title: Echocardiographic features of an atypical presentation of rapidly progressive cardiac amyloidosis.
abstract: 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.
authors.affiliation: Jasper J Brugts, Jaco Houtgraaf, Marcel JM Kofflard, Department of Cardiology, Albert Schweitzer hospital, 3300 AK Dordrecht, The Netherlands.
authors.forename: Jasper J
authors.initials: JJ
authors.lastname: Brugts
authors.forename: Jaco
authors.initials: J
authors.lastname: Houtgraaf
authors.forename: Bouke Pc
authors.initials: BP
authors.lastname: Hazenberg
authors.forename: Marcel Jm
authors.initials: MJ
authors.lastname: Kofflard
keywords: Amyloidosis; Cardiac involvement; Echocardiography; Prognosis; Treatment