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Division of the morphologically left atrium is a rare abnormality, constituting around 0.1% of congenital heart disease. It may coexist with other cardiac abnormalities, and this association may obscure its recognition. We report an uncommon association with tricuspid atresia and discordant ventriculo-arterial connections, the latter dominating the clinical picture. Accurate diagnosis was made by transthoracic cross-sectional echocardiography, and the patient was referred to surgery without need for cardiac catheterisation.

Background: Less than perfect coronary artery translocation accounts for the majority of perioperative deaths after the arterial switch procedure for transposition of the great arteries. Some types of coronary arterial anatomy are associated with a higher risk of death., Methods: Prospective epicardial ultrasound examination of all neonates with failing left ventricle or difficulty in weaning off cardiopulmonary bypass was performed after completion of the arterial switch operation during a 2-year period from March 1994 to February 1996. The aim was to identify any mechanical, and potentially remediable, factors accounting for ventricular failure., Results: Four neonates fulfilling the above criteria were identified during a 2-year period when epicardial echocardiography was routinely applied. In 2 patients coronary arterial problems in the form of kinking of the proximal left coronary artery (1 patient) and extrinsic compression of the artery by the neo-pulmonary trunk (1 patient) were identified and successfully corrected. In 2 other patients, supravalvar aortic stenosis was recognized, leading to prompt revision., Conclusions: Epicardial echocardiography has an important "troubleshooting" role in the subgroup of patients with a suboptimal hemodynamic result after the arterial switch operation. Patients with unusual coronary anatomy should routinely be candidates for such studies.

Objectives: A combined atrial and arterial switch procedure was performed in selected patients with congenitally corrected transposition to establish the morphological left ventricle as the systemic ventricle. Immediate and early follow up results are presented., Background: Progressive right ventricular dysfunction and tricuspid regurgitation are common in patients with congenitally corrected transposition who undergo repair of associated lesions. A surgical procedure which re-establishes the left ventricle as the systemic ventricle should improve functional results., Methods: Four symptomatic children aged from 9 months to 3 years 1 month (mean 2 years 3 months) with congenitally corrected transposition and ventricular septal defect underwent both an atrial and arterial switch procedure and were followed up for a mean of 12 months (range 6-21 months)., Results: There were no early or late deaths. Conduction abnormalities worsened in two patients. Hospital stay ranged from 8 to 17 days (mean 13 days). The cardiothoracic ratio decreased from a mean (range) of 0.65 (0.6 to 0.71) to 0.58 (0.52 to 0.6). Currently, three patients are in functional class I and one child is in functional class II., Conclusions: The combination of an atrial and an arterial switch procedure in symptomatic children with congenitally corrected transposition establishes the left ventricle as the systemic ventricle. The initial experience is encouraging with excellent immediate and early follow up results.

Seventy three infants who underwent neonatal anatomical correction for transposition of the great arteries with or without a ventricular septal defect were reviewed for evidence of conduction and rhythm abnormalities on preoperative and postoperative 12 lead electrocardiograms and during 24 hour Holter monitoring. There was a partial right bundle branch block pattern in 47% (29/62) of all patients and in 60% (24/40) of those with simple transposition. Complete right bundle branch block was noted in 21% including 5% with simple transposition. Holter monitoring showed sinus rhythm in all patients except three: one had episodes of supraventricular tachycardia, another an intermittent second degree heart block, and a third a complete heart block. Atrial extrasystoles were noted in 47% (29/62) of patients but were frequent in only three patients. Occasional unifocal ventricular extrasystoles were encountered in 37% (23/62) of patients and were frequent in a further 3% (2/62). Only one patient (2%) developed multifocal ventricular extrasystoles. The frequency of important cardiac arrhythmias after neonatal anatomical correction of transposition of the great arteries was 5%, significantly less than that reported after atrial inflow diversion for the same malformation.

Fifty patients, 21 with transposition of the great arteries plus ventricular septal defect, four with double-outlet right ventricle plus ventricular septal defect, and 25 with transposition of the great arteries plus intact ventricular septum, have undergone anatomic correction. There were four hospital deaths, all in the patients with ventricular septal defects (overall mortality rate 8%). The Lecompte maneuver was used in all patients, and in the last 25 patients the coronary arteries were transposed to medially hinged trapdoor flaps created in the neoaorta. In 10 patients (20%) intraoperative adjustments were necessary to the great vessels and coronary arteries to achieve unobstructed coronary artery flow. There have been no late deaths. Postoperative cardiac catheterization in 19 patients has revealed stenosis on the new main pulmonary artery suture line to be the main early problem. Anatomic correction of transposition (with or without ventricular septal defect) and double-outlet right ventricle septal defect would seem to be a good operative alternative to the Mustard or Senning operation, with the advantage of incorporating the left ventricle into the systemic circulation.