Clinical implications of dextrocardia based on four visceroatrial situs studies

IF 2.3 4区 医学 Q2 PEDIATRICS Pediatrics and Neonatology Pub Date : 2024-11-01 DOI:10.1016/j.pedneo.2023.10.011
Mao-Sheng Hwang , Ching-Chia Kuo , Chao-Jan Wang , Wen-Jen Su , Jaw-Ji Chu , Hung-Tao Chung , Hsiang-Ju Hsiao , Yi-Jung Chang
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Abstract

Background

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.

Methods

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.

Results

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.

Conclusion

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.
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基于四项内脏心房位置研究的右心室脱垂临床意义
背景以往关于先天性心脏病(CHD)伴右心房缺血的研究都是基于选择性的患者数据库,并不能反映一般人群中右心房缺血的全部情况。此外,这些研究的分类和表现形式也很复杂。这些研究也没有详细阐述相关 CHD 的复杂性、各种节段连接以及相关 CHD 在四个内脏心房位点之间的分布情况。方法我们回顾性地查看了 211 名原发性右心室缺血患儿的病历。我们采用节段法诊断 CHD。方法我们回顾性地查阅了 211 名原发性右心室缺血患儿的病历,并采用分段法对其进行了诊断,然后分析和比较了上述问题在四种内脏心房位置中的分布情况。虽然有些患者心脏结构正常(22.7%)或伴有简单的先天性心脏病(17.5%),但大多数患者都伴有复杂的先天性心脏病(59.7%),包括单心室(34.6%)或脐带异常(25.1%)(双出口右心室[7.6%]、矫正性大动脉转位[6.2%]、完全性大动脉转位[5.7%]、法洛氏四联症[4.7%]等)。在心脏结构正常或伴有单纯先天性心脏病、两个房室瓣连接通畅和双心室房室瓣连接的情况下,坐骨神经反流或多脾畸形的发病率较高。我们的研究发现,倒置位是右心室突出症中最常见的内脏心房位。尽管有些患者心脏结构正常或伴有简单的先天性心脏病,但大多数患者伴有复杂的先天性心脏病,包括单心室或圆锥心室异常。与坐位倒置和多脾畸形组相比,右心室与坐位和脾畸形组的复杂先天性心脏病发病率更高。
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来源期刊
CiteScore
3.10
自引率
0.00%
发文量
170
审稿时长
48 days
期刊介绍: Pediatrics and Neonatology is the official peer-reviewed publication of the Taiwan Pediatric Association and The Society of Neonatology ROC, and is indexed in EMBASE and SCOPUS. Articles on clinical and laboratory research in pediatrics and related fields are eligible for consideration.
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