Borrelli Nunzia, Di Salvo Giovanni, Sabatino Jolanda, Ibrahim Aladino, Avesani Martina, Sirico Domenico, Josen Manjit, Penco Maria, Fraisse Alain, Michielon Guido
Department of Paediatric Cardiology, Royal Brompton Hospital, Sydney Street, London SW36NP, United Kingdom.
Department of Paediatric Cardiology, Royal Brompton Hospital, Sydney Street, London SW36NP, United Kingdom.
Int J Cardiol. 2020 Oct 15;317:56-62. doi: 10.1016/j.ijcard.2020.03.085. Epub 2020 Apr 1.
Hypoplastic Left Heart Syndrome (HLHS) has high mortality and morbidity and systemic right ventricle (RV) dysfunction may play a key-role. Study aim is to evaluate the accuracy of speckle-tracking echocardiographic (STE) assessment of RV deformation and 2D standard echo parameters in predicting outcome in HLHS patients.
We studied 27 HLHS patients (17 male) who successfully completed Norwood palliation. All the patients underwent in-hospital interstage stay. Serial echocardiographic assessment was performed: baseline, one-month after Norwood, three-months after Norwood, one-week before bidirectional cavopulmonary anastomosis (BCPA) and two-months after BCPA. From the apical view we measured: tricuspid annulus peak systolic excursion (TAPSE), fractional area change (FAC), longitudinal strain (LS) and strain rate (LSR).
After a mean follow-up of 1.18 (± 1.16) years, 8 out of 27 of the included patients met the composite endpoint of death/heart transplant (HT). At pre-Norwood assessment, there was no difference in echo measurements between survivors and patients with events. In death/HT group TAPSE and LS declined already one-month after Norwood procedure: TAPSE ≤5 mm had good sensitivity (85.71%) and moderate specificity (63.16%) for death/HT (AUC = 0.767); a decrease of LS > 8.7% vs baseline showed 100% sensitivity and 84.21% specificity for death/HT (AUC = 0.910). At multivariate analysis, one-month-after-Norwood LS drop >8.7% was the best predictor of outcome (P = 0.01).
RV dysfunction in HLHS carries prognostic value. Our findings encourage serial measurements of RV function to identify the subgroup of HLHS patients at higher risk. In our experience, ∆ LS showed the best predictive value.
左心发育不全综合征（HLHS）的死亡率和发病率很高，系统性右心室（RV）功能障碍可能起关键作用。研究目的是评估斑点追踪超声心动图（STE）对RV变形的评估准确性以及二维标准超声参数在预测HLHS患者预后方面的作用。
我们研究了27例成功完成诺伍德姑息手术的HLHS患者（17例男性）。所有患者均在院内进行分期住院。进行了系列超声心动图评估：基线、诺伍德手术后1个月、诺伍德手术后3个月、双向腔肺吻合术（BCPA）前1周以及BCPA后2个月。从心尖视图测量：三尖瓣环收缩期峰值位移（TAPSE）、面积变化分数（FAC）、纵向应变（LS）和应变率（LSR）。
平均随访1.18（±1.16）年后，27例纳入患者中有8例达到死亡/心脏移植（HT）的复合终点。在诺伍德手术前评估时，幸存者和发生事件的患者之间的超声测量无差异。在死亡/HT组中，诺伍德手术后1个月TAPSE和LS就已下降：TAPSE≤5 mm对死亡/HT具有良好的敏感性（85.71%）和中等特异性（63.16%）（AUC = 0.767）；与基线相比，LS下降>8.7%对死亡/HT的敏感性为100%，特异性为84.21%（AUC = 0.910）。在多变量分析中，诺伍德手术后1个月LS下降>8.7%是预后的最佳预测指标（P = 0.01）。
HLHS中的RV功能障碍具有预后价值。我们的研究结果鼓励对RV功能进行系列测量，以识别HLHS中高风险患者亚组。根据我们的经验，∆LS显示出最佳预测价值。
