Liem Robert I, Nevin Mary A, Prestridge Adrienne, Young Luciana T, Thompson Alexis A
Division of Hematology, Oncology & Stem Cell Transplant, Children's Memorial Hospital, Northwestern University Feinberg School of Medicine, Chicago, Illinois 60614, USA.
Pediatr Pulmonol. 2009 Mar;44(3):281-9. doi: 10.1002/ppul.20996.
Concerns about the morbidity and mortality associated with tricuspid regurgitant jet velocity (TRJV) elevation, which may indicate pulmonary hypertension (PHT), in adults with sickle cell disease (SCD) have prompted growing interest in screening the pediatric sickle cell population. The goals of our study were to estimate the prevalence of TRJV elevation and determine its relationship to pulmonary function in children and young adults with SCD at baseline. Seventy-eight subjects (10-24 years old) with SCD underwent prospective screening by Doppler echocardiogram (ECHO), complete lung function evaluation, and laboratory testing as part of standard care at steady state. Tricuspid regurgitation was quantifiable in 68/78 (87%) subjects and peak TRJV was > or =2.5 m/sec in 26/78 (33.3%) evaluated. The frequency of obstruction, restriction, or abnormal gas exchange found on lung function evaluation was not significantly different in subjects with and without TRJV elevation. However, significant inverse correlations were observed between TRJV and both % predicted forced vital capacity (FVC) (r = -0.29, P = 0.022) and oxygen saturation (r = -0.26, P = 0.036). When compared to subjects without TRJV elevation, subjects with TRJV elevation had significantly lower % predicted forced expiratory volume in 1 sec (FEV(1)) (78.9 +/- 14.4 vs. 86.6 +/- 13.0%, P = 0.023), FVC (82.8 +/- 14.1 vs. 90.7 +/- 12.9%, P = 0.017), and oxygen saturation (95.8 +/- 3.2 vs. 97.5 +/- 2.4%, P = 0.016). We found that the combination of low hemoglobin and low % predicted FVC best predicted TRJV elevation (chi(2) = 17.05, P = 0.001) in our cohort, correctly identifying 70% of cases and resulting in positive and negative predictive values of 60 and 74%, respectively. We conclude that in this young population with SCD, TRJV elevation that is not significantly associated with abnormal lung function is common at baseline.
对镰状细胞病（SCD）成人患者中与三尖瓣反流射流速度（TRJV）升高相关的发病率和死亡率的担忧，这可能提示肺动脉高压（PHT），促使人们对筛查小儿镰状细胞人群的兴趣日益增加。我们研究的目的是估计TRJV升高的患病率，并确定其在基线时与SCD儿童和青年成人肺功能的关系。78名年龄在10至24岁的SCD患者接受了前瞻性筛查，包括多普勒超声心动图（ECHO）、完整的肺功能评估以及实验室检测，作为稳定状态下标准护理的一部分。在68/78（87%）的受试者中可量化三尖瓣反流，在26/78（33.3%）接受评估的受试者中，TRJV峰值大于或等于2.5米/秒。在有和没有TRJV升高的受试者中，肺功能评估中发现的阻塞、限制或异常气体交换的频率没有显著差异。然而，观察到TRJV与预测的用力肺活量百分比（FVC）（r = -0.29，P = 0.022）和氧饱和度（r = -0.26，P = 0.036）之间存在显著的负相关。与没有TRJV升高的受试者相比，有TRJV升高的受试者预测的1秒用力呼气量（FEV₁）百分比显著更低（78.9±14.4对86.6±13.0%，P = 0.023）、FVC（82.8±14.1对90.7±12.9%，P = 0.017）和氧饱和度（95.8±3.2对97.5±2.4%，P = 0.016）。我们发现，低血红蛋白和低预测FVC百分比的组合在我们的队列中最能预测TRJV升高（χ² = 17.05，P = 0.001），正确识别70%的病例，阳性和阴性预测值分别为60%和74%。我们得出结论，在这个年轻的SCD人群中，TRJV升高在基线时很常见，且与肺功能异常无显著关联。
