Natural history of coarctation of the aorta
Natural history of coarctation of the aorta
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DOI：
10.1136/hrt.32.5.633
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发表时间：
1970-09
影响因子：
--
通讯作者：
M. Campbell
中科院分区：
文献类型：
--
作者：
M. Campbell
随着越来越多的主动脉缩窄患者接受手术，其自然病程变得更加难以确定。通过两种完全独立的方法计算了预期寿命：（1）根据304例尸检报告，（2）根据161例受试者中的22例死亡，随访716患者-年。这些病人大多数是病人，而不是学龄儿童，但许多人完全没有症状，只是因为在检查时听到杂音而被送去。知道他们被观察了多久，就可以计算出每十年的死亡率。这一比例逐渐上升，但不是很有规律，从头二十年的1.6%上升到第六个十年及以后的每年6.7%。这两种方法得出的结果非常一致，考虑到患者数量和患者年相对较少，这比预期的结果更接近。用这两种方法得出的每十年末的死亡百分比相差从未超过± 4%，而且往往非常接近。在最初一两年的严重危险中幸存下来的人中，25%在20岁前死亡，50%在32岁前死亡，75%在46岁前死亡，90%在58岁前死亡。死亡年龄的算术平均数是34岁，而不是通常的71岁。然而，这些平均值隐藏了一个非常大的范围，标准差至少为± 15%，对于缩窄，中位数为31岁，模式广泛分布在第二至第五个十年，而不是紧密聚集在一个点周围。这种糟糕的前景使得5%左右的手术死亡率与之后大大增加的安全性相比是一个很小的代价。我们还不知道从长远来看，细菌性心内膜炎或颅内出血的风险会降低多少，因为可能有二叶主动脉瓣或颅内浆果动脉瘤。亚急性细菌性心内膜炎的确切发病率尚不确定，在临床研究中，发病率在每年0.6%（来自尸检系列）和1.3%之间。永存动脉导管的范围相似，但矛盾的是，尸检系列为1.5，临床系列为0.5。显然，表5所示的四种畸形的发生率都很高，约为每年0.9± 0.4%。
With the increasing number of subjects with coarctation of the aorta having operations, its natural history becomes more difficult to determine. The expectation of life has been calculated by two quite independent methods: (1) from 304 reported necropsies, and (2) from 22 deaths among 161 subjects followed for 716 patient-years. These were mostly patients rather than unselected school-children, but many were quite free from symptoms and were sent only because a murmur was heard on examination. Knowing how long they had been under observation, mortality rates could be calculated for each decade. They rise gradually but not very regularly, from 1.6 per cent in the first two decades to 6.7 per cent per annum in the sixth and later decades. The two methods give results in close agreement, closer than could be expected considering the relatively small numbers of patients and patient-years. The percentages of deaths at the end of each decade found by the two methods never differ by more than ±4 per cent and are often much closer. Of those surviving the serious hazards of the first one or two years, 25 per cent die before they reach 20, 50 per cent by 32, 75 per cent by 46, and 90 per cent by 58 years. The arithmetical mean of the ages of death is 34 years instead of 71 years as normally. These means, however, hide a very wide range, with standard deviations of at least ±15 per cent. For coarctation the median is 31 years and the mode is widely spread through the second to fifth decades rather than closely clustered round a point. This poor outlook makes an operative mortality in the region of 5 per cent a small price to pay for the greatly increased security afterwards. We do not yet know how much the risk of bacterial endocarditis or of intracranial haemorrhage will be reduced in the long run because there may be bicuspid aortic valves or intracranial berry aneurysms. The exact incidence of subacute bacterial endocarditis is uncertain, somewhere between 0.6 (from necropsy series) and 1.3 per cent per annum in clinical series. The range was similar for persistent ductus arteriosus, but paradoxically 1.5 for the necropsy series and 0.5 for the clinical series. Clearly the incidence is high in all the four malformations shown in Table 5, about 0.9±0.4 per cent per annum.