ALCAPA SYNDROME - AN EXAMPLE OF CHRONIC MYOCARDIAL HYPOPERFUSION
ALCAPA SYNDROME - AN EXAMPLE OF CHRONIC MYOCARDIAL HYPOPERFUSION
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DOI：
10.1016/0735-1097(94)90767-6
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发表时间：
1994-03-01
影响因子：
24
通讯作者：
FLAMENG, W
中科院分区：
文献类型：
--
作者：
SHIVALKAR, B;BORGERS, M;FLAMENG, W
目标。本研究的目的是评估慢性低灌注心肌在血运重建前后的功能变量和形态学相关因素。先天性左冠状动脉与肺动脉起源异常的新生儿（ALCAPA综合征）在出生后不久发生心肌坏死。该事件的幸存者留下了局部梗死和几乎完全侧支循环依赖的左心室灌注，导致整体左心室功能差。由于严重的左心衰竭，在婴儿期后存活是罕见的。然而，血运重建术带来了良好的临床效果的功能恢复。因此，ALCAPA综合征的特点是慢性侧枝循环依赖性低灌注、低收缩匹配和潜在的可复性。本文报告5例ALCAPA综合征患者，术前和术后临床表现及术中经壁活检标本的组织学资料。术前血管造影评估的射血分数为33 +/- 19%（平均+/- SD）。术后超声心动图随访显示所有患者左心室功能正常。异常动脉灌注区活检标本的组织学研究显示不同程度的纤维化（51 +/- 32%）。剩余肌细胞的超微结构显示有活力的特征，但相当大的比例（46 +/- 26%）显示收缩物质的比例明显减少。这些超微结构研究提示ALCAPA综合征患者慢性低灌注心肌的亚细胞适应性反应延迟。
Objectives. The purpose of this study was to evaluate functional variables and morphologic correlates of chronically hypoperfused myocardium before and after revascularization.Background. 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.Methods. Five patients with ALCAPA syndrome are presented, with preoperative and postoperative clinical findings and histologic data obtained from intraoperative transmural biopsy specimens.Results. 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.Conclusions. These ultrastructural studies suggest delayed subcellular adaptive responses in the chronically hypoperfused myocardium of patients with ALCAPA syndrome.