Hornberger L K, Sanders S P, Rein A J, Spevak P J, Parness I A, Colan S D
Department of Cardiology, Children's Hospital, Boston, MA 02115, USA.
Circulation. 1995 Sep 15;92(6):1531-8. doi: 10.1161/01.cir.92.6.1531.
Isolated case reports that suggest the potential for development of left heart hypoplasia late in gestation provide the only information about the in utero natural history of left heart obstructive lesions.
We reviewed the prenatal and postnatal echocardiograms of 21 fetuses with left heart obstructive lesions, including 15 with serial antenatal study, to elucidate the antenatal natural history of this spectrum of disease and to identify features indicative of postnatal disease severity. Ventricular, atrioventricular valve, and great artery dimensions were measured and growth curves were developed with comparisons to data from 47 normal fetuses. Fetuses were divided into groups according to whether postnatally the left heart was capable (group 1, n = 10) or incapable (group 2, n = 7) of supporting the systemic circulation in the presence of a patent aortic valve. Group 3 (n = 4) included fetuses with aortic atresia. At the initial examination (21.7 +/- 3.4 weeks' gestation), left heart dimensions were normal or reduced, with the most diminutive measurements in group 3. Three fetuses in group 2 and most in group 1 had normal initial left heart dimensions. Subsequent growth of left heart structures either paralleled normal growth or was reduced, the latter resulting in the development or progression of left heart hypoplasia. All left heart dimensions grew more slowly in group 2 and group 3 than in group 1 (P < .05). Other prenatal features observed only in groups 2 and 3 included reversed (n = 10) or bidirectional (n = 1) foramen ovale flow and retrograde distal arch flow (n = 9). Initial midtrimester mitral valve and ascending aorta z scores and the growth rates of all left heart structures correlated strongly with postnatal left ventricular end-diastolic dimension (P = .0007 to .03, r = .57 to .82) and could be additional indicators of postnatal disease severity. One group 1 fetus developed severe aortic stenosis late in gestation.
The potential for the in utero development or progression in severity of left heart obstruction and hypoplasia in left heart obstructive lesions necessitates serial prenatal study in affected fetuses carried to term.
仅有个别病例报告提示在妊娠晚期可能发生左心发育不全,这是关于左心梗阻性病变宫内自然病史的唯一信息来源。
我们回顾了21例患有左心梗阻性病变胎儿的产前和产后超声心动图,其中15例进行了系列产前研究,以阐明此类疾病的产前自然病史,并确定提示产后疾病严重程度的特征。测量了心室、房室瓣和大动脉的尺寸,并与47例正常胎儿的数据进行比较,绘制出生长曲线。根据产后左心在主动脉瓣开放时能否支持体循环,将胎儿分为两组:第1组(n = 10)能够支持,第2组(n = 7)不能支持。第3组(n = 4)包括患有主动脉闭锁的胎儿。在初次检查时(妊娠21.7±3.4周),左心尺寸正常或减小,第3组测量值最小。第2组中的3例胎儿和第1组中的大多数胎儿初次检查时左心尺寸正常。随后,左心结构的生长要么与正常生长平行,要么减缓,后者导致左心发育不全的发生或进展。第2组和第3组所有左心尺寸的生长均比第1组缓慢(P <.05)。仅在第2组和第3组中观察到的其他产前特征包括卵圆孔血流反向(n = 10)或双向(n = 1)以及主动脉弓远端血流逆行(n = 9)。孕中期二尖瓣和升主动脉的初始z值以及所有左心结构的生长速率与产后左心室舒张末期内径密切相关(P = 0.0007至0.03,r = 0.57至0.82),可能是产后疾病严重程度的额外指标。第1组中有1例胎儿在妊娠晚期发生了严重主动脉狭窄。
左心梗阻性病变在宫内发生或严重程度进展的可能性,使得对足月的受累胎儿进行系列产前研究成为必要。