Medication in adults after atrial switch for transposition of the great arteries: Clinical practice and recommendations

Odilia I. Woudstra, Joey M. Kuijpers, Monique R.M. Jongbloed, Arie P.J. Van Dijk, Gertjan T. Sieswerda, Hubert W. Vliegen, Anastasia D. Egorova, Philippine Kiès, Anthonie L. Duijnhouwer, Daniëlle Robbers-Visser, Thelma C. Konings, Aeilko H. Zwinderman, Folkert J. Meijboom, Barbara J.M. Mulder, Berto J. Bouma*

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review


Aims: Heart failure is the main threat to long-term health in adults with transposition of the great arteries (TGA) corrected by an atrial switch operation (AtrSO). Current guidelines refrain from recommending heart failure medication in TGA-AtrSO, as there is insufficient data to support the hypothesis that it is beneficial. Medication is therefore prescribed based on personal judgements. We aimed to evaluate medication use in TGA-AtrSO patients and examine the association of use of renin-angiotensin-aldosterone system (RAAS) inhibitors and β-blockers with long-term survival. Methods and results: We identified 150 TGA-AtrSO patients [median age 30 years (interquartile range 25-35), 63% male] included in the CONCOR registry from five tertiary medical centres with subsequent linkage to the Dutch Dispensed Drug Register for the years 2006-2014. Use of RAAS inhibitors, β-blockers, and diuretics increased with age, from, respectively, 21% [95% confidence interval (CI) 14-40], 12% (95% CI 7-21), and 3% (95% CI 2-7) at age 25, to 49% (95% CI 38-60), 51% (95% CI 38-63), and 41% (95% CI 29-54) at age 45. Time-varying Cox marginal structural models that adjusted for confounding medication showed a lower mortality risk with use of RAAS inhibitors and β-blockers in symptomatic patients [hazard ratio (HR) = 0.13 (95% CI 0.03-0.73); P = 0.020 and HR = 0.12 (95% CI 0.02-0.17); P = 0.019, respectively]. However, in the overall cohort, no benefit of RAAS inhibitors and β-blockers was seen [HR = 0.93 (95% CI 0.24-3.63); P = 0.92 and HR = 0.98 (0.23-4.17); P = 0.98, respectively]. Conclusion: The use of heart failure medication is high in TGA-AtrSO patients, although evidence of its benefit is limited. This study showed lower risk of mortality with use of RAAS inhibitors and β-blockers in symptomatic patients only. These findings can direct future guidelines, supporting use of RAAS inhibitors and β-blockers in symptomatic, but not asymptomatic patients.

Original languageEnglish
Pages (from-to)77-84
Number of pages8
JournalEuropean Heart Journal - Cardiovascular Pharmacotherapy
Issue number1
Publication statusPublished - 1 Jan 2022

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