Kiyoo Mori

Pericardial Involvement in IgG4-related Disease

We herein report the case of a 65-year-old man with pericardial involvement associated with autoimmune pancreatitis. Chest CT imaging showed pericardial thickening. The patient responded to corticosteroid therapy, and the pericardial thickening resolved. Multiple organs are involved in immunoglobulin G4 (IgG4)-related disease (IgG4-RD); however, only a few cases of IgG4-related chronic constrictive pericarditis have been reported. To our knowledge, this is the first reported case of IgG4-RD with pericardial involvement at an early stage. This case indicates that recognizing pericardial complications in autoimmune pancreatitis is important and that CT imaging may be useful for obtaining the diagnosis and providing follow-up of pericardial lesions in cases of IgG4-RD.

Syncope Due to Paroxysmal Atrioventricular Block in a Patient With Systemic Sclerosis: A Case Report

A 79-year-old woman with systemic sclerosis was admitted to our hospital because of syncope. On admission, electrocardiogram showed progression of intraventricular conduction defect. Chest radiograph showed marked cardiomegaly. Echocardiogram revealed deterioration of left ventricular systolic function. We suspected progressive myocardial disease with Stokes-Adams attack. When we were preparing a temporary pacemaker, paroxysmal atrioventricular block with asystole for 15 seconds and convulsion occurred. Electrophysiological study showed His-ventricular block and sinus node dysfunction. A permanent pacemaker was implanted. In systemic sclerosis, progression of ventricular conduction defect may warrant prompt electrophysiological study and prophylactic pacemaker implantation.

Polymorphic ventricular tachycardia in a patient with hypertrophic cardiomyopathy and digitalis intoxication

We report the case of a 74-year-old woman who presented with recurrent episodes of polymorphic ventricular tachycardia (PVT) with a normal QT interval due to digitalis intoxication (serum digoxin concentration, 5.0 ng/mL) and severe hyperkalemia (serum potassium level, 8.3 mEq/L). In addition, laboratory data showed elevated levels of blood urea nitrogen (54 mg/dL) and serum creatinine (1.57 mg/dL), suggesting dehydration. She had been treated with a combination of digoxin and eplerenone for atrial fibrillation and heart failure. The PVT resolved after treatment for hyperkalemia. Cardiac magnetic resonance imaging and left ventriculography showed left ventricular hypertrophy predominantly in the apex, suggesting apical hypertrophic cardiomyopathy (HCM). We presume that the presence of HCM was related to the occurrence of PVT in this patient with digitalis intoxication and hyperkalemia. <Learning objective: PVT with a normal QT interval caused by digitalis intoxication with hyperkalemia was observed in a patient with HCM treated with digoxin and eplerenone for atrial fibrillation and heart failure. The presence of HCM may be related to the occurrence of PVT. Combination therapy with digoxin and aldosterone receptor antagonist may predispose severe hyperkalemia, and monitoring of serum digitalis concentration and potassium level should be done strictly.>.

Pilsicainide intoxication presenting as left ventricular dyssynchrony in a patient on hemodialysis

We report here a case of pilsicainide intoxication presenting as left ventricular dyssynchrony in a patient who had been treated on hemodialysis. This is the first report that assessed cardiac function during pilsicainide intoxication by left ventriculography and right heart catheterization.

Pericarditis-complicated takotsubo cardiomyopathy in a patient with rheumatoid arthritis

A 64-year-old woman with medication-controlled rheumatoid arthritis (RA) was admitted to our hospital complaining of chest pains. An electrocardiogram showed elevated ST-segments in the inferior leads, and inverted T-waves in the left precordial leads. Left ventriculography demonstrated apical ballooning, and cardiac magnetic resonance imaging demonstrated apical ballooning of the left ventricle, and moderate pericardial effusion. The patient was diagnosed with takotsubo cardiomyopathy (TTC), complicated by pericarditis. In the literature, autoimmune disorders have been associated with TTC. We suggest that this patients pericardial effusion was caused by TTC, and that her coexisting RA might have played a role in the etiology of the significant pericardial fluid accumulation.

Coronary arteriovenous fistulas complicated by complete atrioventricular block: A case report

We report the case of a patient with bilateral coronary arteriovenous fistulas (CAVFs) connecting the right coronary artery and left circumflex coronary artery with the right atrium who had progression of first-degree atrioventricular (AV) block to complete AV block during a 4-year period. The His bundle electrogram revealed that the complete AV block was the result of a block at the level of the AV node. Dipyridamole stress thallium-201 myocardial imaging showed decreased perfusion in the inferoapical wall. Coronary angiography and computed tomography showed fistulas that arose from the AV nodal branch of the right coronary artery and from the distal portion of the circumflex coronary artery and drained into the right atrium. Because the fistulas were small, they were not repaired surgically, and a permanent pacemaker was implanted to treat the complete AV block. We presumed that the complication by complete AV block was due to abnormalities of the arteries feeding the AV node and chronic ischemia resulting from a coronary steal associated with the fistulas. To the best of our knowledge, this is the first report of CAVF complicated by complete AV block.

A case of left ventricular diverticulum: Change of characteristics after myocardial infarction and usefulness of cardiac computed tomography

A 72-year-old man with an old myocardial infarction was admitted to our hospital for cardiac reexamination. He had suffered from an inferior myocardial infarction when he was 60-year-old. The left ventriculogram had then shown a small contractile diverticulum at the apical portion of the left ventricle. Anterior myocardial infarction had recurred when he was 63-year-old. The left ventriculogram performed after the anterior myocardial infarction revealed that the diverticulum had become dilated and non-contractile. On admission, electrocardiography and chest X-ray showed no remarkable changes from the previous studies. Cardiac computed tomography (CT) demonstrated an apical left ventricular diverticulum with narrow communication to the main chamber and myocardial discontinuity of the wall at the site of the diverticulum. Tc-99m tetrofosmin myocardial images showed a perfusion defect in the apex. We presumed that a muscular left ventricular diverticulum had changed to the fibrous type after the anterior myocardial infarction. Cardiac CT imaging provides accurate evaluation of the left ventricular diverticulum and is useful for the differentiation between a left ventricular diverticulum and an aneurysm.

A CASE OF ENDOCARDIAL CUSHION DEFECT IN A 59-YEAR-OLD FEMALE

59才にて生存せるECD (心内膜床欠損症)の1例を報告する. 50才以上のECDの長期生存者の報告が少ないこと,その特異な奇形のため診断上にも種々の問題点があり,これらについて考案する. 8年来の動悸,労作時の呼吸困難にて来院した.胸骨左縁第3肋間に最強点を有するLevine 4/6度の収縮期雑音と, II音肺動脈成分の亢進が認められた.胸部X線像では心拡大(CTR 66%)と肺動脈の拡張蛇行がみられ,入院時の心電図は心房細動,右室負荷の所見であつた.心房細動は,強心薬,利尿薬などの治療後I度房室ブロックを伴う洞調律となつた. MモードUCGでは,心室中隔の奇異性運動,僧帽弁が心室中隔へ入り込む像がとらえられ,心断層図では,心房一次中隔欠損,僧帽弁の変形,亀裂がとらえられ,診断上有用であつた.観血的心カテーテル検査の所見と合わせ,心室中隔欠損,三尖弁亀裂のない不完全型ECDであつた. ECDは幼少時にてほとんどの例が死亡するが,それは,僧帽弁逆流が大量であること,短絡量が多く呼吸器感染に罹患し易いなどの因子が挙げられる.一方,高令者においては,不整脈死が多いとされる.本例では,僧帽弁逆流が少量であること, I度房室ブロックはあるが洞調律を保ち得ていることなどが,長期生存ならしめているものと考えられる.今後,不整脈,心不全,感染症などの合併症に注意し,観察してゆく予定である.