Diastolic dysfunction is associated with exercise impairment in patients with sickle cell anemia

Diastolic dysfunction is associated with exercise impairment in patients with sickle cell anemia
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DOI:
10.1002/pbc.27113
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发表时间:
2018-08-01
影响因子:
3.2
通讯作者:
Taylor, Michael D.
Taylor, Michael D.
中科院分区:
医学3区
文献类型:
--
作者:
Alsaied, Tarek;Niss, Omar;Taylor, Michael D.

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研究背景左心室舒张功能不全(DD)是镰状细胞贫血(SCA)患者死亡的独立危险因素,并与心脏MRI(CMR)显示的细胞外容积(ECV)增加相关。目的探讨心脏骤停(SCA)患者运动功能损害的发生原因及预后价值。方法采用前瞻性研究的方法,探讨心脏骤停(SCA)患者运动功能损害的发生原因及预后价值(NCT 02410811),20例SCA儿童和成人接受CMR、超声心动图和踏车测力计CPET(年龄范围8- 43岁)。18例患者达到最大运动量,17例(94%)运动能力降低(%预测VO 2小于80%)。6例患者有DD,无收缩功能障碍。与舒张功能正常的患者相比,DD患者的运动能力较低(%预测VO 2 48.29.1% vs.61.2 +/- 11.7%; P=0.01)。左室外侧E/e'比值的z评分是DD的标志,与%预测VO 2呈负相关(r=-0.61,P=0.01)。所有中度至重度运动损伤患者(%预测VO 2
BackgroundLeft ventricular diastolic dysfunction (DD) is an independent risk factor for mortality in sickle cell anemia (SCA) and is associated with increased extracellular volume (ECV) on cardiac MRI (CMR). Exercise impairment is common in SCA, but its causes and prognostic value are not well understood.ObjectiveTo study the effects of DD and ECV on cardiopulmonary exercise test (CPET) in patients with SCA.Methods and ResultsAs part of a prospective study to characterize the cardiomyopathy of SCA (NCT02410811), 20 children and adults with SCA underwent CMR, echocardiography, and cycle ergometer CPET (age range 8-43years). Maximum exercise was reached in 18 patients and 17 (94%) had reduced exercise capacity (%predicted VO2 less than 80%). Six patients had DD and none had systolic dysfunction. Patients with DD had lower exercise capacity compared to patients with normal diastolic function (%predicted VO2 48.29.1%vs. 61.2 +/- 11.7%; P=0.01). The z-score of left ventricular lateral E/e' ratio, which is a marker of DD, was negatively associated with %predicted VO2 (r=-0.61, P=0.01). All patients with moderate-to-severe exercise impairment (%predicted VO2