Amyloidosis is caused by deposition of proteinaceous material in the extracellular spaces. Cardiac involvement in amyloidosis can lead to heart failure with clinical features not very different from other causes of heart failure (1). The electrocardiographic and imaging features can be nonspecific or appear late which leads to this condition being underdiagnosed until late stages (1). The diagnosis of systemic amyloidosis requires a tissue biopsy with Congo red staining. The imaging features of cardiac amyloidosis include concentric biventricular thickening, prominent valves, and infiltration of the...

We are presenting a patient with confirmed diagnosis of amyloid induced peripheral neuropathy (Sural nerve biopsy) with complain of shortness of breath and fatigue. The 99mTc-PYP scintigraphy was strongly positive for TTR cardiac amyloidosis (H/CL ratio of 1.84) (Figures 1A, 1B, 2) while other imaging techniques (echocardiography and CMR) showed no or very little sign of cardiac amyloidosis (mild thickening of the basal interventricular septum) (Figures 3A, 3B). The following genetics study was positive for TTR gene mutation associated with familial amyloidosis. The diagnosis of...

Although the current gold standard for diagnosis of cardiac amyloidosis and the differentiation of AL from ATTR is invasive endo-myocardial biopsy (4), this may not be necessary if there are typical imaging features of cardiac amyloidosis with a confirmed histological diagnosis from another tissue (2). Cardiac scintigraphy using 99mTc-PYP is a promising technique that can not only detect the presence and extent of cardiac amyloidosis but also can differentiate AL from TTR types of cardiac amyloidosis (5). In this patient, the 99mTc-PYP scintigraphy was strongly...

Cardiac scintigraphy with 99mTc-PYP is a simple non-invasive and widely available imaging technique that can be used to investigate patients with suspected cardiac amyloidosis and has the advantage of differentiating AL from ATTR.

1. Rapezzi C, Merlini G, Quarta CC, Riva L, Longhi S, Leone O, Salvi, F, Ciliberti P, Pastorelli F, Biagini E, Coccolo F, Cooke RM, Bacchi-Reggiani L, Sangiorgi D, Ferlini A, Cavo M, Zamagni E, Fonte ML, Palladini G, Salinaro F, Musca F, Obici L, Branzi A, Perlini S. Systemic cardiac amyloidoses: disease profiles and clinical courses of the 3 main types. Circulation. 2009;120:1203–1212. 2. Falk RH. Contemporary Reviews in Cardiovascular Medicine: Diagnosis and Management of the Cardiac Amyloidoses. Circulation. 2005; 112: 2047-2060 3. Syed...