Hwang Mao-Sheng, Kuo Ching-Chia, Wang Chao-Jan, Su Wen-Jen, Chu Jaw-Ji, Chung Hung-Tao, Hsiao Hsiang-Ju, Chang Yi-Jung
Department of Pediatrics, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan.
Department of Pediatrics, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan.
Pediatr Neonatol. 2024 Nov;65(6):566-570. doi: 10.1016/j.pedneo.2023.10.011. Epub 2024 Apr 23.
Previous studies on congenital heart diseases (CHD) associated with dextrocardia were based on selective patient databases and did not reflect the full spectrum of dextrocardia in the general population. Additionally, these studies had complex classification and presentation. Nor did these studies elaborate on the distribution of the associated CHD's complexity, the various segmental connections, and associated CHD among the four visceroatrial situs.
We retrospectively reviewed the medical records of 211 children with primary dextrocardia. We used a segmental approach to diagnose CHD. We then analyzed and compared the distribution of the above-mentioned issues among the four visceroatrial situs.
Dextrocardia occurred most commonly with situs inversus (52.6%), followed by situs solitus (28.4%), asplenia (17.1%), and polysplenia (1.9%). Although some patients had a structurally normal heart (22.7%) or they were associated with simple CHD (17.5%), most patients had complex CHD (59.7%) consisting of a single ventricle (34.6%) or conotruncal anomaly (25.1%) (double-outlet right ventricle [7.6%], corrected transposition of the great arteries [6.2%], complete transposition of the great arteries [5.7%], tetralogy of Fallot [4.7%], etc.). Situs inversus or polysplenia had a higher prevalence of a structurally normal heart or associated with simple CHD, two patent atrioventricular (AV) valves connections, and biventricular AV connections. Situs solitus or asplenia had a higher prevalence of associated complex CHD, common AV valve connection, univentricular AV connection, pulmonary outflow tract obstruction, and anomalous pulmonary venous drainage.
Our study finds that situs inversus is the most common visceroatrial situs in dextrocardia. Although some patients had a structurally normal heart or were associated with simple CHD, most patients have associated complex CHD consisting of a single ventricle or conotruncal anomaly. Dextrocardia is associated with a higher incidence of complex CHD in situs solitus and asplenia groups than in situs inversus and polysplenia groups.
既往关于与右位心相关的先天性心脏病（CHD）的研究基于选择性患者数据库，未反映普通人群中右位心的全貌。此外，这些研究的分类和表现复杂。这些研究也未详细阐述相关CHD的复杂程度分布、各种节段连接以及四种内脏心房位中的相关CHD情况。
我们回顾性分析了211例原发性右位心患儿的病历。我们采用节段性方法诊断CHD。然后我们分析并比较了上述问题在四种内脏心房位中的分布情况。
右位心最常与内脏反位（52.6%）相关，其次是内脏正位（28.4%）、无脾综合征（17.1%）和多脾综合征（1.9%）。虽然一些患者心脏结构正常（22.7%）或与简单CHD相关（17.5%），但大多数患者患有复杂CHD（59.7%），包括单心室（34.6%）或圆锥动脉干畸形（25.1%）（右心室双出口[7.6%]、矫正型大动脉转位[6.2%]、完全性大动脉转位[5.7%]、法洛四联症[4.7%]等）。内脏反位或多脾综合征中，心脏结构正常或与简单CHD相关、两个房室（AV）瓣连接以及双心室AV连接的发生率较高。内脏正位或无脾综合征中，相关复杂CHD、共同AV瓣连接、单心室AV连接、肺流出道梗阻及肺静脉异位引流的发生率较高。
我们的研究发现，内脏反位是右位心中最常见的内脏心房位。虽然一些患者心脏结构正常或与简单CHD相关，但大多数患者患有由单心室或圆锥动脉干畸形组成的相关复杂CHD。与内脏反位和多脾综合征组相比，内脏正位和无脾综合征组中右位心与复杂CHD的发生率更高。
