顾性单中心分析。临床,人口特征，和EST数据进行了分析，在研究期间或事件发生之前，使用具有主要结局的受试者的最后一个EST。主要复合终点包括心脏死亡，移植,或需要植入心脏复律除颤器的心律失常。使用Cox比例风险模型进行结果分析。
■该研究队列包括140名患者，52%具有公认的遗传变异。由于安全问题，有2项测试中止(ST段变化，心室异位）。首次EST的中位年龄为13.6岁。百分之九十的患者使用周期测功进行了测试，44%的患者服用β受体阻滞剂。峰值耗氧量中位数为37.1mL/kg/min（IQR：12.5mL/kg/min）或预测的81.2％，平均无氧阈值为21.8mL（IQR：8.3mL），峰值功率中位数为2.6±1.1W/kg，预测值为73.7%。44％的患者在EST期间出现异位，8%的人对运动有异常的血压反应。12例患者达到终点。任何程度的异位的存在都是复合终点的预测因子（风险比：5.8；95％CI：1.3-26.7）。
■EST对某些患有HCM的儿科患者具有临床有用性。EST的异位性是心脏死亡的危险因素，心脏移植,和心律失常需要植入式心脏复律除颤器。
■本研究的目的是确定EST在小儿HCM中的临床应用。
■这是对2000年1月1日至2019年1月1日期间患有EST的21岁以下HCM患者的回顾性单中心分析。临床,人口特征，和EST数据进行了分析，在研究期间或事件发生之前，使用具有主要结局的受试者的最后一个EST。主要复合终点包括心脏死亡，移植,或需要植入心脏复律除颤器的心律失常。使用Cox比例风险模型进行结果分析。
■该研究队列包括140名患者，52%具有公认的遗传变异。由于安全问题，有2项测试中止(ST段变化，心室异位）。首次EST的中位年龄为13.6岁。百分之九十的患者使用周期测功进行了测试，44%的患者服用β受体阻滞剂。峰值耗氧量中位数为37.1mL/kg/min（IQR：12.5mL/kg/min）或预测的81.2％，平均无氧阈值为21.8mL（IQR：8.3mL），峰值功率中位数为2.6±1.1W/kg，预测值为73.7%。44％的患者在EST期间出现异位，8%的人对运动有异常的血压反应。12例患者达到终点。任何程度的异位的存在都是复合终点的预测因子（风险比：5.8；95％CI：1.3-26.7）。
■EST对某些患有HCM的儿科患者具有临床有用性。EST的异位性是心脏死亡的危险因素，心脏移植,和心律失常需要植入式心脏复律除颤器。
