Delayed arterial switch operation for d-transposition of the great arteries and glucocorticoid remediable aldosteronism

The Jatene arterial switch operation (ASO) for dextro-transposition of the great arteries is ideally performed within the first 2 weeks of life. Clinical circumstances, however, may dictate a delayed ASO and left ventricle "retraining" prior to the procedure. Glucocorticoid remediable aldosteronism (GRA) accounts for 0.5% to 1% of primary aldosteronism. It presents as severe hypertension in infants and children, with poor response to standard antihypertensive medications. To the authors' knowledge, this is the first reported case of GRA in the context of transposition physiology. The management of GRA and a rationale for delayed ASO are discussed.