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Table 1.  

Suggested Evaluation of Patients with Atrial-level Repair of Transposition of the Great Arteries

Evaluation and Management of the Adult Patient With Transposition of the Great Arteries Following Atrial-level (Senning or Mustard) Repair

Authors: Barry A Love, MD ; Davendra Mehta, MD ; Valentin F Fuster, MD, PhDFaculty and Disclosures


Summary and Introduction


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.


Transposition of the great arteries (TGA) is a common but serious congenital heart malformation, with an incidence of about 1 per 5,000 live births.[1] Until the late 1950s, this lesion was uniformly fatal, with most infants dying before the age of 1 year.[2] The development of the Senning and Mustard atriallevel repairs, however, led to good immediate outcomes and longterm survival. By the 1980s, late complications of these repairs had become well recognized and this, combined with advances in infant cardiac surgery led to the adoption of the neonatal arterial switch procedure, which is now standard therapy for transposition. We estimate there to be approximately 9,000 patients in the US currently alive with an atriallevel repair.[3]

Cardiologists caring for these patients need to understand the anatomy of the repair and the potential late complications associated with it, and be aware of the appropriate therapies. We discuss these issues in this Review.

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