[Complex plasty of the atrioventricular valve in defects with a functional single ventricle--surgical technic and...

Congenital heart diseases with a functionally single ventricle can be surgically treated using total cavopulmonary connection. Regurgitation of atrioventricular valves represents one of risk factors for death and unfavourable long-term result after this operation. During 1996-2001 total cavopulmonary connection with a complex plastic repair of atrioventricular valves was performed in 8 patients at the age from 1.4 to 13.3 years (median 7.8 years). All patients had very complex congenital heart diseases with severe malformation of atrioventricular valves. All but one had pulmonary stenosis or atresia. Six patients had common atrioventricular orifice, 5 patients had bilateral superior caval veins and 4 patients had dextrocardia or mesocardia. In 6 (75%) patients one or two palliative operations had been performed earlier. Before surgery all patients were cyanotic with severe hypoxemia and polyglobulia. All patients had regurgitant atrioventricular valves. The surgery was performed from the midline sternotomy approach, in extracorporeal circulation and moderate hypothermia. The surgery consisted in excision of the atrial septum, construction of a direct connection of all superior and inferior caval veins with the pulmonary artery, transsection of the pulmonary trunk and complex plastic repair of atrioventricular valves. The inferior caval vein was derived to the pulmonary artery using an intraatrial lateral tunnel in 3 patients and using an external conduit from a Goretex vascular prosthesis in 5 patients. In 5 patients a fenestration in tunnel or conduit permitting decompression of the systemic venous return was done. Individualized plastic repair of atrioventricular valves consisted in the suture of the central portions of both common leaflets. Four patients required also commissuroplasty of one to four commissures and two patients required annuloplasty. All patients survived the early postoperative period. In the majority of them complications occurred: hypoxemia in 3, pleural effusions in 3, low cardiac output in 2 and bleeding in 1 patient. In two patients reintervention for severe hypoxemia was necessary. Postoperative echocardiographic examination proved good result in all patients. In 6 (75%) patients the grade of regurgitation of atrioventricular valves decreased immediately after the repair, in the two remaining patients the degree of regurgitation decreased during the follow up. One patient with the most severe residual regurgitation which progressed during the follow up died 2.5 years later under the signs of congestive heart failure after spontaneous closure of fenestration. The 7 (87.5%) living patients remain in good clinical condition in NYHA class I or II. Our experience proved that it is possible to perform total cavopulmonary connection with low mortality and good midterm results even in patients with hemodynamically significant regurgitation of atrioventricular valves. Persistent severe or progressive regurgitation, however, requires surgical reintervention.