Corrective surgery
30 of the 37 patients (81%) were considered correctable. One patient is awaiting correction after complete unifocalization.Twenty-nine patients underwent a total correction as described earlier which was successful in 28 patients (97%). Median age at correction was 2.6 years (range 1 to 17 years) and median interval between unifocalization and correction was 8 months (range 1 to 48 months). In all patients the VSD was closed. One patient needed a takedown during operation because of suprasystemic pressures of the right ventricle and impossibility to wean the patient from the extracorporeal circulation. One patient had a resternotomy for persistent bleeding the same day and recovered without complications.
Operative mortality was 3% (n=1). This patient had a reoperation for leakage of the proximal suture of the homograft at the same day of the correction. Because of hypoperfusion a major CVA developed and the patient died. Seven patients were not eligible for correction. In three patients there was pulmonary hypertension. One patient had malperfusion of the complete left lung, one patient had a hypoplasia of the right ventricle not suitable for repair, one patient had hypoplasia of the lungs and airways in combination with neuro-cognitive disorders and one patient was considered a too high risk operation due to developmental disorders. Figure 1 shows a flowchart of our patient population.
In the ten patients with 22q11 deletion, 1 is awaiting correction, 3 were ineligible (2 died, 1 still alive) for final correction and 6 patients had a definitive correction. One patient died post-operatively due to cerebral-vascular accident. Compared to non-syndromic patients, a lower percentage of the 22q11 deletion patients were reaching their final correction (23 out of 29 (79%)) versus 6 out of 9 (67%) respectively), although not significant (p=0.55).