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室间隔完整的胎儿肺动脉瓣狭窄或闭锁的预后决定因素。

Determinants of outcome in fetal pulmonary valve stenosis or atresia with intact ventricular septum.

作者信息

Roman Kevin S, Fouron Jean-Claude, Nii Masaki, Smallhorn Jeffrey F, Chaturvedi Rajiv, Jaeggi Edgar T

机构信息

Division of Cardiology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.

出版信息

Am J Cardiol. 2007 Mar 1;99(5):699-703. doi: 10.1016/j.amjcard.2006.09.120. Epub 2007 Jan 8.

DOI:10.1016/j.amjcard.2006.09.120
PMID:17317375
Abstract

Pulmonary valve stenosis or atresia with intact ventricular septum represents a spectrum of severity. This study aimed to identify ultrasound markers of biventricular versus non-biventricular outcome. The fetal echocardiograms of 41 fetuses diagnosed with pulmonary stenosis or atresia and right ventricular (RV)/left ventricular (LV) length ratios >0.4 from 17 to 31 weeks of gestation were reviewed. Of 27 live-born patients with intention to treat, 8 had non-biventricular outcomes and 19 had biventricular circulation. At the time of diagnosis, poor RV function, flow reversal in the arterial duct, the degree of tricuspid valve (TV) regurgitation, and inferior vena cava Doppler flow pattern did not differ between the 2 outcome groups. However, RV sinusoids, the RV/LV length ratio, the TV/mitral valve ratio, and TV inflow duration were significantly different. Cut-off values derived from receiver-operating characteristic curves yielding the best sensitivity and specificity for a non-biventricular outcome were TV/mitral valve ratio <0.7, RV/LV length ratio <0.6, TV inflow duration <31.5% of cardiac cycle length, and the presence of RV sinusoids. If 3 of these 4 criteria were fulfilled, this predicted a non-biventricular outcome with sensitivity of 100% and specificity of 75%. In conclusion, in fetuses < or =31 weeks of gestation with pulmonary stenosis or atresia and intact ventricular septum, progression to a non-biventricular outcome can be predicted by a 4-criterion scoring system. The criteria may be useful in selecting fetuses for prenatal catheter intervention to prevent progressive RV hypoplasia.

摘要

室间隔完整的肺动脉瓣狭窄或闭锁代表了一系列严重程度。本研究旨在确定双心室与非双心室结局的超声标志物。回顾了41例妊娠17至31周被诊断为肺动脉狭窄或闭锁且右心室(RV)/左心室(LV)长度比>0.4的胎儿的超声心动图。在27例有治疗意向的活产患者中,8例有非双心室结局,19例有双心室循环。在诊断时,2个结局组之间的右心室功能差、动脉导管内血流逆转、三尖瓣(TV)反流程度和下腔静脉多普勒血流模式并无差异。然而,右心室窦状隙、RV/LV长度比、TV/二尖瓣比值和TV流入持续时间有显著差异。从受试者工作特征曲线得出的对非双心室结局具有最佳敏感性和特异性的临界值为TV/二尖瓣比值<0.7、RV/LV长度比<0.6、TV流入持续时间<心动周期长度的31.5%以及存在右心室窦状隙。如果满足这4条标准中的3条,则预测非双心室结局的敏感性为100%,特异性为75%。总之,在妊娠≤31周、患有肺动脉狭窄或闭锁且室间隔完整的胎儿中,可通过一个4条标准的评分系统预测进展为非双心室结局。这些标准可能有助于选择胎儿进行产前导管干预,以防止右心室进行性发育不全。

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