Abstract

Background. Perioperative and long-term problems associated with the Fontan circulation are substantial. There has been an exploration of extending the limits of a biventricular ventricular repair by using a superior vena cava-to-pulmonary artery anastomosis. This type of repair is known as a 1 1 2 ventricle repair. Methods. Patients having defects of the pulmonary ventricle in size or function have undergone 1 1 2 ventricle repairs with or without creation of an atrial septal defect. Repairs with tricuspid z values as small as −10 and predicted pulmonary ventricular volumes as low as 30% have been reported. The 1 1 2 ventricle repair technique has also been used in special situations associated with an arterial switch or double switch procedure. Results. Mortality has ranged from 0% to 12%. Complications have included persistent elevation of superior vena cava pressure, intermittent periorbital edema, and 1 superior vena caval aneurysm requiring takedown. There appears to be an increased risk of perioperative pleural effusions and chylothorax. Protein-losing enteropathy and chronic atrial arrhythmias have not been present. Conclusions. Successful 1 1 2 ventricle repairs have been reported for morphologically small or poorly functioning pulmonary ventricles and special situations. Intermediate-term follow-up is favorable when compared with reported outcomes for the Fontan circulation.

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