Objective To optimize the surgical strategies and managements of doubleoutlet right ventricle(DORV)with atrioventricular discordance, the anatomic features, the surgical managements and results were reviewed. Methods From September 1990 to August 2004, 7 cases of DORV with atrioventricular discordance received surgical therapy. The age ranged from 3 years to 7 years. Surgical managements included: ventricular septal defect (VSD )repair and homograft connected the morphologic left ventricle with pulmonary artery in 3 cases, double-switch in 2 cases, modified Fontan in 2 cases. Results The early mortality rate was 14.3% (1/7). The death was because by Homograft infection 2 months after repair. Complete atrioventricular block occurred in one case who received permanent pacemaker later. Obstruction of superior vena cava return and pericardial effusion occurred in one Fontan case who was recovered at the time of discharge. The time of ICU stay was shortest in double-switch patients in 6 days postoperation. Conclusion Double-switch operation is the first choice in correction of DORV with atrioventricular discordance, especially in patients with right ventricular disfunction or tricuspidal regurgitation. If the heart can not be corrected because of combined complicated malformations, the strategy of one ventricular repair can be chosen.
Objective To report the surgical treatment for double outlet of ventricle with atrioventricular discordance. Methods 11 patients aged from 3 to 25 years underwent surgical treatment for double outlet of ventricle with atrioventricular discordance. 10 of them were double outlet right ventricle and the other one was double outlet left ventricle. The surgical treatment included biventricular repair (n=9) and single ventricular repair (n=2). The biventricular repair was performed by intraventricular patch repair and extracardiac valved conduit or homograft valved conduit. The single ventricular repair included bidirected Glenn operation and total cavopulmonary connect. Results There were two early deaths in biventricular repair and no death in single ventricular repair. The cause of death was severe low cardiac output syndrome. Four survivors in biventricular repair were followed up from 1 to 11.5 years, three of them were in NYHA class Ⅰ or Ⅱ and one in NYHA class Ⅲ. Conclusions Double outlet of ventricle with atrioventricular discordance can be treated by biventricular repair or single ventricular repair according to the development of ventricle and pulmonary arteries. Closure of ventricular septal defect , no obstruction from ventricle to great artery and no harm of conduction system are the keys of conventional management to achieve good results.