内脏异位综合征并心脏畸形的超声表现及不同表现的性别差异

Ultrasonic manifestations of heterotaxy syndrome with heart malformations and gender differences in different manifestations

目的探讨内脏异位综合征合并心脏畸形的超声表现及不同类型患者性别分布。方法回顾性分析292例经超声心动图诊断为内脏异位综合征合并心脏畸形患者的临床与影像资料,结合肝脾情况及合并心脏畸形情况进行分析;同时分析患者的性别差异。采用χ^(2)检验比较内脏异位综合征合并心脏畸形无脾、多脾及单侧脾患儿3种情况的性别差异。结果292例患者符合内脏异位综合征合并心脏畸形,男173例、女119例;无脾125例(42.8%)、多脾45例(15.4%)、单侧脾122例(41.8%);左位心占43.8%、右位心50.0%、中位心6.2%;严重畸形中单心室61.9%、单心房42.8%、圆锥动脉干畸形54.5%;房室间隔缺损16.4%。无脾、多脾及单侧脾3种类型的内脏异位综合征合并心脏畸形性别比较,差异有统计学意义(χ^(2)=8.061,P<0.05),其中无脾男性83例(66.4%)明显高于女性42例(33.6%),差异具有统计学意义(χ^(2)=13.448,P<0.05);异位心单侧脾中男性72例(59.0%)明显高于女性50例(41.0%),差异具有统计学意义(χ^(2)=3.967,P<0.05);多脾中女性26例(57.8%)与男性19例(42.2%)相比差异无统计学意义(χ^(2)=1.089,P>0.05)。结论超声检查可有效诊断内脏异位综合征合并心脏畸形,且无脾型、多脾型以及单侧脾型异构畸形性别差异显著,可为临床诊断提供借鉴。

Objective To explore the gender distribution and the ultrasonic manifestations of different types of patients with heterotaxy syndrome combined with heart malformations.Methods The clinical and imaging data of 292 patients diagnosed with heterotaxy syndrome and heart malformations by echocardiography were retrospectively analyzed,combined with the condition of the liver,spleen,and heart malformations.At the same time,the gender differences of the patients were analyzed.Theχ^(2) test was used to compare the gender differences in children with heterotaxy syndrome combined with heart malformations with asplenia,polysplenia,and unilateral spleen.Results There were 292 patients with heterotaxy syndrome combined with heart malformations,including 173 males and 119 females;there are 125 cases(42.8%)with asplenia,45 cases(15.4%)with polysplenia,and 122 cases(41.8%)with unilateral spleen and heterotaxy.Among them,43.8%of the patients were levocardia,50.0%were dextrocardia,and 6.2%were mesocardia.For severe cardiac malformations,61.9%were single ventricle,42.8%were single atrium,54.5%were with conotruncal anomalies,and 16.4%were with the atrioventricular septal defect.There were significant gender differences in asplenia,polysplenia,and unilateral spleen,three types of heterotaxy syndrome combined with cardiac malformations(χ^(2)=8.061,P<0.05),among them,83(66.4%)males with asplenia were significantly higher than 42(33.6%)females,the difference was statistically significant(χ^(2)=13.448;all P<0.05);72(59.0%)with unilateral were significant higher than 50(41.0%)females,the difference was statistically significant(χ^(2)=3.967,P<0.05);26(57.8%)females with polysplenia had no significant difference compared with 19(42.2%)males(χ^(2)=1.089,P>0.05).Conclusion Ultrasonography can effectively diagnose heterotaxy syndrome and heart malformations,and there are significant gender differences in asplenic,polysplenia,and unilateral splenic heterogeneous malformations,which can provide references for clinical diagnosis.