Incidence of cardiac malformations at birth and later, and neonatal mortality.
Incidence of cardiac malformations at birth and later, and neonatal mortality.
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出生时及以后心脏畸形的发生率以及新生儿死亡率。
DOI：
10.1136/hrt.35.2.189
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发表时间：
1973
影响因子：
--
通讯作者：
M. Campbell
中科院分区：
文献类型：
--
作者：
M. Campbell
在每000名新生儿中，约有8名发现心脏畸形。此外，每000名新生儿中约有4名患有二尖瓣主动脉瓣，但在生命早期很少被识别。除此之外，室间隔缺陷是最常见的畸形，约占所有其他畸形的30%。可能小型室间隔缺陷和二尖瓣是常见的，因为它们在生殖期几乎没有缺陷，所以很难通过自然选择来检查。房间隔缺损和永存动脉导管紧随其后，各约占IO%。肺动脉狭窄和主动脉缩窄各占约7%。主动脉瓣狭窄和法洛四联症约占6%，大干转位约占4%。这8种畸形占总数的80%，但列出了许多不太常见的畸形。新生儿死亡率如此之高，以至于所有心脏畸形的发生率很快就下降到出生时的一半左右。死亡率有很大的不同。转位和主干的发生率约为GO%，这使得这些在年龄较大的儿童中的比例变得微不足道。主动脉缩窄的发生率在50%到60%之间，因为大多数患有P缩窄的患者过早死亡，法洛四联症和多发性畸形患者也是如此。在其他常见畸形中，室间隔和房间隔缺损的发病率可能高于主动脉瓣狭窄(约29%)，而肺动脉狭窄(24%)和持续性动脉导管未闭(23%)的发病率较低。出生时室间隔缺陷的显著程度因大量的自发闭合而降低，这也适用于持续性动脉导管，但程度较小。描述了在学龄儿童、大龄儿童和年轻人中观察到的发病率。这与根据出生发病率和新生儿死亡率以及前二十年的差别死亡率计算的数字相当吻合。出生时，二尖瓣、主动脉狭窄、缩窄和大多数青紫型疾病的男性发病率较高，并在较年长的年龄继续或增加。只有永存动脉导管在出生时女性发病率较高，并随年龄增加而增加，但成人房间隔缺损和室间隔缺损的发病率呈上升趋势。只有肺动脉狭窄在两组之间没有差异
Malformations of the heart are found in about 8 of every 0OOO births. In addition, about 4 of every 0OOO births have bicuspid aortic valves, but they are rarely recognized early in life. Apart from these, ventricular septal defect is much the commonest malformation, forming about 30 per cent of all others. Probably small ventricular septal defects and bicuspid aortic valves are common because they have few drawbacks during the reproductive period so are hardly checked by natural selection. Atrial septal defect and persistent ductus arteriosus come next, each forming about IO per cent. Pulmonary stenosis and aortic coarctation each form about 7 per cent. Aortic stenosis and Fallot's tetralogy form about 6 per cent and transposition of the great trunks 4 per cent. These 8 malformations are responsible for 8o per cent of the total, but many less common ones have been listed. The neonatal mortality is so high that the incidence of all cardiac malformations is soon reduced to about half its incidence at birth. The mortality rates are strikingly different. They are about go per cent for transposition and truncus, reducing these to insignificant proportions in older children. They are between 50 and 6o per cent for aortic coarctation, because most with preductal coarctation die early, for Fallot's tetralogy, and for those with multiple malformations. For other common malformations they are probably higher for ventricular and atrial septal defects (both about 36%), than for aortic stenosis (about 29%) and lower for pulmonary stenosis (24%) and persistent ductus arteriosus (23%). The prominence of ventricular septal defect at birth is reduced by the large number that close spontaneously, and this applies also to persistent ductus arteriosus but to a lesser extent. The observed incidence in schoolchildren and in older children and young adults is described. This agrees reasonably well with the figures calculatedfrom the incidence at birth and the differential mortality, neonatal and during the first two decades. At birth, bicuspid aortic valves, aortic stenosis, coarctation, and most cyanotic conditions have a higher male incidence which continues or increases at older ages. Only persistent ductus arteriosus has a higher female incidence at birth and it too increases with age, but adults with atrial septal defect and ventricular septal defect show an increasing female incidence. Only pulmonary stenosis shows no difference between the