Bhupathi Satya S, Chalasani Sreelatha, Rokey Roxann
Department of Internal Medicine, Marshfield Clinic, WI 54449, USA.
Clin Med Res. 2011 Jun;9(2):92-9. doi: 10.3121/cmr.2010.899. Epub 2010 Sep 17.
Isolated cardiac amyloidosis, or "Stiff Heart Syndrome," is a rare manifestation of amyloidosis. Some degree of cardiac amyloid deposition is common in elderly patients, as reported in prior post-mortem studies; however, isolated cardiac involvement with predominantly cardiac symptoms and no evidence of systemic disease is a rare presentation. Establishing the correct diagnosis, even with the use of extensive testing including amyloid typing, understanding the clinical significance, and management can be challenging in such cases.
孤立性心脏淀粉样变性,即“僵硬心脏综合征”,是淀粉样变性的一种罕见表现形式。正如先前尸检研究报道的那样,一定程度的心脏淀粉样蛋白沉积在老年患者中很常见;然而,以心脏症状为主且无全身疾病证据的孤立性心脏受累情况则较为罕见。即便使用包括淀粉样蛋白分型在内的广泛检测手段来确立正确诊断、理解其临床意义并进行管理,在这类病例中仍具有挑战性。