ANOMALOUS CORONARY-ARTERIES - A MULTICENTER PEDIATRIC AUTOPSY STUDY
ANOMALOUS CORONARY-ARTERIES - A MULTICENTER PEDIATRIC AUTOPSY STUDY
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DOI：
10.3109/15513819409024261
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发表时间：
1994-03-01
期刊：
影响因子：
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通讯作者：
BYARD, RW
中科院分区：
文献类型：
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作者：
LIPSETT, J;COHLE, SD;BYARD, RW
冠状动脉异常(ACAs)是心肌缺血的潜在原因，可导致心律失常、心绞痛、梗塞和猝死。我们分析了来自四个三级中心的7857例儿童尸检病例，以全面了解ACAS在这一儿童尸检人群中的范围和意义。ACAs的发生率为0.5%，其中来自主动脉的异位最常见(43%)，其次是起源于肺干的ACA(40%)。平均死亡年龄为2.2岁(4h~14岁)。ACAs合并其他畸形的比例为57%，其中43%为心脏缺陷。50%的病例出现生长迟缓。心脏检查显示92%的患者心脏增大，63%的患者心脏明显增大(>；95%)，但仅50%的患者组织学上可见明显的缺血性损害。与其他类型的畸形相比，起源于肺干的ACAs与更早的死亡(1.4年)、更常见的心脏肥大(100%)和更频繁的心肌瘢痕形成(92%)相关。尽管45%的病例最终表现为猝死，但合并其他心脏畸形的ACAs主要表现为与后者相关的问题。这一结果强调了在儿科尸检过程中仔细检查冠状动脉的必要性。重要的不仅是冠状动脉开口的位置，还有动脉起飞角度的变化，通过主动脉外膜的初始过程，随后的过程，以及可能导致血流显著损害的口脊或膜的存在。
Anomalous coronary arteries (ACAs) are a potential cause of myocardial ischemia resulting in arrhythmias, angina, infarction, and sudden death. We analyzed 7857 pediatric autopsy cases from four tertiary centers to gain an overall picture of the range and significance of ACAs within this pediatric autopsy population. The incidence of ACAs was 0.5%, with ectopic origin from the aorta (43%) being most frequent, followed by ACA arising from the pulmonary trunk (40%). The mean age at death was 2.2 years (4 h-14 years). ACAs were associated with other anomalies in 57% of cases, 43% of these being cardiac defects. Growth retardation was noted in 50% of the cases. Examination of the heart showed cardiomegaly in 92% of cases, which was marked (>95 percentile) in 63%, but histologically apparent ischemic damage was seen in only 50% of cases. ACAs arising from the pulmonary trunk were associated with earlier death (1.4 years), more frequent cardiomegaly (100%), and more frequent myocardial scarring (92%) than other types of anomalies. Although the terminal presentation was sudden death in 45% of cases, ACAs associated with other cardiac defects presented mainly with problems related to the latter anomalies. The results highlight the need to examine coronary arteries carefully during the pediatric autopsy. Not only are the positions of the coronary artery ostia important, but also the variations in the angles of arterial take-off, initial courses through the aortic adventitia, subsequent courses, and presence of ostial-ridges or membranes that may result in significant compromise of blood flow.