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2 Table.. Laboratory Data on First Admission. Fig.. Electrocardiogram on first admission cm 5 kg 30/80 mmhg 8 / Table. LDH -GTP BNP 25 pg/ml 2 ECG Fig. a t r i a l flutter AFL 90 / V V3 R X 54 % Fig % AFL p i l s i c a i n i d e cibenzoline 3 9 c a t h e t e r a b l a t i o n CA AFL CA block line 5.5 sick sinus syndrome SSS Fig AFL S S S 2 3 SSS DDD 8

3 Fig.4. Chest X-ray on second admission. Table.2. Laboratory Data on Second Admission. Fig.2. Echocardiography on first admission. LV: left ventricle, LA: left atrium, Ao: aorta, : pericardial effusion. Fig.3. Myocardial biopsy on first admission (hematoxylin and eosin stain) VVI Fig Table. 2 9

4 Fig.5. Electrocardiogram on second admission. Fig.6. The electrocardiogram when syncope occurred. 4.4 mm.9 mm 63 % / v e n t r i c u l a r t a c h y c a r d i a VT n i f e k a l a n t VT 7 6 nifekalant VT Fig. 6 VT intracardiac defibrillator ICD AST -GTP LAP atrial standstill E C G d i p - Fig. 5 and-plateau Fig. 7 Congo red 70 / Fig. 8 atrial standstill avl V2 V6 R ECG A L CK 20

5 Fig.7. Recording of right ventricular pressurewave. I, II: electrocardiogram (lead Iand II), RV: right ventricular pressure wave. Bence-Jones VT sotalol ICD 440 g 3 Fig.8. Myocardial biopsy on second admission (Congo red stain). AL 5 g 3, 4) Bence-Jones 2

6 6 James 3, 4) 0 2) 3) 5) 6) 9 ) 0) Garcia 5 ) ) Ridolfi 23 3 ECG 2 ) 3) 4) dip-and-plateau 5 ECG 2 ) 77.4 % QS 4.9 % 7.7 % 27.4 % % 6 ) 35.5 % 4.5 % % ECG screw-in lead R Q ST-T 7) 7 ) () AFL SSS ST-T (2) 7) 5 4 (3) (4) 8 ) S S S V T (5) 22

7 8) Fogo A, Virmani R. Cardiac amyloidosis. Prim (6) VT Cardiol 984; 0: ) James TN. Pathology of the cardiac conduction system in amyloidosis. Ann Intern Med 966; 65: amyloidosis. J Electrocardiol 978; : ) Garcia R and Saeed SM. Amyroidosis. Cardiovascular manifestations in five illustrative cases. Arch Intern Med 968; 2: ) Ridolfi RL, Bulkley BH and Hutchins GM. The conduction system in cardiac amyloidosis. Clinical and pathologic features of 23 patients. Am J Med ; 62: ) Paulsen S. Multinodular stenosing cardiovascular tissue. Acta Pathol Microbiol Scand Sec A 974; 82: ) Buja LM, Khoi NB and Roberts WC. Clinically significant cardiac amyloidosis. Clinicopathologic 4) Smith RR and Hutchins GM. Ischemic heart disease findings in 5 patients. Am J Cardiol 970; 26: 394- secondary to amyloidosis of intramyocardial arteries Am J Cardiol 979; 44: ). Amyloidosis. 5),,.. 975; 30: ) Isokane N, Fukushima N, Miyazaki T and Dohi I. A case of sick sinus syndrome in primary systemic amyloidosis with involvement of the conductive 3 3) Kyle RA. Amyloidosis. Circulation 995; 9: ) Falk RH, Comenzo RL and Skinner M. The systemic 979: amyloidoses. N Engl J Med 997; 337: ),. 5) ;. 974; 2: : ),,,,, 6),,,.,,,. Screw-in. 984; 7: lead 7),,... 99; : 999; 48:

8 Abstract A Case of Primary Cardiac Amyloidosis with Various Cardiac Symptoms Masachika Tamura, Kiyoshi Nakazawa, Taishi Mikami, Mariko Arai, Yoshihiro Akashi, Keizou Osada, Akihiko Takagi, Ryouji Kishi, Tomoyuki Kunishima, Masayoshi Sakakibara, Fumihiko Miyake and Akio Kazama 2 A case of amyloidosis with various cardiac symptoms in a 6-year-old female is reported. She started to complain symptoms of cardiac failure from January 200, and was admitted to our hospital on February. Arrhythmias were simply treated with pacemaker implantation (DDD) for Sick Sinus Syndrome, and with catheter ablation for atrial flutter, because no evidence for structural heart disease was revealed with detailed examinations. The patient was discharged in April after the pacemaker setting was changed to VVI mode, due to the gradual atrial lead pacing threshold worsening from unknown cause. In July, the patient was readmitted due to relapsed cardiac failure and newly developed syncope. Twelve lead standard ECG have remarkably changed from the initial admission, which indicated progression of atrial stand-still phenomenon and ventricular muscle disorder. Syncope was diagnosed as from ventricular tachycardia, thus implantable cardioverter defibrillator was implanted. Restudy of cardiac catheter examination demonstrated restricted right ventricular dilatation, and endocardiac biopsy revealed amyloid deposition, leading to the diagnosing cardiac amyloidosis. She was discharged in August, because the cardiac failure symptom was relieved, but readmitted again in September from poor ingestion. During this admission under nutrition adjustment, sudden respiratory distress and cardiogenic shock led to her to death within 0 months after her visit. Autopsy revealed a primary amyloidosis which was believed to be the cause of her heart failure, arrhythmia, and pacing failure. Department of Internal Medicine, Division of Cardiology 2 Department of Pathology St. Marianna University School of Medicine, 2-6- Sugao, Miyamae-ku, Kawasaki 26-85, Japan 24