37 〔原著〕 左室肥大を呈する心臓サルコイドーシス 左室肥大を呈する心臓サルコイドーシスの臨床的・病理学的特徴 土田哲人 1) ,縣 潤 1) ,南場雅章 1) ,遠藤利昭 1) ,安藤利昭 1) , 四十坊典晴 2) ,佐々木真由美 3) ,飯村 攻 1) ,平賀洋明 2) 【要旨】 心臓サルコイドーシス(心サ症)では心室中隔の障害とこれによる菲薄化が特徴的であるが,左室肥大例も報告されてい る.そこで今回,サ症確定診断例において心臓超音波法および心筋シンチグラム上異常所見を示した連続70症例を対象に, 左室肥大例の出現頻度,臨床的・病理学的特徴について解析した.左室肥大は20例(29%)に認められ,中隔菲薄型31例 (44 %)に次いで多い形態異常であった.超音波上,特発性肥大型心筋症(HCM)と類似した左室中隔肥厚(ASH)を伴う 例が17例あり,また多くの例で中隔基部の輝度上昇を伴っていた.10症例において心筋生検を施行したが,光顕上,心筋細 胞肥大,間質の線維化および脂肪変性を認めたがHCMに特徴的な心筋錯綜配列は認めなかった.心サ症において左室肥大を 示す例は稀ではないが,形態的にはASHを示す症例が多く,HCMとの鑑別が問題になると考えられた. [日サ会誌 2004;24:37-41] キーワード: 心臓サルコイドーシス,左室肥大,非対称性中隔肥大,肥大型心筋症 ………………………………………………………………………………………………………………… Clinical and Pathological Characteristics of Cardiac Sarcoidosis with Left Ventricular Hypertrophy Akihito Tsuchida 1) , Jun Agata 1) , Masaaki Nannba 1) , Toshiaki Endo 1) , Toshiaki Andoh 1) , Noriharu Shijubo 2) , Mayumi Sasaki 3) , Osamu Iimura 1) , Youmei Hiraga 2) 【ABSTRACT】 Left ventricular hypertrophy has been reportedly shown in cases of sarcoidosis. We investigated the frequency, and clinical and morphological characteristics of left ventricular hypertrophy in 70 patients with cardiac sarcoidosis who showed abnormali- ties in echocardiogram and myocardial scintigrams. Left ventricular hypertrophy was found in 20 (29%) of the 70 patients, and the most-frequently observed morphological abnormalities followed the thinning of the septum (29 cases, 41%). Asymmetrical septal hypertrophy such as hypertrophic cardiomyopathy (HCM) was found in 17 of the 20 cases. A hyperechoic area was found in the sepal wall in most of the cases. Myocardial biopsy was performed in 10 cases, and pathological findings revealed myocar- dial hypertrophy, fatty degeneration, and fibrosis, but there was no clear evidence of disarrangement of myocardial cells as seen in HCM. Left ventricular hypertrophy was not unusual in the patients with cardiac sarcoidosis. However, it is difficult to differ- entiate sarcoidosis from HCM on the basis of morphology and pathological findings. [JJSOG 2004;24:37-41] keywords ; Cardiac sarcoidosis, Left ventricular hypertrophy, Asymmetrical septal hypertrophy, Hypertrophic cardiomyopathy 1) 札幌鉄道病院 循環器科 2)同 呼吸器科 3)同 病理科 著者連絡先:土田哲人 060-0033 札幌市中央区北3条東1丁目 札幌鉄道病院循環器科 TEL:011-241-4971 FAX:011-222-9260 E-mail:[email protected]1) Division of Cardiology, Sapporo Hospital of Hokkaido Railway Company 2) Division of Respiratory Medicine, Sapporo Hospital of Hokkaido Railway Company 3) Division of Pathology, Sapporo Hospital of Hokkaido Railway Company …………………………………………………………………………………………………………………
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Clinical and Pathological Characteristics of Cardiac Sarcoidosis with Left Ventricular HypertrophyAkihito Tsuchida1), Jun Agata1), Masaaki Nannba1), Toshiaki Endo1), Toshiaki Andoh1), Noriharu Shijubo2), Mayumi Sasaki3), Osamu Iimura1), Youmei Hiraga2)
【ABSTRACT】 Left ventricular hypertrophy has been reportedly shown in cases of sarcoidosis. We investigated the frequency, and clinicaland morphological characteristics of left ventricular hypertrophy in 70 patients with cardiac sarcoidosis who showed abnormali-ties in echocardiogram and myocardial scintigrams. Left ventricular hypertrophy was found in 20 (29%) of the 70 patients, andthe most-frequently observed morphological abnormalities followed the thinning of the septum (29 cases, 41%). Asymmetricalseptal hypertrophy such as hypertrophic cardiomyopathy (HCM) was found in 17 of the 20 cases. A hyperechoic area was foundin the sepal wall in most of the cases. Myocardial biopsy was performed in 10 cases, and pathological findings revealed myocar-dial hypertrophy, fatty degeneration, and fibrosis, but there was no clear evidence of disarrangement of myocardial cells as seenin HCM. Left ventricular hypertrophy was not unusual in the patients with cardiac sarcoidosis. However, it is difficult to differ-entiate sarcoidosis from HCM on the basis of morphology and pathological findings.
Table 1. Morphological pattern (Maron claccification), known hypertensive (HT), family history of cardiopmopathy, several cardiac functional indicators obtained by echocardiogram; left ventricular ejection fraction (LVEF)(%), E/A, deceleration time (DcT), the level of angiotensin-converting enzyme (ACE) activity and C-reactive protein (CRP) in all cases and the finding of scintigraphy.
Figure 2. A case with septal wall hypertrophy. Panel A: Echocardiography. Panel B: Myocardial biopsy speci-men showing myocardial cell hyper-trophy and fatty cell infiltration.
Figure 3. A case of left ventricular hypertrophy with mid-ventricular obstruction (MVO). Panel A: echocardiography. Panel B: Myocardial biopsy specimens showing fibrous change (left) and fatty change (right). Panel C: Left ventriculography showing mid-ventricular obstruction in end-systolic phase.
Table 2. Summary of pathohistological finding in myocardial biopsy.Morphological semi-quantitive analysis: ( - ):non-detectable, (+):mild, (2+):moderate, (3+):severe changes.
引用文献1) Sekiguchi M, Yazaki Y, Isobe M, et al: Cardiac sarcoidosis:
diagnostic, prognostic, and therapeutic considerations. Car-diovasc Drugs Ther 1996;10: 490-510.
2) Yazaki Y, Isobe M, Hayasaka M, et al.: Cardiac sarcoidosismimicking hypertrophic cardiomyopathy -clinical utility ofradionuclide imaging for differential diagnosis-. Jpn Circ J1998; 62:465-468.
3) Matsumori A, Hara M, Nagai S, et al.: Hypertrophic cardi-omyopathy as a manifestation of cardiac sarcoidosis. JpnCirc J 2000; 64: 679-683.
4) Lewin RF, Mor R, Spitzer S, et al: Echocardiographic evalu-ation of patients with systemic sarcoidosis. Am Heart J1985; 110: 116-122.
5) Yazaki Y, Isobe M, Hiroe M, et al.: Prognostic determinantsof long-term survival in Japanese patients with cardiac sar-coidosis treated with prednisone. Am J Cardiol 2001; 88:1006-1010.
6) Umetani K, Ishihara T, Yamamoto K, et al.: Successfullytreated complete atrioventricular block with corticosteroid ina patient with cardiac sarcoidosis: usefulness of Gallium-67and Thallium-201 scintigraphy. Int Med 2000; 39: 245-248.