Surgery treatment of Berry syndrome / 中华胸心血管外科杂志

ABSTRACT

Objective The purpose of this manuscript was to compare the outcomes of different surgical methods of Berry syndrome and to figure out the occurrence of postoperative morbidity.Methods From January 2003 through December 2017,nineteen infants with Berry syndrome underwent one-stage repair at Shanghai Children's Medical Center.There were 12 male and 7 female.Among them,six patients were neonates.The IAA morphology was type A in 17 patients and type B in 2 patients.The APW morphology was type Ⅰ a in 6 patients,type Ⅱ b in 11 patients,and type Ⅲ in 2 patients.Preoperative mechanical ventilation was required in 4 patients.Three different surgical correction techniques were employed to repair the APW and AORPA,including intra-aortic baffle in 8 patients,right pulmonary artery (RPA) detachment in 6 patients,and RPA angioplasty with aortic cuff in 5 patients.The descending aorta was then anastomosed to the aortic arch by an end-to-side anastomosis with a patch augmentation in the anterior wall.Results The mean CPB and aortic cross-clamp time was (146.7 ± 63.5)minutes (range,79 to 260 minutes) and (74.3 ± 27.4)minutes(range,46 to 147 minutes),respectively.There were 3 deaths.One patient died of severe pneumonia and multiorgan dysfunction on postoperative day 26.One patient suffered severe low cardiac output syndrome after surgery and died on postoperative day 1.One patient died of congestive heart failure at 2 months after discharge.Reoperations were required in 3 patients during the follow-up period.RPA arterioplasty with bovine pericardial patch augmentation was performed in 2 patients for RPA restenosis.Lecompte maneuverer was performed in 1 patient to release the compression of the RPA from the ascending aorta.Conclusion The mortality of one-stage repair of Berry syndrome was high.Surgical correction should be performed as soon as diagnosed.An intra-aortic baffle patch is suitable for type Ⅱ a APW defect patients beyond the neonatal period.Pulmonary hypertension crisis is important after surgery.RPA restenosis is the main reason for reoperation.