Electrophysiologic consequences of the Mustard repair of d-transposition of the great arteries

Division of Cardiology, Children's Hospital of Philadelphia, Pennsylvania 19104.

This study describes the electrophysiologic effects of the Mustard repair of d-transposition of the great arteries on the sinus node and on conduction and refractoriness in the atrium, atrioventricular (AV) node and ventricle. Seventy-two electrophysiologic studies were performed on 64 patients after the Mustard operation. Standard rest and 24 hour ambulatory electrocardiograms were evaluated. Catheter endocardial atrial mapping, available in 67 of the 72 studies, revealed that sinus rhythm was present in the atria in 33 patients, ectopic atrial rhythm in 26 and junctional rhythm in 8. Intraatrial conduction delays with very late activation of the low medial, low lateral or low right atrium at the AV junction were present in 53 of 59 patients with atrial or sinus rhythm. Sinus node function was normal in only nine patients. Prolonged refractory periods were found in the right atrial portion of the new right atrium when compared with the left atrial portion of the new right atrium. Forty-one percent of the patients had prolongation of atrial refractoriness with shortening of the paced cycle lengths. Fifty-one percent developed sustained intraatrial reentry with programmed atrial stimulation. Forty-eight percent of these patients with inducible atrial tachycardia have since developed clinical episodes of atrial flutter. In summary, electrophysiologic studies revealed significant abnormalities of sinus node function and atrial conduction and refractoriness. Alone or in combination, these abnormalities, which result in severe bradycardia or rapid atrial arrhythmias, may lead to sudden death. The Jatene or arterial switch procedure may provide an appropriate alternative operation in this group of patients.

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