Papa M, Camesasca C, Santoro F, Zoia E, Fragasso G, Giannico S, Chierchia S L
Division of Cardiology, Istituto Scientifico San Raffaele, University of Milan.
Br Heart J. 1995 Apr;73(4):355-8. doi: 10.1136/hrt.73.4.355.
Prenatal detection of congenital heart disease is possible from the 16th week of pregnancy, the ideal time being the mid-trimester, when most cardiac abnormalities can be detected. However, identification of anomalous pulmonary venous connection is difficult before birth and the sensitivity of fetal echocardiography in detecting this anomaly is low. Four cases are reported in which fetal echocardiographic findings obtained during the third trimester of pregnancy were highly suggestive of anomalous pulmonary venous connection. Right ventricular and atrial dominance associated with an enlarged coronary sinus or dilated superior vena cava were identified and considered to be indirect markers of the anomaly. No other cardiac anomaly was detectable. In all cases right ventricular and atrial dominance with dilated coronary sinus or superior vena cava were confirmed after birth despite the presence of normal pulmonary venous connections. These results confirm that the prenatal detection of this condition is difficult and should be based on the direct visualisation of anomalous pulmonary venous connections. The sole detection of indirect signs, such as right atrial and ventricular dominance with or without a dilated coronary sinus, superior vena cava, or inferior vena cava, does not warrant the diagnosis of anomalous pulmonary venous connection. Since the pulmonary venous flow in the human fetus is not as small as is commonly assumed, an anomalous drainage should be detectable when present and therefore should be specifically sought if the anomaly is suspected. The reasons for the presence of such transient cardiac anomalies remain obscure, but they might be related to functional or morphological rearrangement of the heart during fetal and perinatal life.
先天性心脏病在妊娠第16周起即可进行产前检测，理想时间是孕中期，此时多数心脏异常能够被检测出来。然而，产前识别肺静脉连接异常很困难，胎儿超声心动图检测这种异常的敏感性较低。本文报告4例妊娠晚期胎儿超声心动图检查结果高度提示肺静脉连接异常的病例。发现右心室和右心房优势伴冠状窦扩大或上腔静脉扩张，并将其视为该异常的间接标志。未检测到其他心脏异常。尽管出生后肺静脉连接正常，但所有病例出生后均证实存在右心室和右心房优势伴冠状窦或上腔静脉扩张。这些结果证实，产前检测这种疾病很困难，应基于对异常肺静脉连接的直接可视化。仅检测到间接征象，如伴有或不伴有冠状窦、上腔静脉或下腔静脉扩张的右心房和右心室优势，并不足以诊断肺静脉连接异常。由于人类胎儿的肺静脉血流并不像通常认为的那么少，存在异常引流时应可检测到，因此如果怀疑有该异常，应专门进行检查。出现这种短暂性心脏异常的原因尚不清楚，但可能与胎儿期和围生期心脏的功能或形态重排有关。
