摘要Background While echocardiography has been a pivotal screening test in pulmonary arterial hypertension (PAH),the presence of structural cardiac defects may affect the ability to reliably predict pulmonary artery pressures (PAPs).This study sought to evaluate the accuracy of Doppler echocardiography (DE) for estimating PAPs in adult atrial septal defect (ASD) patients with PAH.Methods A prospective study was carried out to compare the echocardiographic assessment of PAP with the same pressures obtained by right heart catheterization (RHC) in adult ASD patients with PAH who underwent simultaneous DE and RHC.Bland-Altman analyses were performed to evaluate the agreement between DE and RHC measurements of PAPs.Results Two hundred and fifty-seven patients were included in the study.A significant overestimation of the systolic pulmonary arterial pressure (sPAP) and mean pulmonary artery pressure (mPAP) was reported by echocardiography compared with those by catheterization ((81.8±26.9) mmHg vs.(72.9±26.9) mmHg,P ＜0.01; (51.9±16.4) mmHg vs.(41.4±17.2) mmHg,P ＜0.01,respectively).Twenty-one percent (55/257) of the patients had PAH when estimated by echocardiography whereas showed normal results in the subsequent catheterization test.Using Bland-Altman analytic methods,the bias for the echocardiographic assessment of the sPAP was 9.1 mmHg with 95％ limits of agreement ranging from-24.4 to 42.6 mmHg.For mPAP measurement,the bias was 10.5 mmHg with 95％ limits of agreement ranging from-12.4 to 33.4 mmHg.On multiple linear regression analysis,age,gender,body surface area,ASDs' diameter,PVR,diastolic blood pressure,and echocardiographic assessment of right atrial pressure (RAP) explained 68.8％ of the total variability in the model (r2=0.688,P ＜0.01).Conclusion Inaccuracy was frequently reported in Doppler echocardiographic assessment of the PAP in adult ASD patients with PAH and was often associated with age,gender,body surface area,ASDs' diameter,pulmonary vascular resistance,diastolic blood pressure and echocardiographic estimation of RAP.