Myocardial inflammation in Duchenne Muscular Dystrophy as a precipitating factor for heart failure: a prospective study.
Myocardial inflammation in Duchenne Muscular Dystrophy as a precipitating factor for heart failure: a prospective study.
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DOI：
10.1186/1471-2377-10-33
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发表时间：
2010-05-21
期刊：
影响因子：
2.6
通讯作者：
Cokkinos DV
中科院分区：
文献类型：
--
作者：
Mavrogeni S;Papavasiliou A;Spargias K;Constandoulakis P;Papadopoulos G;Karanasios E;Georgakopoulos D;Kolovou G;Demerouti E;Polymeros S;Kaklamanis L;Magoutas A;Papadopoulou E;Markussis V;Cokkinos DV
在杜氏肌营养不良症（DMD）患者中，肌营养不良蛋白的缺失或减少导致进行性骨骼肌和心力衰竭。我们评估了心肌炎症作为DMD心力衰竭发生的促发因素的作用。对20例DMD患者（年龄15-18岁）和20例年龄匹配的健康志愿者进行了为期2年的研究和随访。采用STIR T2加权（T2 W）、T1加权（T1 W）对比剂前后和延迟增强图像（LGE）进行心血管磁共振成像（CMR）评价心肌炎。计算左心室容积和射血分数。对CMR阳性的患者进行心肌活检，并进行免疫组织化学和聚合酶链反应（PCR）分析。在DMD患者中，左心室舒张末期容积（LVEDV）与对照组相比无差异。左心室收缩末期容积（LVESV）较高（45.1 ± 6.6 vs. 37.3 ± 3.8 ml，p < 0.001），左心室射血分数（LVEF）较低（53.9 ± 2.1 vs. 63 ± 2.4%，p < 0.001）。DMD患者的T2心脏/骨骼肌比值和早期T1比值与对照组相比无差异。在6名DMD患者中确定了LGE区域。在其中4例有心肌炎CMR证据的患者中，进行了心肌活检。活动性心肌炎被确定在一个和愈合心肌炎在三个使用免疫组织学。所有6例CMR心肌炎证据的患者在第二年左心室功能迅速恶化。经CMR证实的伴有心肌炎症的DMD患者严重进展为心力衰竭。
In patients with Duchenne Muscular Dystrophy (DMD), the absent or diminished dystrophin leads to progressive skeletal muscle and heart failure. We evaluated the role of myocardial inflammation as a precipitating factor in the development of heart failure in DMD. 20 DMD patients (aged 15-18 yrs) and 20 age-matched healthy volunteers were studied and followed-up for 2 years. Evaluation of myocarditis with cardiovascular magnetic resonance imaging (CMR) was performed using STIR T2-weighted (T2W), T1-weighted (T1W) before and after contrast media and late enhanced images (LGE). Left ventricular volumes and ejection fraction were also calculated. Myocardial biopsy was performed in patients with positive CMR and immunohistologic and polymerase chain reaction (PCR) analysis was employed. In DMD patients, left ventricular end-diastolic volume (LVEDV) was not different compared to controls. Left ventricular end-systolic volume (LVESV) was higher (45.1 ± 6.6 vs. 37.3 ± 3.8 ml, p < 0.001) and left ventricular ejection fraction (LVEF) was lower (53.9 ± 2.1 vs. 63 ± 2.4%, p < 0.001). T2 heart/skeletal muscle ratio and early T1 ratio values in DMD patients presented no difference compared to controls. LGE areas were identified in six DMD patients. In four of them with CMR evidence of myocarditis, myocardial biopsy was performed. Active myocarditis was identified in one and healing myocarditis in three using immunohistology. All six patients with CMR evidence of myocarditis had a rapid deterioration of left ventricular function during the next year. DMD patients with myocardial inflammation documented by CMR had a rigorous progression to heart failure.
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影响因子：
37.8
作者：
PERLOFF, JK;DELEON, AC;ODOHERTY, D
通讯作者：
ODOHERTY, D
影响因子：
24
作者：
Silva, Marly Conceicao;Meira, Zilda Maria Alves;Rochitte, Carlos Eduardo
通讯作者：
Rochitte, Carlos Eduardo
DOI：
10.1002/jmri.1880040113
发表时间：
1994-01-01
期刊：
JMRI-JOURNAL OF MAGNETIC RESONANCE IMAGING
影响因子：
--
作者：
HUANG, YM;MAJUMDAR, S;MILLER, RG
通讯作者：
MILLER, RG
影响因子：
37.8
作者：
KREMASTINOS, DT;TINIAKOS, G;TOUTOUZAS, PK
通讯作者：
TOUTOUZAS, PK
影响因子：
3.1
作者：
Mavrogeni, Sophie;Papavasiliou, Antigoni;Cokkinos, Dennis V.
通讯作者：
Cokkinos, Dennis V.