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Natallia Laptseva, Michel Zuber, Peter K. Bode, Andreas J. Flammer, Cardiac amyloidosis: still challenging, European Heart Journal, Volume 38, Issue 2, 7 January 2017, Page 122, https://doi.org/10.1093/eurheartj/ehw290
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A 67-year-old men complaining of rapidly progressive dyspnoea was hospitalized with a first episode of biventricular cardiac decompensation at a local hospital. Initial evaluation revealed a hypertrophic ventricle with preserved ejection fraction, a restrictive filling pattern and dilated atria as well as a diffuse and patchy midventricular late gadolinium enhancement in MRI (Panel A). Therefore, some form of hypertrophic or restrictive cardiomyopathy was suspected. No signs for systemic diseases were found, particularly not for haemochromocytosis or multiple myeloma (immune-electrophoresis). Symptomatic heart failure treatment was initiated with a loop diuretic and a follow-up with a cardiologist was scheduled.
Echocardiography some weeks later showed symmetrical thickening of the left and right ventricles with normal ejection fraction, an increased echogenicity with a granular sparkling appearance, as well as dilated and immobile atria (Panel B; Supplementary material online, Video S1). Furthermore, LV-inflow pattern, tissue Doppler images, and pulmonary vein inflow was notable for severe diastolic dysfunction (restrictive filling pattern; Panel C). Cardiac amyloidosis was suspected and the patient was referred to the ‘amyloidosis network’ of the University Hospital Zurich for endomyocardial biopsy. Although cardiac biopsies showed an increased interstitial matrix (Panel D, left), Congo-Red staining did not show typical green birefringence by polarizing microscopy and immunohistochemical stainings for AL amyloid and transthyretin-related amyloid were negative. However, in electron microscope the morphology and arrangement of the interstitial fibrils were characteristic for amyloid (Panel D, right). Typical for amyloidosis, an advanced 3D-echocardiography then clearly demonstrated a baso-apical gradient with preserved systolic function at the apex, a relative apical sparing in speckle-tracking longitudinal strain pattern (Panel E; Supplementary material online, Video S2), and decreased LV torsion (Panel F).
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