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患者は51歳,男性.主訴は呼吸困難.27歳時にネフローゼ症候群,高血圧の診断を受け,42歳時に透析導入となった(心胸郭比44%).48歳時,心不全にて当院初診(心胸郭比60%).心電図は洞調律,ST-T変化を伴う左室肥大あり.心エコーでは左室壁厚13mm(対称性),左室拡張末期径80mm,左室内径短縮率7%であり,少量のpimobendanとdenopamineにて症状は改善.しかし,50歳時より心不全が悪化した(血漿BNP8,800pg/ml).一時軽快するも(血漿BNP6,000pg/ml),今回51歳時,心不全が再増悪し入院(血漿BNP16,300pg/ml.心胸郭比は71%,心電図ではQRSの波高の低下およびQRS幅の増大が認められ,心エコーでは左室壁厚が菲薄化(8mm),左室拡張末期径はさらに増大(94mm),左室内径短縮率4%となった.病状は難治性であり,2ヵ月後には心室頻拍を発症し(血漿BNP33,500pg/ml),3ヵ月後には心室細動となり死亡.血漿BNPは心拡大とともに著明に増加した.
A 51-year-old male presented with dyspnea at rest. His past history revealed nephrotic syndrome and hyper-tension at the age of 23 and initiation to hemodialysis at the age of 42 (CTR ; cardiothoracic ratio 44%). At the age of 48 years, he visited our hospital for the first time because of congestive heart failure (CTR 60%). ECG showed sinus rhythm and left ventricular hypertrophy with a strained pattern. Echocardiography revealed left ventricular dilatation, concentric left ventricular hyper-trophy and decreased systolic function. Oral use of pimobendan (1.25 mg/day) and denopamine (10 mg/day) was effective until he reached 50 years of age. when congestive heart failure reappeared with a plasma BNP concentration of 8,800 pg/ml. As his heart failure had further deteriorated by the age of 51 years (CTR 71%), he was admitted to hospital. Plasma BNP concentration was elevated up to 16,300 pg/ml. ECG showed dimini-shed and wide QRS configuration. Echo-cardiography revealed severe left ventricular dilatation, severely de-creased systolic function and myocardial wall thinning. Ventricular tachycardia occurred when plasma BNP concentration reached 33,500 pg/ml. His general condi-tion deteriorated and he died suddenly of ventricular fibrillation in spite of amiodarone administration 3 months after admission.
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