洞不全症候群に対してペースメーカ植え込み術を施行した心アミロイドーシスの1例

A Patient with Cardiac Amyloidosis who Underwent Pacemaker Implantation for Sick Sinus Syndrome

　症例は60歳男性．労作時呼吸困難を主訴として入院となり，胸部X線写真で心拡大と右側胸水貯留を認め心不全と診断した．心電図では，左軸偏位，V_1〜3のST上昇，V_4〜6の深いS波を認め，心エコーでは心筋肥厚，心嚢液貯留を認めた．心臓カテーテル検査を行ったところ，両心室の収縮能は正常であったが，拡張障害を認めた．心筋生検の結果，アミロイドが検出され心アミロイドーシスと診断した．心不全に対し，利尿剤，血管拡張剤の投与を行ったが改善傾向なく，洞不全症候群を合併し心不全が増悪した．一時的ペーシングを行ったところ，胸水は消失し心不全は改善された．後日，恒久的ペースメーカ植え込み術を行い，心不全のコントロールがついた．心アミロイドーシスは根治療法のない進行性の疾患である．今回VVIペーシングが心不全の管理に有用であったが，一時的なものであると考えられ，今後も慎重な経過観察が必要であると思われる．

A 60-year-old male was hospitalized due to exer-tional dvspnea. Chest X-ray revealed cardiac dilatation and retention of pleural effusion on the right side, sug-gesting heart failure. Electrocardiogram showed left axis deviation, ST-segment elevation in lead V₁ through V₃, and deep S wave in lead V₄ through V₆. Echocardio-graphy revealed myocardial thickening and retention of pericardial effusion. Cardiac catheter examination revealed normal for the left and right ventricular con-traction capacities, but the hemodynamics study showed a restrictive phenomenon. Myocardial biopsy showed the deposit of amyloid fiblil, so we diagnosed the patient to have cardiac amyloidosis. Diuretics and vasodilator agents were administered to treat heart failure, but resulting in no improvement. Concurrently sick sinus syndrome developed, resulting in exacerbation of heart failure. Shortly after temporary pacing was performed, pleural effusion disappeared, and heart function im-proved. A few days later, permanent pacemaker im-plantation was performed, and heart failure was suc-cessfully controlled. Cardiac amyloidosis is a progres-sive disease for which radical treatment has not yet been established. In the present case, VVI pacing was useful for controlling heart failure. However,the effect of VVI pacing may be transient, so further careful follow-up is necessary.