Department of Pediatric Cardiology, Istanbul Saglik Bilimleri University Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Education and Research Hospital, Istanbul, Turkey.
Department of Pediatric Cardiology, Istanbul Saglik Bilimleri University Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Education and Research Hospital, Istanbul, Turkey; Department of Pediatric Cardiology & Pediatric Cardiac Intensive Care, Istanbul Saglik Bilimleri University Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Education and Research Hospital, Istanbul, Turkey.
Rev Port Cardiol (Engl Ed). 2020 Jul;39(7):391-396. doi: 10.1016/j.repc.2019.09.017. Epub 2020 Jul 16.
Congenitally corrected transposition of the great arteries (ccTGA) is a rare congenital heart defect. There are different subgroups according to the location of the heart in the thorax, apical position and situs.
The purpose of this study was to assess pediatric patients with situs inversus (SI) ccTGA (SI-ccTGA), a rare subgroup of this condition, in detail.
The records of patients with SI-ccTGA followed between January 1, 2010 and January 1, 2019 in our clinic were analyzed retrospectively. Demographic features, associated cardiac defects, arrhythmias and follow-up data were recorded.
Twenty-one out of 120 ccTGA patients had SI. The median age was 30 months (4 days-18 years). There were hemodynamically significant associated lesions in 85.7% (n=18) of the patients. A large ventricular septal defect was found in 16 patients (76.2%), severe pulmonary stenosis in 11 (52.4%), pulmonary atresia in six (28.5%), and severe tricuspid regurgitation in two (9.5%). Eleven out of 21 patients had biventricular physiology and eight had single-ventricle physiology. Bidirectional cavopulmonary anastomosis followed by a hemi-Mustard-Rastelli operation were planned for the remaining two patients. Twelve out of 18 patients with associated defects (66.6%) were operated and surgery was planned for three more patients (16.6%). The remaining three patients were scheduled for clinical follow-up. Arrhythmias developed in two (9.5%) patients on follow-up; ablation was performed in one of them and pacemaker implantation followed by cardiac resynchronization therapy was performed in the other. Two patients died during follow-up, one after a central shunt operation and the other preoperatively due to pneumonia and sepsis.
SI-ccTGA is not a mirror image of situs solitus ccTGA (SS-ccTGA) due to important anatomic and physiologic differences between them. SI-ccTGA patients have a lower risk of tricuspid valve regurgitation than SS-ccTGA patients. The timing of clinical presentation of these patients mainly depends on the type and severity of the associated lesions, as in all subtypes of ccTGA.
先天性矫正型大动脉转位（ccTGA）是一种罕见的先天性心脏病。根据心脏在胸腔中的位置、心尖位置和 situs，有不同的亚组。
本研究旨在详细评估 situs inversus（SI）ccTGA（SI-ccTGA）这一罕见亚组的儿科患者。
回顾性分析 2010 年 1 月 1 日至 2019 年 1 月 1 日期间在我院就诊的 SI-ccTGA 患者的病历。记录患者的人口统计学特征、相关心脏缺陷、心律失常和随访数据。
在 120 例 ccTGA 患者中，有 21 例存在 SI。中位年龄为 30 个月（4 天-18 岁）。85.7%（n=18）的患者存在血流动力学意义重大的相关病变。16 例患者存在大室间隔缺损（76.2%），11 例存在严重肺动脉瓣狭窄（52.4%），6 例存在肺动脉闭锁（28.5%），2 例存在严重三尖瓣反流（9.5%）。11 例患者存在双心室生理学，8 例存在单心室生理学。其余 2 例患者计划行双向腔肺吻合术，随后行半Mustard-Rastelli 手术。18 例存在相关缺陷的患者中有 12 例（66.6%）接受了手术治疗，还有 3 例（16.6%）计划手术。其余 3 例患者接受临床随访。2 例（9.5%）患者在随访中出现心律失常；其中 1 例行消融治疗，另 1 例植入起搏器，随后行心脏再同步治疗。2 例患者在随访期间死亡，1 例死于中心分流术后，另 1 例术前因肺炎和败血症死亡。
由于 SI-ccTGA 与 situs solitus ccTGA（SS-ccTGA）之间存在重要的解剖和生理差异，因此它不是 SS-ccTGA 的镜像。与 SS-ccTGA 患者相比，SI-ccTGA 患者三尖瓣反流的风险较低。这些患者的临床表现时间主要取决于相关病变的类型和严重程度，与所有 ccTGA 亚型相同。
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