Ikuta Yoji, Miura Masaru, Goto Tomohide, Miyama Sahoko
Higashi-Koganei Child Neurology and Epilepsy Clinic, Koganei, Japan.
Department of Neurology, Tokyo Metropolitan Children's Medical Center, Fuchu, Japan.
Front Pediatr. 2022 May 10;10:889752. doi: 10.3389/fped.2022.889752. eCollection 2022.
Ventricular hypertrophy is a well-known side effect of adrenocorticotropic hormone (ACTH) therapy in patients with West syndrome (WS), but there are only a few reports of echocardiographic evaluation of these patients' diastolic function.
The present, retrospective study analyzed echocardiographic findings in 24 patients with WS treated with ACTH therapy between April 2010 and December 2014. The therapy protocol involved administering tetracosactide acetate 0.01-0.0125 mg/kg via intramuscular injection once a day for weeks 1-2, then gradually tapering off. Echocardiographic evaluation was done before treatment initiation and at weeks 1, 2, and 4 after the initiation of treatment.
The systolic and diastolic blood pressure values were elevated at week 1 after commencement of the therapy and remained elevated throughout its duration. Both the interventricular septal end-diastolic thickness and left ventricular posterior wall end-diastolic diameter increased in thickness at week 1 and remained thickened. None of the patients experienced heart failure or systolic dysfunction. Early diastolic mitral flow velocity (E)/early diastolic mitral annular velocity (E') increased at week 1 and remained high at weeks 2 and 4. The E wave deceleration time (DcT) was prolonged at week 2 and returned to the baseline at week 4.
Increased ventricular wall thickness, decreased diastolic capacity, and elevated BP were noted in children with WS during ACTH therapy. Cardiac function, including ventricular wall thickness and diastolic function, should be monitored during ACTH therapy. E/E' and DcT are useful in evaluating diastolic function.
心室肥厚是韦斯特综合征（WS）患者接受促肾上腺皮质激素（ACTH）治疗时众所周知的副作用，但仅有少数关于这些患者舒张功能的超声心动图评估报告。
本回顾性研究分析了2010年4月至2014年12月期间接受ACTH治疗的24例WS患者的超声心动图检查结果。治疗方案包括在第1 - 2周每天通过肌肉注射给予醋酸二十四肽促皮质素0.01 - 0.0125 mg/kg，然后逐渐减量。在治疗开始前以及治疗开始后的第1、2和4周进行超声心动图评估。
治疗开始后第1周收缩压和舒张压值升高，并在整个治疗期间持续升高。室间隔舒张末期厚度和左心室后壁舒张末期直径在第1周均增厚，并持续增厚。所有患者均未发生心力衰竭或收缩功能障碍。舒张早期二尖瓣血流速度（E）/舒张早期二尖瓣环速度（E'）在第1周升高，并在第2和4周保持高位。E波减速时间（DcT）在第2周延长，并在第4周恢复至基线水平。
在接受ACTH治疗的WS患儿中观察到心室壁厚度增加、舒张功能降低和血压升高。在ACTH治疗期间应监测心脏功能，包括心室壁厚度和舒张功能。E/E'和DcT有助于评估舒张功能。
