Department of Internal Medicine, Cleveland Clinic Foundation, Cleveland, Ohio.
Robert and Suzanne Tomsich Department of Cardiovascular Medicine, Sydell, and Arnold Miller Family Heart, Vascular and Thoracic Institute, Cleveland Clinic, Cleveland, Ohio.
Am J Cardiol. 2024 Jul 15;223:100-108. doi: 10.1016/j.amjcard.2024.05.007. Epub 2024 May 11.
In patients with cardiac amyloidosis, pericardial involvement is common, with up to half of patients presenting with pericardial effusions. The pathophysiological mechanisms of pericardial pathology in cardiac amyloidosis include chronic elevations in right-sided filling pressures, myocardial and pericardial inflammation due to cytotoxic effects of amyloid deposits, and renal involvement with subsequent uremia and hypoalbuminemia. The pericardial effusions are typically small; however, several cases of life-threatening cardiac tamponade with hemorrhagic effusions have been described as a presenting clinical scenario. Constrictive pericarditis can also occur due to amyloidosis and its identification presents a clinical challenge in patients with cardiac amyloidosis who concurrently manifest signs of restrictive cardiomyopathy. Multimodality imaging, including echocardiography, cardiac computed tomography, and cardiac magnetic resonance imaging, is useful in the evaluation and management of this patient population. The recognition of pericardial effusion is important in the risk stratification of patients with cardiac amyloidosis as its presence confers a poor prognosis. However, specific treatment aimed at the effusions themselves is seldom indicated. Cardiac tamponade and constrictive pericarditis may necessitate pericardiocentesis and pericardiectomy, respectively.
在患有心脏淀粉样变性的患者中，心包受累很常见，多达一半的患者出现心包积液。心脏淀粉样变性中心包病变的病理生理机制包括右侧充盈压的慢性升高、由于淀粉样沉积物的细胞毒性作用导致的心肌和心包炎症，以及随后的肾受累导致的尿毒症和低白蛋白血症。心包积液通常较小；然而，已有数例危及生命的心脏压塞伴血性积液的病例被描述为临床表现。缩窄性心包炎也可因淀粉样变性而发生，在同时表现出限制性心肌病迹象的心脏淀粉样变性患者中，其识别具有临床挑战性。包括超声心动图、心脏计算机断层扫描和心脏磁共振成像在内的多模态成像可用于评估和管理该患者人群。在心包积液的识别在心衰患者的风险分层中很重要，因为其存在预示着预后不良。然而，针对积液本身的特定治疗很少有指征。心脏压塞和缩窄性心包炎可能需要分别进行心包穿刺和心包切除术。
