胎儿心脏畸形的超声诊断及临床分型

目的：总结不同类型胎儿心脏畸形超声心动图特征、临床分型及预后特点。方法对2010年8月至2013年8月在暨南大学第二临床医学院深圳市人民医院行产前筛查并经产后随访证实为心脏畸形的429例胎儿超声心动图特征、临床及预后分型特点进行总结分析。结果产前超声心动图诊断心脏畸形胎儿429例，其中单一结构畸形11种共177例，以房间隔缺损（6例）和室间隔缺损（55例）检出例数居多（34.5%，61/177），其余单一结构畸形包括单心房10例，单心室34例，三尖瓣下移20例，三尖瓣闭锁11例，室间隔完整的肺动脉瓣闭锁6例，肺动脉狭窄20例，主动脉弓缩窄10例，主动脉弓离断4例，肺动脉悬吊1例。联合畸形14种共233例，以左右心室双出口（41例，17.5%，41/233）和法洛四联症（40例，17.2%，40/233）检出例数居多；其余联合畸形包括完全性大血管转位16例，矫正性大血管转位18例，房室间隔缺损38例，永存动脉干30例，完全性肺静脉异位引流8例，部分性肺静脉异位引流10例，右心室发育不良综合征14例，左心发育不良综合征6例，伴室间隔缺损的肺动脉闭锁9例，多脾综合征伴复杂心脏畸形2例，无脾综合征伴复杂心脏畸形1例。非结构性心脏畸形2种共19例，其中心脏占位畸形（16例，84.2%，16/19）较心内膜弹力纤维增生症胎儿（3例，15.8%，3/19）检查例数居多。手术病理及生后随访证实产前超声漏诊9例，误诊6例。临床分型：429例心脏畸形胎儿中无法根治、预后不良的复杂心脏畸形136例（31.7%，136/429），重症、可根治的心脏畸形93例（21.7%，93/429），生后需紧急处理的心脏畸形54例（12.6%，54/429），轻型心脏畸形146例（34.0%，146/429）。结论产前超声诊断胎儿心脏畸形可提示临床依据病情采取有效干预和治疗措施，降低心脏畸形胎儿出生率及围生儿死亡率。

Echocardiographic diagnosis and clinical classiifcation of fetal congenital heart anomalies

ObjectiveTo summarize the echocardiographic diagnosis, clinical classification and prognosis of different types of fetal congenital cardiac malformation （FCMH）.MethodsThe echocardiography sonogram characteristics for 429 cases with FCMH were summarized and analyzed by pathological findings and postnatal following-up.ResultsThe incidence of FCMH was 1.3% （429/33 800）. In one hundred and seventy-seven caese of single structure malformation, there were 5 cases of Atrial septal defect and 55 cases of interventricular septal defect （34.5%, 61/177）. The others including （116 cases）： 10 cases of single atrium and 34 cases of single ventricle, 20 cases of Ebstein’s anomaly, 11 cases of tricuspid atresia and 6 cases of pulmonary atresia with intact ventricular seprum, 20 cases of pulmonary stenosis, 10 cases of coarctation of aortic arch and 4 cases of interruption of aortic arch, 1 case of pulmonary artery sling. In two hundred and thirty-three cases of symphysic teratism, there were 41 cases of double-outllet right or left ventricle （17.5%, 41/233） and Tetralogy of Fallot （40 cases, 17.2%, 40/233）. The others （152 cases） including： 16 cases of complete and 18 cases of corrected transposition of great arteries, 38 cases of atrioventricular septal defect, 30 cases of truncus arteriosus persistens, 8 cases of total and 10 cases of partial abnormal pulmonary venous drainage, 14 cases of hypoplastic right heart and 6 cases of left heart syndrome, 9 cases of pulmonary artery atresia with ventricular septal defect, 2 cases of plysplenia and 1 case of plenia syndrome. Nineteen cases of instructure cardiac anomalies including 16 cases of heart tumor and 3 cases of endocardial ifbroelastosis. Nine cases were missed diagnosis and 7 cases were misdiagnosis. A total of 136 cases couldn′t be cured with an unfavorable outcome. Nighty three cases with severe FCMH could be cured. Fifty four cases needed emergent treatment postnatal, and 146 cases with mild abnormality.ConclusionsFetal congenital malfo