Year: 2019
Type: article
Study design: Case Report
Abstract: Description of clinical presentation: A 34 year-old gentleman with no past medical history of interest presented with two month history of nausea and vomiting associated with epigastric pain and weight loss. A CT on admission showed hepatoesplenomegaly and blood tests showed deranged liver function. A liver biopsy demonstrated AL lambda amyloid deposits. To further investigate the possibility of cardiac amyloidosis, a cardiac magnetic resonance (CMR) was performed. Diagnostic techniques and the most important findings: Cine SSFP imaging at presentation showed normal biventricular dimensions with normal systolic function and mass. On tissue characterisation, native T1 and T2 were markedly elevated (1244ms by MOLLI and 64ms respectively), there was diffuse biventricular transmural late gadolinium enhancement (LGE) and extracellular volume fraction (ECV) at 15min was elevated (0.45). The patient started chemotherapy with Velcade, Cyclophosphamide and Dexamethasone achieving a complete response after two months. A follow up CMR 6 months after presentation was performed showing similar ventricular dimensions, function and mass. Native T1 and T2 were significantly lower (1102ms by MOLLI and 50ms respectively) and the LGE pattern improved, showing only subendocardial LGE at basal segments with abnormal gadolinium kinetics. Interestingly, ECV only improved marginally (0.41) despite the improvement in all other parameters. Learning points: At presentation CMR showed very elevated native T1 and T2 values with only moderately elevated ECV values. These findings suggest that, although there are amyloid deposits in the myocardium, oedema is the main pathophysiological mechanism. These findings are even more supported when after achieving a complete haematological response with chemotherapy, native T1 and T2 reduced significantly with only minor reduction in ECV. That probably means that the oedema disappeared and some amyloid deposits remained in the myocardium. Multiparametric mapping is paramount to understand the different pathophysiological mechanisms implicated in cardiac amyloidosis. Abstract P119 Figure. Oedema regression in cardiac amyloidosis
Authors: A Martinez Naharro, T Kotecha, L Chacko, J D Brown, D S Knight, A Steriotis, P Kellman, J D Gillmore, P N Hawkins, M Fontana
Language: English
Subfield: Molecular Biology
Domain: Life Sciences
Open Access status: bronze
OpenAlex ID: W2948651132