A Middle-Aged Man Presenting With Progressive Heart Failure, Myopathy, and Monoclonal Gammopathy of Uncertain Significance

Kaspar Broch; Trine Popperud; Einar Gude; Yngvar Fløisand; Ellen-Ann Antal; Gerhard Bosse; Christoffer Jonsrud; Terje Hegard; Silje Skaara; Ahmed Elsais

doi:10.1016/j.jaccas.2020.02.024

A Middle-Aged Man Presenting With Progressive Heart Failure, Myopathy, and Monoclonal Gammopathy of Uncertain Significance

JACC Case Rep. 2020 Apr 8;2(5):785-789. doi: 10.1016/j.jaccas.2020.02.024. eCollection 2020 May.

Authors

Affiliations

¹ Department of Cardiology, Oslo University Hospital Rikshospitalet, Oslo, Norway.
² Department of Neurology, Oslo University Hospital Rikshospitalet, Oslo, Norway.
³ Department of Hematology, Oslo University Hospital Rikshospitalet, Oslo, Norway.
⁴ Department of Pathology, Oslo University Hospital Rikshospitalet, Oslo, Norway.
⁵ Department of Radiology and Nuclear Medicine, Oslo University Hospital Rikshospitalet, Oslo, Norway.
⁶ Department of Medical Genetics, University Hospital of North Norway, Tromsø, Norway.
⁷ Department of Medicine, Telemark Hospital Trust, Notodden Hospital, Notodden, Norway.
⁸ Department of Neurology, Telemark Hospital Trust, Skien, Norway.

Abstract

A 48-year-old man presented with rapidly progressive heart failure and monoclonal gammopathy of uncertain significance. No specific cause was detected on endomyocardial biopsy. As the heart failure worsened, he also developed progressive skeletal myopathy. This provided the clue to the diagnosis, and cardiac function recovered rapidly with cause-directed therapy. (Level of Difficulty: Intermediate.).

Keywords: MGUS, monoclonal gammopathy of uncertain significance; SLONM, sporadic late-onset nemaline myopathy; cardiomyopathy; chronic heart failure; echocardiography; imaging; right-sided catheterization.

Publication types

Case Reports