Hemodynamics And Right Heart Function And Prognosis Of Pulmonary Hypertension

Part ?:Prognostic value of pulmonary artery compliance in patients with pulmonary arterial hypertension associated with adult congenital heart diseaseObjective:In congenital heart disease(CHD),the presence of pulmonary arterial hypertension(PAH)is associated with poor prognosis.In this study,we aim to investigate the role of pulmonary artery compliance(Cp)in predicting mortality of PAH associated with adult congenital heart disease(CHD-PAH).Methods:Patients who were diagnosed as CHD-PAH by right heart catheterization in Fuwai hospital between January in 2007 and December in 2012.All the patients underwent a comprehensive clinical evaluation including WHO function class,six minute walking distance,echocardiography and right heart catheterization.The results of laboratory test were also collected.Cp was calculated as stroke volume in milliliters divided by pulse pressure.All patients were followed up in a 6-month interval and the primary end point was all cause of death.Results:175 patients with CHD-PAH were included in this study.The duration of mean follow-up was 67 ±26 months,of which there were twenty-three death.Cp had an inverse correlation with pulmonary artery resistance(PVR),regardless of the clinical phenotype(Eisenmenger syndrome,PAH with small defect and PAH after defect correction).Patients in the lowest Cp group(Cp?0.84ml/mmHg)had advanced WHO function class,worse exercise tolerance,liver function and status of oxygen saturation.In univariate cox regression analysis,heart rate(HR=1.038,p=0.028),Cp(HR=1.359,p<0.001),PVR(HR=0.972,p=0.001),pulmonary blood flow(HR=1.092,p=0.001),and 6-minute walking distance(HR=1.003,p=0.037)were predictors of survival.After adjustment by bivariate analysis,Cp was the independent predictor.Kaplan-Meier survival curves showed that patients with Cp<1.04ml/mmHg had worse prognosis.Conclusion:Cp possibly reflects disease severity and decreased Cp was associated with poor prognosis in patients with CHD-PAH.Part ?:Pulmonary vascular compliance is associated with vasoreactivity and long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertensionObjective:This study aimed to identify the value of pulmonary vascular compliance(Cp)in predicting long-term response to calcium channel blochers(CCB)treatment in patients with idiopathic pulmonary arterial hypertension(IPAH).Methods:This study included IP AH patients who were newly diagnosed in the pulmonary vascular disease center of Fuwai hospital between January in 2009 and December in 2014.Acute pulmonary vasoreactivity testing was performed with inhaling iloprost.Cp before and after inhaling iloprost was evaluated.Cp was calculated as stroke volume in milliliters divided by pulse pressure.Acute vasodilator responsive patients accepted CCB treatment and the patients were followed up for at least one year.Results:Cp had significant correlations with WHO function class,6-minute walk distance,mean pulmonary arterial pressure and pulmonary vascular resistance(r=-0.363,p<0.001;r=0.333,p<0.001;r=-0.514,p<0.001;r=-0.739,p<0.001).35 acute vasodilator responsive IP AH patients(11.4%)displayed less severe disease and a higher baseline Cp(1.5 ± 0.6 ml/mmHg vs.1.1 ± 0.7 ml/mmHg,p = 0.003).After inhaling iloprost,Cp increased significantly by mean of 79%±48%and compared with non-responsive patients with 19%±47%(p<0.001).Furthermore,Cp increased more during acute vasodilator testing in the 24 patients who benefit from long-term CCB treatment(93%±72%vs.48%±49%,p=0.039).Increased Cp during vasodilator testing was the independent predicting factor of long-term response to CCB in patients with IP AH as assessed by multivariable logistic regression analysis.Conclusion:Cp was higher in acute vasodilator responsive IP AH patients and may be a predictor of long-term response to CCB therapy(OR=1.24,p=0.031).Part ?:Impact of right ventricular dyssynchrony on right heart function and prognosis on patients with pulmonary hypertensionObjective:Right ventricular intraventricular dyssynchrony(RVD)had been described in patients with pulmonary hypertension.This study aimed to investigate the impact of RVD on right ventricular function and prognosis on patients with pulmonary hypertension(PH).Methods:One hundred and fifty PH patients were included in this study.All the patients underwent comprehensive clinical evaluation,including WHO function class,six minutes walking distance and right heart catheterization.RVD was evaluated by speckle-tracking echocardiography.The time to peak strain(Tpeak)of right ventricular segments were analyzed by the software of Qlab.RVD was quantified by the standard deviation of the heart rate-corrected Tpeak of right ventricular four segments(RV-SD4).RVD is defined as RV-SD4>18ms.Right ventricular end-systolic volume(RVESV)and right ventricular end-diastolic volume(RVEDV)were detected by three-dimensional echocardiology,and right ventricular ejection fraction(RVEF)was calculated.All patients were followed up in a 6-month interval and the primary end point was all cause of death.Results:RV-SD4 had positive correlations with RVESV,RVEDV and pulmonary vascular resistance(r=0.566,p<0.001;r=0.532,p<0.001;r=0.372,p<0.001);but negatively correlated with tricuspid annular plane systolic excursion(TAPSE),RVEF,right ventricular fractional area change(RVFAC)and cardiac index(CI)(r=-0.394,p<0.001;r=-0.336,p<0.001;r=-0.427,p<0.001;r=-0.420,p<0.001).85 patients(56.7%)presented with RVD(RV-SD4>18ms)had delayed contraction of the basal right ventricular free wall and basal interventricular septum.Patients with RVD had advanced WHO function class,and worse RV dilation and hemodynamics.According to the multivariate logistic regression analysis,RVESV,RVFAC and CI were factors associated with RVD.After followed up for 42116 months,there were 29 deaths.ROC curve found the cut-off value of RV-SD4 for predicting prognosis was 38ms.Multivariate Cox regression analysis showed that RV-SD4>38ms was the independent prognosis factor in PH patients with HR=2.962,p=0.048.Conclusion:RVD is associated with reduced right heart function and RVD is an independent factor of poor prognosis in patients with pulmonary hypertension.