Division of Cardiology, Department of Pediatrics, Stanford Medical School, 750 Welch Road, Stanford, CA, USA.
Division of Pediatric Cardiology, 1540 East Hospital Drive, Ann Arbor, MI, 48109, USA.
Pediatr Cardiol. 2021 Aug;42(6):1394-1404. doi: 10.1007/s00246-021-02624-y. Epub 2021 May 13.
Overall survival of patients with hypoplastic left heart syndrome (HLHS) has shown continued improvement. Right ventricular (RV) dysfunction, in the long term, adversely affects prognosis in these patients. This study examines changes in echocardiographic markers of RV function in a longitudinal cohort. We retrospectively reviewed patients with HLHS managed at our institution from 7/1994 to 1/2016. Follow-up included surgical and clinical data, and echocardiographic measures. Measures of RV function preceding and following all three stages of single ventricular palliation were collected. Freedom from transplant-free survival was assessed by Kaplan-Meier analysis. Multivariable associations with time to death or transplant were explored using the Cox proportional hazards model. A total of 120 patients with HLHS were identified. Norwood operation was performed in all patients. The probability of survival for the cohort was 71 ± 4.4%, 69 ± 4.5% and 66 ± 4.7% at 1, 2 and 5 years respectively after stage I Norwood operation. RV fractional area change (FAC), compared to post-Norwood was decreased at all subsequent stages with the greatest change noted post-superior cavo-pulmonary shunt from 40.7 ± 9.3% to 31.1 ± 8.3% (p < 0.001). Similarly, tricuspid valve annular systolic excursion (TAPSE) Z-score declined from -2.9 ± 1.3 to -9.7 ± 1.3 (p < 0.001) with a decrement at every stage of evaluation. In comparison to patients with post-Norwood RV FAC >35% and TAPSE Z-score > -5, patients with RV FAC ≤ 35% and TAPSE Z-score ≤ -5 had a significantly lower transplant-free survival (p < 0.0001). In patients with HLHS undergoing staged palliation, decrement in RV function manifests longitudinally. Post-Norwood RV FAC and TAPSE Z-score appear to be early markers of poor outcome in this population.
患有左心发育不全综合征（HLHS）的患者的总体生存率持续提高。长期来看，右心室（RV）功能障碍会对这些患者的预后产生不利影响。本研究通过纵向队列研究检查 RV 功能的超声心动图指标变化。我们回顾性地审查了 1994 年 7 月至 2016 年 1 月在我院接受治疗的 HLHS 患者。随访包括手术和临床数据以及超声心动图测量。收集了所有单心室姑息治疗三个阶段前后的 RV 功能测量值。通过 Kaplan-Meier 分析评估无移植生存。使用 Cox 比例风险模型探讨与死亡或移植时间相关的多变量关联。共确定了 120 例 HLHS 患者。所有患者均接受了 Norwood 手术。一期 Norwood 手术后，队列的生存率分别为 1 年、2 年和 5 年时的 71±4.4%、69±4.5%和 66±4.7%。与 Norwood 手术后相比，在所有后续阶段，RV 分数面积变化（FAC）均降低，在接受 superior cavo-pulmonary shunt 后变化最大，从 40.7±9.3%降至 31.1±8.3%（p<0.001）。同样，三尖瓣环收缩期位移（TAPSE）Z 评分从-2.9±1.3 降至-9.7±1.3（p<0.001），在评估的每个阶段均有下降。与 Norwood 后 RV FAC>35%和 TAPSE Z 评分>-5 的患者相比，RV FAC≤35%和 TAPSE Z 评分≤-5 的患者无移植生存明显较低（p<0.0001）。在接受分期姑息治疗的 HLHS 患者中，RV 功能的下降呈纵向表现。Norwood 后 RV FAC 和 TAPSE Z 评分似乎是该人群不良预后的早期标志物。
