Surgical Technique
Re-sternotomy was performed in all cases. Femoral cannulation and initiation of cardiopulmonary bypass before sternal re-entrance was not deemed mandatory in any of the cases. Following aortic and bicaval (or tricaval in cases with a persistent superior venae cavae) cannulation aorta was cross clamped except pt. no 12. Cold antegrade blood cardioplegia was administered where intermittent doses were applied at every 15 minutes. Pulmonary artery was divided above the level of pulmonary valve, except for the cases with pulmonary atresia where there was no antegrade pulmonary blood flow. Pulmonary cusps were excised or squeezed between the double row matress suture lines closing the pulmonary outflow of the ventricle. Inferior venae cavae (IVC) was transected at the level of atrial entrance and a PTFE tubular conduit was anastomosed to IVC with an end to end fashion. End to side anastomosis was performed between the pulmonary artery and the conduit and care was taken in order not to compress the pulmonary venous return throughout the course of the conduit from IVC to pulmonary artery in the pericardial cavity. In cases where an intra-extracardiac technique was implemented, the anastomosis was performed within the atrium encircling the ostium of the IVC. Then the conduit was passed from the atrium to outside into the pericardial cavity. Total circulatory arrest was not used in any case. When fenestration was needed, in cases with an extracardiac technique, a side to side anastomosis was performed between the conduit and the atrium. In intra-extracardiac Fontan cases, a punch was used to make a fenestration and leave it inside the atrium. Temporary inotropic agents were infused in pt. no 3,4 and 7 where dobutamine was the first line choice. In patient no. 14, pulmonary reconstruction was performed following transection of the ascending aorta in order to provide better exposure of the pulmonary arteries. Permanent pace maker was not necessary in any case. The mean cardiopulmonary bypass and aortic cross clamp times were 69.7 ± 27.1 and 51.6 ± 25.3 minutes, respectively. All of the patients were hemodynamically stable with the sternum closed when they leaved the operating room. Any postoperative revision for bleeding or any other reason was not encountered in the early postoperative period.
The mean duration of intubation and intensive care unit stay were 7.8 ± 3.5 hours and 2.3 ± 3.5 days, respectively. The chest drainage tubes were in place until the daily drainage was less than 2cc/kg. Mean duration of drainage was 3.9 ± 5.3 days. Oral warfarin, aspirin, sildenafil citrate and bosentan were routinely administered in all of the patients after FP. The target INR was 2 to 2.5. Steroids were administered when the daily drainage exceeded 5cc/kg. Following removal of the chest tubes, daily consecutive chest x-rays were obtained in order to detect pleural effusion. Any chest tube re-insertion was not needed for re-accumulation of the pleural fluid. We did not encounter any phrenic nerve paralysis in this patient population. Mean duration of discharge was recorded as 10 ± 1.8 days.
Mean follow up period was 4.8 ± 7.7 years (range: 1 to 11 years). All of the patients are on lifelong Aspirin (5mgr/kg) therapy. Oral warfarin and sildenafil citrate (3mgr/kg in 3 divided doses) are administered at least for one year, and afterwards individual decision is given primarily according to a presence of fenestration. Oral endothelin receptor antagonist, bosentan is administered at least for one month. All of the patients were discharged with diuretics and angiotensin converting enzyme inhibitors in order to achieve a normal blood pressure.
Early or mid-term Fontan failure and mortality was not encountered. In pt. no 14, fenestration was closed via femoral access one year after the FP. We have not encountered any case with significant arrhythmia, plastic bronchitis or protein losing enteropathy in our patient population. In cases with AV valve repair, trace to mild regurgitations are encountered. All of the patients in our population are followed up with ejection fractions above 50% with NYHA class I-II symptoms at annual outpatient visits.