Covi Stuart, Ravindranath Yaddanapudi, Farooqi Ahmad, Savasan Sureyya, Chu Roland, Aggarwal Sanjeev
Division of Pediatric Cardiology, Children's Hospital of Michigan, Wayne State University School of Medicine, Detroit, MI, 48201, USA.
Division of Pediatric Cardiology, St. John Hospital and Medical Center, Detroit, MI, 48236, USA.
Pediatr Cardiol. 2018 Feb;39(2):365-374. doi: 10.1007/s00246-017-1764-9. Epub 2017 Nov 1.
Hematopoietic stem cell transplant (HSCT) is a therapeutic option for patients with sickle cell disease (SCD) and severe acquired aplastic anemia (SAA). HSCT may have beneficial effects on ventricular function in damaged myocardium. We hypothesized improvement in ventricular performance and pulmonary hypertension following HSCT with strain echocardiography in SCD and SAA. Echocardiographic strain and other standard functional data were obtained via retrospective cohort analysis of patients (n = 23) with SCD and SAA who underwent HSCT and were followed at a single center between 2000 and 2014. Left ventricular global longitudinal strain was below normal at baseline, and decreased significantly (from - 16.6 to - 11.1, P = 0.05) from pre-HSCT to the initial post-HSCT echocardiogram at 109 (SD ± 83) days. At 351 (SD ± 115) days, longitudinal strain improved significantly from initial decline (from - 11.1 to - 17.5, P = 0.009) but was comparable to baseline (P = 0.43). Other measurements of bi-ventricular function did not change significantly. Tricuspid regurgitation velocities as surrogates for pulmonary hypertension improved in the subset of patients with baseline elevated values although data points were limited. Abnormal myocardial systolic function was detected at baseline with strain imaging. HSCT was associated with initial worsening longitudinal strain values, followed by improvement to baseline levels by 1 year. Insufficient data exist on whether pulmonary hypertension improves after HSCT.
造血干细胞移植（HSCT）是镰状细胞病（SCD）和严重获得性再生障碍性贫血（SAA）患者的一种治疗选择。HSCT可能对受损心肌的心室功能产生有益影响。我们假设通过应变超声心动图评估，HSCT后SCD和SAA患者的心室功能及肺动脉高压会得到改善。通过对2000年至2014年间在单一中心接受HSCT并随访的SCD和SAA患者（n = 23）进行回顾性队列分析，获取超声心动图应变及其他标准功能数据。左心室整体纵向应变在基线时低于正常水平，从HSCT前到HSCT后109（标准差±83）天的首次超声心动图检查时显著降低（从-16.6降至-11.1，P = 0.05）。在351（标准差±115）天时，纵向应变从最初的下降显著改善（从-11.1升至-17.5，P = 0.009），但与基线水平相当（P = 0.43）。双心室功能的其他测量指标无显著变化。在基线值升高的患者亚组中，作为肺动脉高压替代指标的三尖瓣反流速度有所改善，尽管数据点有限。应变成像在基线时检测到心肌收缩功能异常。HSCT与纵向应变值最初恶化相关，随后在1年内改善至基线水平。关于HSCT后肺动脉高压是否改善的数据不足。
