Effects of modified Fontan procedure and common atrioventricular valve replacement on heterotaxy syndrome

Objective: To summarize the experiences of surgical treatment of Heterotaxy syndrome with modified Fontan procedure and common atrioventricular valve replacement.Methods: Three Heterotaxy syndrome patients with moderate to severe common atrioventricular valve regurgitation were treated with modified Fontan procedure combined with ATS prosthetic valve replacement.The size of valve ranged from 29 to 31mm.Results: No patient died,heart functions of the three patients were categorized as NYHA Ⅰ,one patient developed postoperative complete heart block and then treated with pacemaker inplantment.Conclusions: Modified Fontan procedure and common atrioventricular valve replacement is effective for children patients with heterotaxy syndrome,for whom CAVV angioplasty is not satisfying.During the CAVV replacement,chordal attachments should be maintained as much as possible.Besides,pledgeted interrupted sutures should be placed circumferentially around the margins of the valve.Postoperative anticoagulation therapy should start as early as possible to keep INR between 1.5-2.5.