Al-Shammari Ahmad A, Muslim Rawan Al, Almuslim Jenan, Elashaal Ehab, Lardhi Haitham, AlQahtani Saleh A, AlBassam Bassam N, Lardhi Amer
Department of Pediatrics, College of Medicine, Imam Abdulrahman Bin Faisal University, Dammam, Saudi Arabia.
King Fahd Hospital of the University, Khobar, Saudi Arabia.
Front Pediatr. 2022 Jul 19;10:944627. doi: 10.3389/fped.2022.944627. eCollection 2022.
Restrictive cardiomyopathy (RCM) is a rare disease in children, accounting for <5% of all pediatric cardiomyopathies. It may be idiopathic or may be a secondary to a systemic disease. The disease is characterized by normal systolic function with impaired ventricular filling caused by stiff ventricular walls. Children with RCM often present with symptoms of exercise intolerance, shortness of breath, weakness, and chest discomfort. Thromboembolism events are an unusual presentation of RCM. We are reporting a preadolescent female from the eastern province of Saudi Arabia who presented with sudden right lower limb swelling, paresthesia, and pain caused by a complete occlusion of the terminal part of the abdominal aorta and both common iliac arteries. Echocardiography revealed dilated atria, normal ventricle dimensions and two floating thrombi in the left atrium. The patient was successfully managed with an anticoagulant, surgical thrombectomy and cardiac transplantation.
限制型心肌病（RCM）在儿童中是一种罕见疾病，占所有小儿心肌病的比例不到5%。它可能是特发性的，也可能继发于全身性疾病。该疾病的特征是收缩功能正常，但由于心室壁僵硬导致心室充盈受损。患有RCM的儿童常表现出运动不耐受、呼吸急促、虚弱和胸部不适等症状。血栓栓塞事件是RCM的一种不常见表现。我们报告了一名来自沙特阿拉伯东部省份的青春期前女性，她因腹主动脉末端和双侧髂总动脉完全闭塞而出现右下肢突然肿胀、感觉异常和疼痛。超声心动图显示心房扩大、心室大小正常，左心房有两个漂浮血栓。该患者通过抗凝、手术取栓和心脏移植成功得到治疗。
