Two-dimensional echocardiographic assessment of right ventricular functionas a predictor of outcome in hypoplastic left heart syndrome

This study was undertaken to assess the importance of right ventricular fun ction at the time of initial presentation on early and intermediate outcome in patients with hypoplastic left heart syndrome (HLHS). Several studies h ave attempted to define physiologic risk factors for poor early outcome fol lowing the Norwood palliation for HLHS. No clinical or hemodynamic factors including right ventricular function have been found to reliably predict No rwood I operative survival. The relation between initial ventricular functi on and later survival has not been investigated. To assess the importance o f right ventricular (RV) function at the time of initial presentation on ou tcome in patients with HLHS, systolic function was determined by qualitativ e and quantitative methods in 60 consecutive patients before surgical inter vention. The effects on stage I operative survival, survival to stage II, a nd overall survival were analyzed. Initial RV function did not impact on st age I survival, However, analysis of later outcome of the stage I survivors showed that those with prestage I RV dysfunction had significantly greater mortality before stage II. Actuarial survival 18 months after Norwood surg ery was 93% for patients with initially normal RV function compared with 47 % for those with abnormal function (p = <0.005), The relative risk for late r mortality was approximately 11 times greater for patients with initial RV dysfunction, Thus, RV dysfunction identifiable soon after initial presenta tion does not impact on early survival after Norwood I operation for HLHS. Intermediate and overall survival, however, is significantly decreased in p atients with initially diminished RV function. (C)2000 by Excerpta Medico, Inc.