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PLSVC and accompanying cardiac anomalies

To date, many CA associated with PLSVC have been identified and grouped in different ways [1, 14, 15]. Shunt lesions (Figs. (Figs.44 and and5),5), conotruncal malformations (CTMs) (Figs. (Figs.66 and and7),7), left-sided obstructive lesions (LOLs) (Fig. (Fig.8),8), right-sided lesions, and single ventricular anomalies (Fig. (Fig.9)9) constitute the main CA groups. Aortic arch anomalies are also associated with PLSVC (Figs. (Figs.1010 and and11).11). The subgroups of these anomalies are listed in Table Table3.3. Besides, a summary of the literature about PLSVC and accompanying CAs is compiled in Table Table4.4. Additionally, heterotaxy forms another disease spectrum associated with PLSVC and will be discussed under a separate title.

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In the literature, there is a wide range of information about the frequency of cardiac anomalies accompanying PLSVC [1, 6, 14-22]. According to Lendzidan et al., the most common cardiac anomalies accompanying PLSVC are single ventricle, atrioventricular septal defect (AVSD), and tetralogy of Fallot (TOF). Cha et al. reported that the most frequent concomitant anomaly is ASD, whereas, according to Eldin et al., complete atrioventricular septal defect comes the first [17-19].

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Moreover, attention has been drawn to the relationship of some specific cardiac anomalies with PLSVC in many publications. In addition to left-sided pathologies such as mitral atresia, cor triatriatum, and hypoplastic left heart, transposition of the great arteries (TGA) and tricuspid atresia are other rarer anomalies that have been reported to be closely related to PLSVC in the literature [6, 16, 20].

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Different cardiac anomalies come to the fore in different situations such as type of accompanying cardiac anomaly (cyanotic or acyanotic), presence of heterotaxy, and drainage location of PLSVC [14, 15, 21]. Different parameters, such as odds ratio and PLSVC index, are calculated in the literature and used to determine the relationship between PLSVC and cardiac anomaly [1, 8]. In some publications, cardiac anomalies accompanying PLSVC were grouped and evaluated as in Table Table3,3, and in others, they were examined separately [1, 17].

Association of PLSVC with aorta-related pathologies such as right-sided arcus aorta (RAA) and coarctation of the aorta (CoA) have also been emphasized in the literature. It was mentioned that the association of PLSVC with RAA is approximately 16% [14]. In another study, CoA was reported to be an independent and powerful factor for the existence of PLSVC [8]. Gustapane et al. underlined the coexistence of PLSVC with coarctation of the aorta (CoA) (21.3%) and suggested that fetuses with PLSVC that are detected in the antenatal period should be followed during pregnancy in terms of CoA development [22].

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