Quantification of diffuse myocardial fibrosis and its association with myocardial dysfunction in congenital heart disease.
Quantification of diffuse myocardial fibrosis and its association with myocardial dysfunction in congenital heart disease.
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DOI：
10.1161/circimaging.108.842096
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发表时间：
2010-11
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影响因子：
--
通讯作者：
Jerosch-Herold M
中科院分区：
文献类型：
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作者：
Broberg CS;Chugh SS;Conklin C;Sahn DJ;Jerosch-Herold M
成人先天性心脏病（ACHD）心室功能不全的病因尚不清楚。弥漫性纤维化可能是常见的最终途径，可使用磁共振成像（MRI）进行定量。对ACHD患者（N=50）进行心脏MRI研究，以量化全身心室容积和功能以及弥漫性纤维化。通过测量钆（0.15 mmol/kg）给药前后血池和心肌的T1值，然后调整红细胞压积，量化系统心室单个心室中平面的纤维化指数。将结果与健康志愿者（正常对照，N=14）和获得性心力衰竭患者（阳性对照，N=4）进行比较。研究的患者（年龄37±12岁，40%女性）包括11例系统性右心室（RV），17例法洛四联症，10例紫绀和12例其他病变。与正常对照组相比，ACHD患者的纤维化指数显著升高（31.9±4.9% vs. 24.8± 2.0%，p=0.001）。全身性RV患者（35.0± 5.8%，p<0.001）和紫绀患者（33.7± 5.6%，p<0.001）的数值最高。纤维化指数与舒张末期容积指数（r=0.60，p<0.001）和心室射血分数（r=-0.53，p<0.001）相关，但与紫绀患者的年龄和血氧饱和度无关。晚期钆增强不能解释所观察到的差异。ACHD患者有弥漫性细胞外基质重塑的证据，与获得性心力衰竭患者相似。心肌纤维化指数的测定有助于研究心肌纤维化和心力衰竭的发生机制和治疗方法。
The etiology of ventricular dysfunction in adult congenital heart disease (ACHD) is not well understood. Diffuse fibrosis is a likely common final pathway and is quantifiable using magnetic resonance imaging (MRI). ACHD patients (N=50) were studied with cardiac MRI to quantify systemic ventricular volume and function, and diffuse fibrosis. The fibrosis index for a single mid-ventricular plane of the systemic ventricle was quantified by measuring T1 values for blood pool and myocardium before and after administration of gadolinium (0.15 mmol/kg), then adjusted for hematocrit. Results were compared to healthy volunteers (normal controls, N=14) and patients with acquired heart failure (positive controls, N=4). Patients studied (age 37±12 years, 40% female) included 11 with a systemic right ventricle (RV), 17 with tetralogy of Fallot, 10 with cyanosis and 12 with other lesions. The fibrosis index was significantly elevated in ACHD patients compared to normal controls (31.9±4.9% vs. 24.8±2.0%, p=0.001). Values were highest in systemic RV patients (35.0±5.8%, p<0.001) and cyanotic patients (33.7±5.6%, p<0.001). The fibrosis index correlated with end-diastolic volume index (r=0.60, p<0.001) and ventricular ejection fraction (r=−0.53, p<0.001), but not with age, nor oxygen saturation in cyanotic patients. Late gadolinium enhancement did not account for the differences seen. ACHD patients have evidence of diffuse, extracellular matrix remodeling, similar to patients with acquired heart failure. The fibrosis index may facilitate studies on the mechanisms and treatment of myocardial fibrosis and heart failure in these patients.