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+A 60-year-old Caucasian woman with no significant medical history developed exercise intolerance, fatigue and shortness of breath on exertion over the several months prior to her presentation to our outpatient clinic.
+At the time of presentation, she also reported of dry cough, orthopnea, paroxysmal nocturnal dyspnoea, generalised weakness and intermittent numbness of her hands and feet.
+Social history was negative for smoking, alcohol or illicit drugs.
+Physical examination was unremarkable, except for jugular venous distension, audible S1 and S2 sounds, bilateral crackles on lung auscultation and bilateral pitting oedema.
+Chest X-ray showed cardiomegaly, pulmonary oedema and small-sized bilateral pleural effusions.
+A 12-lead ECG demonstrated normal sinus rhythm, low-voltage complexes and extreme right-axis deviation (figure 1).
+Transthoracic echocardiogram (TTE) demonstrated asymmetric biventricular hypertrophy with preserved ejection fraction of 60%, elevated right-ventricular systolic pressure of 36 mm Hg and abnormal myocardial texture, described as ‘granular sparkling’ (figure 2).
+Coronary CT angiogram demonstrated normal coronary arteries.
+Subsequently, cardiac MRI demonstrated early and diffuse subendocardial delayed enhancement, concerning for infiltrative myocardial disease and for diffuse biventricular hypertrophy, with normal ejection fraction (figure 3).
+The patient underwent right heart catheterisation with endomyocardial biopsy, revealing diffuse amyloidosis with amorphous proteinaceous material around cardiac myocytes and within blood vessels, which was positive for Congo red stain (figure 4).
+Subsequent laboratory evaluation registered elevated, free λ light chains (86.3 mg/L (normal: 5.7–26.3 mg/L)) and positive Bence-Jones protein (0.37 g/24 h) in the urine.
+Bone marrow biopsy revealed greater than 10% infiltration of CD19− CD56+ CD138+ plasma cells with reversal of marrow κ/λ ratio (<1:2, normal: 2:1), consistent with MM.
+The patient was eventually diagnosed with systemic AL amyloidosis with advanced, stage III, cardiac amyloidosis due to underlying MM.
+Given her advanced cardiac amyloidosis, the patient was deemed a poor candidate for autologous stem-cell transplantation.
+The patient was started on systemic chemotherapy with melphalan and dexamethasone.
+Despite receiving standard therapy for heart failure—including diuretics, β-blockers and ACE inhibitors—the patient's condition continued to worsen and she succumbed to sudden cardiac death.