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+A 53-year-old man came to our hospital with signs and symptoms of acute heart failure after a 2-week history of progressive breathlessness.
+He had a history of recurrent skin abscesses and atopic dermatitis and regularly visited a dermatologist in our hospital.
+On arriving at our hospital, his extremities were warm and dry.
+According to the New York Heart Association criteria, he had class III congestive heart failure (CHF).
+An electrocardiogram revealed diffuse nonspecific T-wave changes, low voltage (<5 mm) in the extremity leads and poor R-wave progression in the anterior chest leads.
+Multiple sporadic ventricular premature beats were seen (Figure 1).
+Chest x-ray film confirmed right pleural effusion, and mild cardiomegaly but no pulmonary congestion (Figure 2).
+Blood tests showed severely elevated B-type natriuretic peptide (901 pg/mL) and markedly raised IgE (12 000 IU/mL) without eosinophilia (eosinophil count of 1.62×108/L).
+Biochemical analysis revealed no significant findings: blood urea nitrogen of 14.3 mg/dL [reference value (RV): 7.00–22.00], creatinine of 0.90 mg/dL (RV: 0.60–1.00), C-reactive protein (CRP) of 0.2 mg/dL (RV: 0.00–0.50), serum amyloid A (SAA) of 7.0 μg/mL (RV: 0–8.0), and troponin T of 0.07 ng/mL (RV: 0–0.1).
+Immunology testing revealed negative perinuclear anti-neutrophil cytoplasmic antibodies and no elevation of myeloperoxidase antibodies.
+The distribution of albumin and globulin in the serum was normal.
+Serum protein immuno-electrophoresis did not reveal M-protein, and urinalysis revealed no Bence-Jones protein.
+Transthoracic echocardiography (Figure 3) showed concentric mild left ventricular (LV) hypertrophy (12 mm) without the characteristic granular sparkling appearance and pericardial effusion, preserved ejection fraction (60%), and bi-atrial enlargement with normal ventricular chambers.
+Doppler-derived LV diastolic filling demonstrated a restrictive pattern with a trans-mitral early filling wave deceleration time of 160 ms and an elevated E/A ratio of 2.8.
+A markedly elevation of E/e’ ratio of 27.3 indicated elevated LV filling pressure.
+On day 1 of hospitalization, we prescribed an angiotensin-converting enzyme (ACE) inhibitor and low-dose diuretics.
+On day 3 of hospitalization, after initiating ACE inhibitor and diuretic therapy, the patient’s symptoms resolved.
+The dermatologists performed a biopsy of a blue macula of the forehead skin.
+On day 4, we introduced a low-dose β-blocker and performed an endomyocardial biopsy (EMB), obtaining 3 fragments of the right ventricular septum because diagnostic confirmation of cardiac amyloidosis requires the demonstration of amyloid deposits.
+In addition, we performed right heart catheterization (RHC), and coronary angiography to exclude obstructive coronary artery disease.
+The data obtained from the RHC indicated subset type IV according to the Forrester classification.
+The right ventricular pressure curve did not show a dip-and-plateau configuration.
+Skin biopsy revealed hyperkeratosis in the epidermis and mild inflammatory changes throughout the dermis with an infiltration of lymphocytes.
+The specimen exhibited apple-green birefringence with polarized light after Congo red staining (Figure 4).
+Histological examination of the myocardial specimen showed no signs suggesting myocarditis, eosinophilic granulomas, or cardiomyopathy with iron deposition, but Congo red staining revealed amyloid deposits (Figure 5).
+In addition, a strongly positive immunohistochemical reaction to immunoglobulin λ-chain in the myocardial interstitium led us to diagnose this patient’s systemic amyloidosis as AL amyloidosis.
+The patient generally tolerated the low-dose β-blocker well and was discharged on day 10 with no complications.
+The patient is currently being followed-up at 6-month intervals as an out-patient, with no exacerbation of the CHF thus far.