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A case of repeated misdiagnosis of amyloid cardiomyopathy
*For correspondence: fengxiangfei@xinhuamed.com.cn
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This article was originally published by Wolters Kluwer - Medknow and was migrated to Scientific Scholar after the change of Publisher.
A 65 yr old woman† was transferred to the department of Cardiology, Xinhua hospital, Shanghai, China in September 2018 due to slowly progressive dyspnoea over a 12 month period. She was previously diagnosed with variable angina with dilated cardiomyopathy or pericardial effusion, however, the prescribed diltiazem, furosemide, or spironolactone did not seem to have worked well. Her electrocardiogram showed significant peripheral low-voltage QRS and transthoracic echocardiogram showed diffuse hyper-refractile ‘granular sparkling’ appearance (Fig. 1), apart from left ventricular dysfunction and pericardial effusion. Cardiac magnetic resonance imaging using phase-sensitive inversion recovery showed global subendocardial late gadolinium enhancement typically seen in light chain amyloidosis (Fig. 2). Free light chain κ and λ levels were elevated in serum and urine, respectively, which further confirmed the above diagnosis. Abdominal skin biopsy also showed a pathognomonic yellow-green birefringence under polarized light. Other investigations, such as coronary computed tomography (CT) angiography (mild stenosis), bone marrow biopsy (slight plasmacytosis) and abdominal fat aspirate (negative Congo red staining) were of little diagnostic value. The patient was treated with bortezomib and dexamethasone. At six month follow up, some improvement was found in her symptoms and lung CT.
Conflicts of Interest: None.