Adult Congenital Heart Disease Unit, Hospital das Clínicas, Facultdade de Medicina de Ribeirão Preto, Universidade de São Paulo, Ribeirão Preto, São Paulo, Brazil.
Cardiology, Boston Children's Hospital, Boston, Massachusetts, United States of America.
Braz J Cardiovasc Surg. 2022 Aug 16;37(4):534-545. doi: 10.21470/1678-9741-2021-0528.
Congenitally corrected transposition of the great arteries (CCTGA) is a rare anomaly. Current data available regarding adult cases is derived from small series, information simultaneously presented in pediatric publications, and one classical multicenter study. This review, not aimed to exhaust the subject, has the purpose to examine the literature addressing presentation, diagnostic methodology, and management of afflicted adult patients.
A comprehensive search was undertaken in three major databases (PubMed, Cochrane, SciELO), using the keywords "congenitally corrected transposition of the great arteries" and "adults". Relevant articles in English, Spanish, and Portuguese were extracted and critically appraised in this review. Steps for study selection were: (1) identification of titles of records through databases searching, (2) removal of duplicates, (3) screening and selection of abstracts, (4) final inclusion in the study.
Four hundred sixty-five publications on CCTGA in adult patients were retrieved, and 166 were excluded; 299 studies were used for this review including 76 full-text articles, 70 studies related to general aspects of the subject, and, due to the small number of publications, 153 case reports. Sixty-one articles referring to combined experiences in pediatric and adult patients and judged to be relevant, but retrieved from another sources, were also included.
Albeit clinical presentation and diagnostic criteria have been well stablished, there seems to be room for discussion related to clinical and surgical management of CCTGA in adults. Considering the rarity of the disease, well designed multicenter studies may provide answers.
先天性矫正性大动脉转位（CCTGA）是一种罕见的异常。目前关于成人病例的数据来自于小系列研究、儿科出版物中的信息以及一项经典的多中心研究。本综述的目的不是详尽无遗地探讨这个问题，而是检查文献中关于受影响的成年患者的临床表现、诊断方法和治疗。
我们在三个主要数据库（PubMed、Cochrane、SciELO）中使用“先天性矫正性大动脉转位”和“成人”这两个关键词进行了全面搜索。提取并批判性地评价了本综述中英语、西班牙语和葡萄牙语的相关文章。研究选择的步骤如下：（1）通过数据库搜索识别记录的标题，（2）删除重复项，（3）筛选和选择摘要，（4）最终纳入研究。
共检索到 465 篇关于成人 CCTGA 的文献，排除了 166 篇；有 299 项研究被用于本综述，包括 76 篇全文文章、70 项与该主题一般方面相关的研究，由于出版物数量较少，还有 153 篇病例报告。也纳入了 61 篇涉及儿科和成人患者综合经验的文章，这些文章被认为是相关的，但从其他来源检索到的。
尽管临床表现和诊断标准已经得到很好的确定，但在成人 CCTGA 的临床和手术管理方面似乎仍有讨论的余地。考虑到该疾病的罕见性，精心设计的多中心研究可能会提供答案。
