Repair of double outlet right ventricle with doubly-committed ventricular septal defect

Objective: To investigate our surgical results of intraventricular reroutin g in patients having double outlet right ventricle with doubly-committed ve ntricular septal defect. Methods: We undertook repair in 8 patients with th is particular feature. Of these, 2 patients had pulmonary stenosis, and ano ther had interruption of the aortic arch. The subarterial defect was unequi vocally related to both the aortic and the pulmonary orifices in all, albei t slightly deviated towards the aortic orifice in one, and towards the pulm onary orifice in another. Intraventricular rerouting was carried out via in cisions to the right atrium and the pulmonary trunk. To ensure reconstructi on of an unobstructed pulmonary pathway, a limited right ventriculotomy was made in 5. Results: All patients survived the procedure, and are currently doing well, with follow-up of 25 to 194 months, with a mean of 117 +/- 68 months. Catheterization carried out 16 +/- 6 months after repair demonstrat ed excellent ventricular parameters. Mean pulmonary arterial pressure was 1 6 +/- 7 mmHg, being higher than 20 mmHg in 2 patients. No significant obstr uction was found between the right ventricle and the pulmonary arteries. A pressure gradient across the left ventricular outflow tract became signific ant in one patient in whom a small outlet septum was present, and a heart-s haped baffle had been used for intraventricular rerouting. Reoperation was eventually needed in this patient for treatment of the obstruction, which p roved to be progressive. Conclusion: Precise recognition of the morphologic features is of pal-amount importance when choosing the optimal options for biventricular repair in patients with double outlet right ventricle and do ubly-committed interventricular communication.