Systemic right ventricular function in congenitally corrected transposition depends on septal geometry. Suprasystemic left ventricular systolic pressures and high end-diastolic pressures after Fontan surgery paradoxically preserve right ventricular function.

We describe an unusual case of congenitally corrected transposition in which left-sided obstructions included a supravalvar tricuspid ring, a parachute deformity of the valve itself, and coarctation of the aorta. Surgical correction was performed in 3 stages. Initially we repaired the coarctation and banded the pulmonary trunk. Then we removed the band and, finally, attempted unsuccessfully to replace the tricuspid valve. When coarctation of the aorta occurs in congenitally corrected transposition, it is always wise to search carefully for abnormalities of the tricuspid valve.

The congenital cardiac malformation characterized by discordant connections between the atriums and ventricles, as well as those between the ventricles and the arterial trunks, has been given many names. The terms atrioventricular discordance, l-transposition of the great arteries, ventricular inversion, and congenitally corrected transposition have all been used. Regardless of terminology, this complex congenital anomaly has only recently been studied to analyze the long-term effects of its natural history and outcomes following traditional surgical repair of the associated malformations which serve to uncorrect the circulatory pathways. As more patients survive into adulthood, the effects of this condition are now better understood, and the surgical approaches used in the past are being re-examined in light of longer-term follow up.

Based on experience in several centers, the double switch operation has reportedly become the standard surgical therapy for congenitally corrected transposition. We report and discuss here the use of a “ventricle and a half” double switch operation performed due to the concerns raised intraoperatively because of the size of the morphologically right ventricle. Although the long-term course of such a procedure in this setting remains unknown, we submit that the proposed benefits of the double switch operation, even when used in the “ventricle and a half” configuration, may be superior to the alternatives.

Congenitally corrected transposition is a rare congenital anomaly, with only a few cases diagnosed and reported prenatally even in the largest fetal series. To determine the morphologic features and outcome for the lesion as recognized during fetal life, we reviewed the fetal and postnatal echocardiograms and medical records of 11 consecutive cases of congenitally corrected transposition. These were identified among 230 (4.7%) consecutive cases of structural cardiac disease referred to our fetal cardiology unit over a period of 4 years. The mean gestational age at diagnosis was 24.7 weeks. Reasons for referral were suspected complete transposition, abnormal position of the heart, and bradyarrhythmias. Associated cardiac lesions included an abnormal cardiac position in 6 cases, ventricular septal defect in 8, obstruction of the subpulmonary outflow tract in 6, tricuspid valvar displacement in 5, and complete atrioventricular block in 2. Only 3 of the cases had mild tricuspid regurgitation prior to birth. Termination was chosen in 4 cases with severe obstruction to pulmonary flow. Of the remaining cases, 2 patients died at 3 and 12 months after birth, respectively. Both developed significant tricuspid regurgitation associated with unexpected major arrhythmias. The remaining 5 patients are alive and relatively well at a mean follow-up of 25.4 months. An epicardial pacemaker was inserted in 1 because of complete atrioventricular block. We conclude that prenatal counseling must be guarded following the diagnosis of congenitally corrected transposition, even in fetuses with an apparently favorable state at initial examination. Some of these cases may undergo major and unexpected changes, particularly with regard to cardiac rhythm and tricuspid valvar function, with concomitant significant changes in prognosis.