A Young Male Cardiac Sarcoidosis Case with Complete Atrioventricular Block Which was Improved by Corticosteroid Therapy

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1 28 TBLB 99m Tc-tetrofosmin [ ] A Young Male Cardiac Sarcoidosis Case with Complete Atrioventricular Block Which was Improved by Corticosteroid Therapy Yoko Kida 1), Hiromi Tomioka 2,4), Hiroshi Eizawa 1), Hiroshi Kato 1), Kimio Hashimoto 3), Hironobu Iwasaki 2) ABSTRACT Cardiac involvement of sarcoidosis is important because of the high risk of death. Although the diagnosis of myocardial sarcoidosis is often difficult to confirm, early diagnosis and early corticosteroid therapy are important factors to improve the prognosis of cardiac sarcoidosis. We describe herein a case of cardiac sarcoidosis with complete atrioventricular block which was improved by corticosteroid therapy. A 28-year-old Japanese man was referred to our hospital because of an abnormal ECG showing complete atrioventricular block which was detected by a heath check-up. His chest radiographs and computed tomographs revealed consolidation on the right upper lobe. Bronchoscopy was performed and transbronchial lung biopsy showed non-caseating epitheloid cell granulomas. Transthoracic echocardiogram showed the thinning of the basal portion of the ventricular septum and 99m Tc-tetorofosmin scintigram showed decreased uptake on ventricular septal, and inferior and free anterior wall. From these observations the diagnosis of cardiac sarcoidosis was made and prednisolone 30mg/day started. Thereafter, ECG abnormalities gradually improved and now his ECG shows normal sinus rhythms at a dose of 10mg a day of prednisolone. With the improvement of atrioventricular conduction disturbance, abnormalities of cardiac scintigraphy and echocardiogram also improved. [ ] keywords ; Cardiac sarcoidosis, Complete atrioventricular block, Steroid therapy 1) 2) 3) 4) TEL FAX htomy@kobe-nishishimin-hospi.jp 1) Division of Cardiology, Nishi-Kobe Medical Center 2) Division of Respiratory Medicine, Nishi-Kobe Medical Center 3) Division of Pathology, Nishi-Kobe Medical Center 4) Division of Respiratory Medicine, Kobe Nishi City Hospital 39

2 1) ,3) 4) BHL A B cm 68kg 126/86mmHg 58/ ACE X BHL CT B 5 BAL S 1 S 3 TBLB 58 Dd 5.9/Ds 4.0 cm 0.6cm FS 32 LVEF 64 99m Tc-tetrofosmin Hematology WBC Hb Plt Biochemistry CRP GOT GPT LDH BUN Cr Ca 6800 / l 15.2 mg/dl / l 0.2 mg/dl 13 IU/l 16 IU/l 156 IU/l 15 mg/dl 0.8 mg/dl 2.4 mmol/l BAL cell count /ml neu 0.0 lymph 14.0 eosino 0.5 macro 85.5 CD4/CD8 1.3 Urinalysis Glucose Occult blood Protein Normal sediment ACE 9.6 mmol/l Lysozyme 7.2 g/ml Figure 1. Chest X-ray at first examination showing infiltrative shadows in the right upper lung field. Antinuclear antibody ( ) 40

3 Figure 2. Chest CT at first examination showing consolidation in the right upper lobe without bilateral hilar lymphadenopathy. Figure 3. TBLB specimen showing noncaseating epithelioid cell granulomas with multinuclear giant cells. A B Figure 4. Electrocardiograms (A) at first examination showing complete atrioventricular block. (B) after steroid therapy showing first degree atrioventricular block. (C) showing normal sinus rhythms. C 41

4 5) Tc 6) 30mg/day A Figure 5. Ultrasonic echocardiograms showing (A) thinning of the basal portion of the ventricular septum before steroid therapy and (B) improvement of the thinning of the basal portion of the ventricular septum after B A B Figure 6. 99m Tc-tetorofosmin scintigrams showing (A) spotty decreased uptake (arrow) of septal and inferior wall and part of free anterior wall before steroid therapy and (B) no defect after steroid therapy. 42

5 ( ) ( ) mg10mg/day 6) 5 10mg/day TBLB 20 7) 8-16) Tc Tl-Cl 99m Tctetorofosmin 99m Tc-MIBI 123 I- Table 2. Cases of steroid therapy for cardiac sarcoidosis Year Auther # of cases response rate ( ) first dose of PSL 1982 Suda mg(+pacemaker) 1986 Matsumoto mg 1989 Orita mg 1990 Fujita unknown 1990 Ohkita mg 1992 Noda mg every other day 1998 Ehara mg 1999 Okada mg 2000 Uemura mg every other day 2000 Uetani unknown 2002 Takeda mg 2002 Asada mg 2003 Kato mg MIBG 123 I-BMIPP 201 Tl-Cl 99m Tc-tetrofosimin 99m Tc-MIBI 9,11,12) 99m Tc-tetrofosimin FDG-PET 9,11,14,15) 7,9,11,16,17) 18) retrospective study 19,20) Kato ) 7,19,20) 43

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