Campbell M
Br Heart J. 1970 Sep;32(5):633-40. doi: 10.1136/hrt.32.5.633.
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.
随着接受主动脉缩窄手术的患者数量不断增加，其自然病史变得愈发难以确定。预期寿命通过两种完全独立的方法计算得出：（1）依据304例尸检报告；（2）依据161例患者在716个患者年中的22例死亡情况。这些大多是患者而非未经挑选的学童，但许多人毫无症状，仅因体检时听到杂音而前来就诊。知晓他们接受观察的时长后，便可计算出每十年的死亡率。死亡率逐渐上升，但并非十分规律，从前两个十年的1.6%升至第六个十年及之后的每年6.7%。这两种方法得出的结果高度一致，考虑到患者数量和患者年数相对较少，这种一致性比预期的还要高。两种方法在每个十年末得出的死亡百分比差异从未超过±4%，且往往更为接近。在度过最初一两年的严重危险期后存活下来的患者中，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值为34岁，而非通常的71岁。然而，这些平均值掩盖了很大的范围，标准差至少为±15%。对于主动脉缩窄，中位数为31岁，众数广泛分布于第二个至第五个十年，而非紧密聚集在某一点。这种不佳的预后使得5%左右的手术死亡率相较于术后大幅提高的安全性而言代价较小。我们尚不清楚从长远来看，细菌性心内膜炎或颅内出血的风险会降低多少，因为可能存在二叶式主动脉瓣或颅内浆果样动脉瘤。亚急性细菌性心内膜炎的确切发病率尚不确定，尸检系列为每年0.6%（来自尸检系列）至临床系列的1.3%之间。动脉导管未闭的范围类似，但矛盾的是，尸检系列为1.5%，临床系列为0.5%。显然，表5所示的所有四种畸形的发病率都很高，约为每年0.9±0.4%。
