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目的:建立利用SPECT定量评估肺动脉高压患者右心室心肌血流量(MBF)的方法，并探讨右心室MBF与血流动力学的关系。方法:回顾性分析2018年1月至2019年6月期间于阜外医院诊断为先天性心脏病相关肺动脉高压的患者14例[女13例，男1例，年龄(30.9±13.5)岁]，患者均无右心功能不全表现。所有患者均行动态SPECT心肌灌注显像，采用碲锌镉(CZT)心脏专用SPECT仪进行图像采集，并进行图像重建和物理校正；采用单组织双腔室模型进行动力学建模，并考虑右心血池对右心室心肌的溢出效应，从而获得患者右心室和左心室的MBF。所有患者在显像后1周内接受右心导管检查，测定血流动力学参数；行经胸超声心动图测右心室舒张末期内径。采用Pearson相关分析MBF与其他参数间的相关性。结果:患者右心室MBF为(0.70±0.19) ml·minn -1·gn -1，平均肺动脉压为(68.64±18.18) mmHg(1 mmHg＝0.133 kPa)，两者间存在相关性(n r＝0.716，n P<0.05)。右心室MBF与肺血管阻力[(14.10±7.81) Wood单位]呈正相关(n r＝0.768，n P<0.05)，与右心室舒张末期内径[(32.00±7.75) mm]呈负相关(n r＝-0.624，n P0.05)。n 结论:初步建立了利用SPECT定量评估肺动脉高压患者右心室MBF的方法。对于右心功能代偿的先天性心脏病相关肺动脉高压患者，右心室MBF随肺动脉压和肺血管阻力的升高而增加。“,”Objective:To establish SPECT quantitative method for assessment of right ventricular myocardial blood flow (MBF) and investigate the relationship between right ventricular MBF and pulmonary hemodynamics in patients with pulmonary arterial hypertension.Methods:From January 2018 to June 2019, 14 patients (13 females, 1 male; age: (30.9±13.5) years) in Fuwai Hospital with pulmonary arterial hypertension related to congenital heart disease (PAH-CHD), whose right ventricular function were preserved, were retrospectively analyzed in this study. All subjects underwent dynamic SPECT myocardial perfusion imaging using cadmium-zine-telluride (CZT) SPECT. Full physical correction was applied for imaging reconstruction. One-tissue two compartmental model was used for kinetic analysis and the spill-over effect from right ventricular blood pool to right ventricular myocardium was considered into the correction, thus right ventricular MBF and left ventricular MBF was calculated. Right heart catheterization was performed within one week after SPECT imaging to evaluate the pulmonary hemodynamic parameters, and the right ventricular end-diastolic dimension (EDD) was measured by transthoracic echocardiography. Correlations between the MBF and other parameters were analyzed with Pearson correlation analysis.Results:The right ventricular MBF was (0.70±0.19) ml·minn -1·gn -1, which was significantly correlated with mean pulmonary artery pressure ((68.64±18.18) mmHg (1 mmHg=0.133 kPa); n r=0.716, n P<0.05) and pulmonary vascular resistance ((14.10±7.81) Wood units;n r=0.768, n P<0.05). The right ventricular MBF was also significantly correlated with right ventricular EDD ((32.00±7.75) mm;n r=-0.624, n P0.05).n Conclusions:A method using SPECT to quantitively measure right ventricular MBF in patients with PAH-CHD is preliminarily established. Right ventricular MBF is increased with the increased pulmonary arterial pressure and pulmonary vascular resistance in patients with PAH-CHD.