INTERMEDIATE-TERM OUTCOME AFTER INTRACARDIAC REPAIR OF ASSOCIATED CARDIAC DEFECTS IN PATIENTS WITH ATRIOVENTRICULAR AND VENTRICULOARTERIAL DISCORDANCE

Background Limited information is available concerning long-term resul ts, especially systemic right ventricular (RV) or tricuspid valvular f unction, after intracardiac repair of anomalies associated with discor dant atrioventricular (AV) and ventriculoarterial (VA) connections ('' congenitally corrected transposition of the great arteries''). Methods and Results We retrospectively reviewed the intermediate-term follow- up of 28 patients, totaling 158 patient-years (median, 60 months), aft er intracardiac repair involving closure of a ventricular septal defec t (VSD) with or without additional surgery, Seven patients had VSD clo sure alone, 5 had VSD repair with pulmonary stenosis relief, and 16 ha d VSD closure with conduit insertion between left ventricle and main p ulmonary artery. Hospital mortality was 4% (1 of 28 patients; 70% conf idence limits, 0.07% to 12%) and the 1-, 5-, and 10-year actuarial sur vival probabilities were 89%, 83%, and 83%, respectively. Twenty-one o f 24 long-term survivors were in New York Heart Association functional class I and 3 were in class Ii, Sixteen of 24 patients showed increas ing tricuspid regurgitation (TR) of more than moderate degree, which o ccurred within 3 years after surgery in 7 patients. Twelve of 22 patie nts showed deterioration of RV pump function, mainly (9 of 12 patients ) within 3 years postoperatively. The pulmonary to systemic flow ratio at the preoperative cardiac catheter study was significantly (P<.05) higher in patients who developed RV dysfunction (2.3+/-1.0, mean+/-SD) than in those with well-maintained RV function (1.4+/-0.6). Conclusio ns Intermediate-term results of intracardiac repair for AV and VA disc ordance were satisfactory in terms of survival and clinical functional status; however, there is concern about systemic RV dysfunction with development of TR relatively early after the operation. Alternative su rgical approaches such as anatomic correction or Fontan repair for cas es unsuitable for biventricular repair may improve the long-term resul ts, including ventricular and valvular function.