Shivalkar B, Borgers M, Daenen W, Gewillig M, Flameng W
Department of Cardiac Surgery, Katholieke Universiteit Leuven, Belgium.
J Am Coll Cardiol. 1994 Mar 1;23(3):772-8. doi: 10.1016/0735-1097(94)90767-6.
The purpose of this study was to evaluate functional variables and morphologic correlates of chronically hypoperfused myocardium before and after revascularization.
Neonates with congenital anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA syndrome) develop some myocardial necrosis shortly after birth. The survivors of this event are left with a localized infarction and an almost entirely collateral circulation-dependent perfusion of the left ventricle that results in poor global left ventricular function. Survival beyond infancy is uncommon because of severe left heart failure. Revascularization, however, brings about functional recovery with good clinical outcome. The ALCAPA syndrome is thus characterized by chronic collateral circulation-dependent low perfusion, low contraction matching and potential revivability.
Five patients with ALCAPA syndrome are presented, with preoperative and postoperative clinical findings and histologic data obtained from intraoperative transmural biopsy specimens.
The angiographically assessed preoperative ejection fraction was 33 +/- 19% (mean +/- SD). Postoperative echocardiographic follow-up revealed normal left ventricular function in all patients. Histologic study of the biopsy specimens taken from the region perfused by the anomalous artery showed a variable degree of fibrosis (51 +/- 32%). The ultrastructure of the remaining myocytes revealed viable characteristics, but a substantial percent (46 +/- 26%) showed a markedly reduced fraction of contractile material.
These ultrastructural studies suggest delayed subcellular adaptive responses in the chronically hypoperfused myocardium of patients with ALCAPA syndrome.
本研究旨在评估血运重建前后慢性灌注不足心肌的功能变量及形态学关联。
患有左冠状动脉起源于肺动脉的先天性异常（ALCAPA综合征）的新生儿在出生后不久会发生一些心肌坏死。该事件的幸存者会留下局部梗死灶，左心室几乎完全依赖侧支循环进行灌注，导致左心室整体功能较差。由于严重的左心衰竭，婴儿期后存活并不常见。然而，血运重建可带来功能恢复并取得良好的临床结果。因此，ALCAPA综合征的特征为慢性侧支循环依赖的低灌注、低收缩匹配以及潜在的可恢复性。
报告了5例ALCAPA综合征患者，提供了术前和术后的临床发现以及从术中透壁活检标本获得的组织学数据。
血管造影评估的术前射血分数为33±19%（平均值±标准差）。术后超声心动图随访显示所有患者左心室功能正常。对取自异常动脉灌注区域的活检标本进行组织学研究，发现纤维化程度不一（51±32%）。剩余心肌细胞的超微结构显示具有存活特征，但相当比例（46±26%）的心肌细胞收缩物质比例明显降低。
这些超微结构研究表明，ALCAPA综合征患者慢性灌注不足心肌存在延迟的亚细胞适应性反应。
