Daliento L, Angelini A, Ho S Y, Frescura C, Turrini P, Baratella M C, Thiene G, Anderson R H
Department of Cardiology, University of Padua Medical School, Italy.
Am J Cardiol. 1997 Oct 15;80(8):1051-9. doi: 10.1016/s0002-9149(97)00602-4.
Quantitative and qualitative cineangiographic analysis of the left ventricle (LV) was performed in 26 patients with isolated Ebstein's malformation, having a mean age of 23 +/- 17 years. Nine autopsied hearts with isolated Ebstein's malformation were submitted to morphologic and morphometric analysis. In 4 of the cases, it was possible to make a direct correlation between the angiographic data obtained during life and the autopsy findings. On the basis of the LV end-diastolic volume we identified 3 groups of patients: 7 with volume <60 ml/m2, another 7 with volume between 60 and 80 ml/m2, and 12 with volume >80 ml/m2. The LV ejection fraction was reduced in 2 patients with normal LV end-diastolic volume and in 6 with increased LV end-diastolic volume. The ratio of ventricular mass to LV end-diastolic volume was always adequate, but a reduction of the ventricular contractive performance (end-systolic pressure to end-systolic volume ratio <3 mm Hg/ml/m2) was found only in patients with a dilated left ventricle. No correlation was demonstrated between the extent of the atrialized component of the right ventricle (mean value 67 +/- 31 cm2, range 13 to 133) and the LV dimensions. All but 2 patients showed a leftward diastolic displacement of the ventricular septum, but in only 1 did this produce an elongated shape of the left ventricle. Sixteen had anomalies of LV dynamics: 10 with hypokinesia (3 of the posterior wall, 4 of the apex, 1 of the inferior wall, 1 of the septum, and 1 global), 6 with dyskinesia (1 of the posterior wall, 2 of the apex, 1 of the posterior wall and apex, 1 of the superior part of the septum, and 1 of the anterior wall), and 8 with premature diastolic distension of the anterobasal wall. Morphometric analysis produced mean values for myocytes of 59 +/- 10%, for the interstitium of 21 +/- 4%, and for fibrous tissue of 20 +/- 9% (normal 4 +/- 1%). Five autopsied hearts had a prolapsing and/or dysplastic mitral valve.
对26例孤立性埃布斯坦畸形患者进行了左心室（LV）的定量和定性电影血管造影分析，这些患者的平均年龄为23±17岁。对9例孤立性埃布斯坦畸形的尸检心脏进行了形态学和形态计量学分析。在其中4例病例中，有可能将生前获得的血管造影数据与尸检结果直接关联起来。根据左心室舒张末期容积，我们将患者分为3组：7例容积<60 ml/m²，另外7例容积在60至80 ml/m²之间，12例容积>80 ml/m²。2例左心室舒张末期容积正常的患者以及6例左心室舒张末期容积增加的患者左心室射血分数降低。心室质量与左心室舒张末期容积的比值始终正常，但仅在左心室扩张的患者中发现心室收缩功能降低（收缩末期压力与收缩末期容积比值<3 mmHg/ml/m²）。右心室心房化部分的范围（平均值67±31 cm², 范围13至133）与左心室大小之间未显示出相关性。除2例患者外，所有患者均表现为室间隔舒张期向左移位，但只有1例导致左心室呈细长形。16例患者存在左心室动力学异常：10例运动减弱（后壁3例、心尖4例、下壁1例、室间隔1例、整体1例），6例运动障碍（后壁1例、心尖2例、后壁和心尖各1例、室间隔上部1例、前壁1例），8例前基底壁舒张期过早扩张。形态计量学分析得出心肌细胞的平均值为59±10%，间质为21±4%，纤维组织为20±9%（正常为4±1%）。5例尸检心脏存在二尖瓣脱垂和/或发育异常。
