Mitral and tricuspid stenosis caused by light chain cardiac amyloid deposition

ESC Heart Fail. 2020 Jun;7(3):1130-1135. doi: 10.1002/ehf2.12668. Epub 2020 Mar 16.


Cardiac amyloidosis results in an infiltrative restrictive cardiomyopathy, with a number of characteristic features: biventricular hypertrophy, abnormal myocardial global longitudinal strain with relative apical sparing, biatrial dilation, and small pericardial effusion along with conduction abnormalities. Amyloid deposits leading to hemodynamically significant valvular heart disease are very rare. We describe a rare case of concomitant moderately severe tricuspid and mitral valve stenosis because of ongoing amyloid deposition in a patient with progressive multiple myeloma and fat pad biopsy-proven light chain amyloidosis. Worsening infiltrative cardiomyopathy and valvulopathy despite evidence-based chemotherapy and heart failure pharmacotherapy led to end-stage disease and death. Valvular involvement in cardiac amyloidosis requires early recognition of the underlying disease condition to guide directed medical therapy and prevent its progression. In this instance, valvuloplasty or valve replacement is not a viable option.

Keywords: Light chain amyloid valvulopathy; Light chain cardiac amyloidosis; Mitral stenosis; Tricuspid stenosis.

Publication types

  • Case Reports