Abstract
BackgroundDextro-transposition of the great arteries (d-TGA) is the most frequent cyanotic congenital heart pathology in neonates. Surgical correction of this condition is possible using the arterial switch operation (ASO) which was first performed by Jatene in 1975.ObjectivesThe aim of this study was to summarise the evidence on short- (less than 1 year), medium- (1–20 years), and long-term (more than 20 years) outcomes of children with d-TGA treated with the ASO. The primary outcome was survival. Secondary outcomes were freedom from cardiac reoperations, occurrence of aortic insufficiency, pulmonary stenosis, coronary artery anomalies, neuropsychological development problems and quality of life.MethodsWe searched MEDLINE, EMBASE, CINAHL, LILACS, and reference lists of included articles for studies reporting outcomes after ASO for d-TGA. Screening, data extraction and risk of bias assessment were done independently by two reviewers. We pooled data using a random-effects meta-analysis of proportions and, where not possible, outcomes were synthesized narratively. We used the Grading of Recommendations Assessment, Development and Evaluation (GRADE) system to assess the certainty of the evidence for each outcome.Main resultsFollowing ASO for TGA, short-term survival was 92.0% (95% CI 91.0–93.0%; I2 = 85.8%, 151 studies, 30,186 participants; moderate certainty evidence). Medium-term survival was 90.0% (95% CI 89.0–91.0%; I2 = 84.3%, 133 studies; 23,686 participants, moderate certainty evidence), while long-term survival was 87.0% (95% CI 80.0–92.0 %; I2 = 84.5%, 4 studies, 933 participants, very low certainty evidence). Evaluation of the different secondary outcomes also showed satisfactory results in the short, medium and long term. Subgroup analysis suggests slightly higher survival following ASO for TGA in the second surgical era (1998 to 2018) than in the first surgical era (1975 to 1997) in the short and medium term [93.0% (95% CI 92.0–94.0) vs 90.0% (95% CI 89.0–92.0) and 93.0% (95% CI 91.0–94.0) vs 88.0% (87.0–90.0%) respectively] but not in the long term [81.0% (95% CI 76.0–86.0%) vs 89.0% (80.0–95.0%)].ConclusionsPooled data from many sources suggests that the ASO for d-TGA leads to high rates of survival in the short, medium, and long term.
Highlights
Dextro-transposition of the great arteries (D-TGA) is the most frequent cyanotic congenital heart pathology in neonates
Pooled data from many sources suggests that the arterial switch operation (ASO) for D-TGA leads to high rates of survival in the short, medium, and long term
Description of the condition Dextro-transposition of the great arteries (D-TGA) known as congenital complete transposition of the great arteries, or transposition of the great arteries (TGA) refers to a clinical condition in which the two main arteries leaving the heart are transposed, with the aorta arising from the right ventricle, and the pulmonary artery originating from the left ventricle [1]
Summary
Dextro-transposition of the great arteries (D-TGA) is the most frequent cyanotic congenital heart pathology in neonates. The reported prevalence varies from 5.0 in 10,000 deliveries in the United States [3] to 1.6 per 10,000 children presenting to the hospital in Nigeria [4] It is distinct from the levo-transposition of the great arteries (L-TGA) or congenitally corrected TGA which combines both an atrioventricular discordance and a ventriculoarterial discordance, with circulation continuing in the appropriate direction but via the “wrong” ventricles [5]. Unlike L-TGA whose patients may present for the first time in adulthood [6], D-TGA is the most frequent cyanotic congenital heart pathology in neonates and may be rapidly fatal at birth [7] This is because, in the presence of D-TGA, the systemic and pulmonary circulations function in parallel rather than in series [8]. Without some connection between the two systems, blood flow is ineffective and the condition will be fatal shortly after birth [8]
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