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Evaluation and management of the adult patient with transposition of the great arteries following atrial-level (Senning or Mustard) repair

Nature Clinical Practice Cardiovascular Medicine volume 5, pages 454–467 (2008)Cite this article

Abstract

Atrial-level repair for transposition of the great arteries is one of the greatest achievements of modern cardiovascular medicine, transforming a once fatal congenital heart condition into one with excellent long-term survival. Although atrial-level repair has been supplanted by the arterial switch, there remains a population of patients who underwent atrial-level repair as children and now require ongoing care as adults. Survival slowly continues to decline in this population mainly owing to systemic right ventricular dysfunction and sudden death. Other problems include sinus node dysfunction, atrial arrhythmias, systemic atrioventricular valve regurgitation, baffle problems, and pulmonary hypertension. Evaluation and management of these late complications is addressed.

Key Points

  • Patients who have undergone atrial-level repair for transposition of the great arteries comprise an important group of adults with congenital heart disease

  • Although most adult patients with atrial-level repair continue to do well clinically, there remains a steady attrition, mainly owing to sudden arrhythmic death and systemic ventricular dysfunction

  • Late morbidity includes sinus node dysfunction, atrial flutter, systemic ventricular dysfunction, systemic atrioventricular valve regurgitation, and stenosis of the superior vena caval baffle

  • Interventional therapy is available for sinus node dysfunction, atrial flutter, baffle stenoses and other anatomic problems; a thorough understanding of the cardiac anatomy is required for successful application of these techniques

  • Interval follow-up is required in all patients and systemic ventricular function should be followed

  • At this time, data do not indicate that decline in ventricular function can be prevented by medical therapy

  • Pregnancy and childbirth is possible for women with this anatomy albeit at some increased risk of worsening ventricular dysfunction and systemic atrioventricular valve regurgitation

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Figure 1: Diagram of typical transposition of the great arteries.
Figure 2: Diagram of atrial-level repair for transposition of the great arteries.
Figure 3: The original description of the Mustard technique (surgeon's view).
Figure 4: Late survival for transposition of the great arteries following atrial-level repair.
Figure 5: Transthoracic echocardiogram (parasternal short-axis view) in a patient with transposition of the great arteries following atrial-level repair.
Figure 6: Superior baffle-limb stenosis in a patient with transposition of the great arteries after atrial-level repair and following implantation of a single-chamber pacemaker.
Figure 7: Electrocardiogram in a patient with transposition of the great arteries following atrial-level repair.
Figure 8: Chest radiograph of a patient with transposition of the great arteries and atrial-level repair and following implantation of a dual chamber pacemaker.

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Acknowledgements

Charles P Vega, University of California, Irvine, CA, is the author of and is solely responsible for the content of the learning objectives, questions and answers of the Medscape-accredited continuing medical education activity associated with this article.

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Authors and Affiliations

  1. BA Love is Director of the Congenital Cardiac Catheterization Laboratory at the Mount Sinai Medical Center, D Mehta is Director of the Cardiac Electrophysiology Section and Arrhythmia Service, and Associate Professor of Medicine at the Mount Sinai Hospital and School of Medicine, and VF Fuster is Director of Mount Sinai Heart, the Zena and Michael A Wiener Cardiovascular Institute and the Marie-José and Henry R Kravis Center for Cardiovascular Health, Mount Sinai School of Medicine, New York, NY, USA.,

    Barry A Love, Davendra Mehta & Valentin F Fuster

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Correspondence to Barry A Love.

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Love, B., Mehta, D. & Fuster, V. Evaluation and management of the adult patient with transposition of the great arteries following atrial-level (Senning or Mustard) repair. Nat Rev Cardiol 5, 454–467 (2008). https://doi.org/10.1038/ncpcardio1252

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