Brugts Jasper J, Houtgraaf Jaco, Hazenberg Bouke Pc, Kofflard Marcel Jm
Jasper J Brugts, Jaco Houtgraaf, Marcel JM Kofflard, Department of Cardiology, Albert Schweitzer hospital, 3300 AK Dordrecht, The Netherlands.
World J Cardiol. 2013 May 26;5(5):154-6. doi: 10.4330/wjc.v5.i5.154.
We present the case of a 66 year old male who presented with dyspnea and reduced exercise tolerance. Echocardiography demonstrated impaired left ventricular (LV) function and restrictive diastolic function with pronounced concentric left ventricular hypertrophy (LVH) without a history of hypertension and no aortic valve stenosis. Differential diagnostics of concentric LVH are discussed in detail. In the current case, cardiac amyloidosis (AL) amyloidosis was diagnosed and confirmed by serum amyloid P (SAP) scintigraphy and abdominal fat aspiration biopsy. This case shows the rapid decline in clinical condition with progression of cardiac involvement of AL. As discussed in detail, cardiac involvement in AL-amyloidosis generally denotes a poor prognosis, regardless of the method of treatment.
我们报告一例66岁男性患者,其表现为呼吸困难和运动耐量下降。超声心动图显示左心室(LV)功能受损和舒张功能受限,伴有明显的同心性左心室肥厚(LVH),无高血压病史且无主动脉瓣狭窄。详细讨论了同心性LVH的鉴别诊断。在本病例中,通过血清淀粉样蛋白P(SAP)闪烁扫描和腹部脂肪抽吸活检诊断并确诊为心脏淀粉样变性(AL)淀粉样变性。该病例显示随着AL心脏受累的进展,临床状况迅速恶化。如详细讨论的那样,无论治疗方法如何,AL淀粉样变性的心脏受累通常预示预后不良。