Altmann K, Printz B F, Solowiejczky D E, Gersony W M, Quaegebeur J, Apfel H D
Department of Pediatrics, Columbia University College of Physicians and Surgeons, Babies and Children's Hospital, New York, New York 10032, USA.
Am J Cardiol. 2000 Nov 1;86(9):964-8. doi: 10.1016/s0002-9149(00)01131-0.
This study was undertaken to assess the importance of right ventricular function at the time of initial presentation on early and intermediate outcome in patients with hypoplastic left heart syndrome (HLHS). Several studies have attempted to define physiologic risk factors for poor early outcome following the Norwood palliation for HLHS. No clinical or hemodynamic factors including right ventricular function have been found to reliably predict Norwood I operative survival. The relation between initial ventricular function and later survival has not been investigated. To assess the importance of right ventricular (RV) function at the time of initial presentation on outcome in patients with HLHS, systolic function was determined by qualitative and quantitative methods in 60 consecutive patients before surgical intervention. The effects on stage I operative survival, survival to stage II, and overall survival were analyzed. Initial RV function did not impact on stage 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 surgery was 93% for patients with initially normal RV function compared with 47% for those with abnormal function (p = <0.005). The relative risk for later mortality was approximately 11 times greater for patients with initial RV dysfunction. Thus, RV dysfunction identifiable soon after initial presentation does not impact on early survival after Norwood I operation for HLHS. Intermediate and overall survival, however, is significantly decreased in patients with initially diminished RV function.
本研究旨在评估左心发育不全综合征（HLHS）患者初次就诊时右心室功能对早期和中期预后的重要性。多项研究试图确定HLHS患者在接受诺伍德姑息手术后早期预后不良的生理危险因素。尚未发现包括右心室功能在内的任何临床或血流动力学因素能够可靠地预测诺伍德一期手术的生存率。初次就诊时的心室功能与后期生存率之间的关系尚未得到研究。为了评估HLHS患者初次就诊时右心室（RV）功能对预后的重要性，我们采用定性和定量方法对60例连续患者在手术干预前的收缩功能进行了测定。分析了其对一期手术生存率、二期生存率和总体生存率的影响。初次就诊时的右心室功能对一期生存率没有影响。然而，对一期幸存者后期预后的分析表明，术前右心室功能不全的患者在二期前的死亡率显著更高。诺伍德手术后18个月，初次就诊时右心室功能正常的患者的精算生存率为93%，而功能异常的患者为47%（p =<0.005）。初次就诊时右心室功能不全的患者后期死亡的相对风险大约高出11倍。因此，初次就诊后不久即可识别的右心室功能不全对HLHS患者诺伍德一期手术后的早期生存率没有影响。然而，初次就诊时右心室功能最初受损的患者的中期和总体生存率显著降低。
