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Transposition of the great arteries (TGA) is one of the most common cyanotic heart defects with a mortality rate of 90% in the first year of life. The prognosis of children born with TGA changed dramatically after the introduction of surgical repair; in the late 1950s, the Senning technique1 was initiated, followed by the Mustard procedure in the early 1960s.2 Both procedures involve atrial switch leading to the restoration of normal cardiac physiology; however, the anatomical constellation remains abnormal, leaving the morphologically right ventricle to support systemic circulation. While the impact of this repair on early mortality is excellent, the presence of systemic right ventricle (SRV) carries significant long-term complications. Until the mid-1980s, atrial switch procedure was the treatment of choice for TGA. In the current era, it has been largely replaced by arterial switch operation, today’s standard treatment, which restores not only normal physiology but also cardiac anatomy.3
Late complications of atrial switch repair are common, including SRV dysfunction, heart failure, arrhythmias, baffle obstruction and/or leak, etc.4–6 Complications increase dramatically after the age of 40–50 years, leading to markedly reduced survival compared with the general population.4 5 The majority of patients with atrial switch repair are now in their third, fourth, or even sixth decade of life and face these …
Contributors Both authors have been equally involved in all aspects of this paper, from conception to the final product.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Provenance and peer review Commissioned; internally peer reviewed.