| PART ⅠCharacteristics,goal-oriented treatments and survival of pulmonary arterial hypertension in China:Insights from a national multicentre prospective registry(Respirology.2022 Mar 15.doi:10.1111/resp.14247.)Background Diverse patterns and changing pictures of the diagnosis and treatment of pulmonary arterial hypertension(PAH)have been reported by numerous PAH registries,providing valuable data on various aspects of the disease.However,nationally representative reports on the characteristics and long-term survival of PAH from developing countries are scarce.The applicability of the current main risk stratifications and the longitudinal changes in goal-oriented treatments have yet to be elucidated in realworld settings.Objectives To provide insights into the characteristics,goal-oriented treatments and survival of PAH in China,and to explore the applicability of the current main risk stratification strategies in our independent PAH cohort.Methods In a national prospective multicentre registry of PAH in China,patients diagnosed with PAH by right heart catheterization were consecutively enrolled from August 2009 to December 2019.Follow-up was performed every 6 months±2 weeks for at least one year.Data on baseline,follow-up re-evaluation and therapeutic changes were collected.We employed the REVEAL 2.0 calculator,REVEAL Lite 2,and two abbreviated versions of the ESC/ERS risk assessment strategy[i.e.,the French invasive/noninvasive and the COMPERA methods]for analyses regarding risk stratification.Results A total of 2031 PAH patients from 34 tertiary hospitals in China were enrolled,with CHD-PAH(PAH associated with congenital heart disease)being the most common aetiology(45.2%).The mean age was 35±12 years,and 76.2%were females.At the time of enrolment,43.1%of the patients were in WHO Ⅲ or Ⅳ,with a mean 6-min walk distance(6MWD)of 412± 97 m.The mean value of mean pulmonary arterial pressure was 59.82 ± 18.28 mmHg,with 6.51± 4.79 mmHg for right atrial pressure,3.05±1.37 L·min-1·m-2 for cardiac index and 1092.88 ± 692.37 dyn·s·cm-5 for pulmonary vascular resistance.At baseline,79.8%of the cohort received at least one PAH-targeted drug,and 19.1%received combination therapy.The combination rate was similar between PAH subgroups(18.5%,19.8%and 18.4%in idiopathic PAH,CHD-PAH and PAH associated with connective tissue disease,respectively,P=0.848).Patients recruited after 2015 had a shorter diagnostic delay(median:7 versus 13 months,P<0.001)and better haemodynamics.Also,the rate of combination therapy increased significantly from 6.7%before 2015 to 35.5%thereafter;the most frequent combination changed from sildenafil plus beraprost to tadalafil plus ambrisentan.At follow-up,the risk stratum improved or was maintained for the maj ority of the patients(58.9%and 36.2%,respectively).However,40.8%of the patients failed to achieve a low-risk profile,and among patients who had combination therapy at baseline,4.2%of them still worsened.Higher percentages of intermediate-and high-risk patients than low-risk patients were prescribed prostacyclin analogues(18.9%and 20.5%versus 7.1%,P<0.001)or combination therapy(58.3%and 63.6%versus 41.6%,P<0.001)at follow-up.During a median follow-up time of 64.2 months(interquartile range:34.4,90.7;maximum:141.9 months),485 patients(23.9%)died.The survival estimates at 1,3,5 and 10 years were 95.6%(94.7-96.5%),87.6%(86.2-89.1%),79.2%(77.3-81.2%)and 63.2%(60.1-66.4%),respectively.The survival of the CHD-PAH group was significantly better than that of the other aetiology groups(P<0.001).The 1-year survival rate was significantly better in patients enrolled after 2015(P=0.03).Regarding risk assessment,when utilized in the same sufficient dataset at baseline(n=1026),the C-index ranged from 0.671 to 0.696 for 1-year risk prediction,and when applied in the same sufficient dataset at follow-up(n=420),the Cindex improved to a range of 0.824 to 0.835.Low-risk groups or patients with a greater number of low-risk criteria had significantly better survival than the others(P<0.001).In addition,the survival of patients whose risk stratum worsened at re-evaluation was worse than that of the others(P<0.05).Conclusion Chinese PAH patients show both similar and distinct features compared to other countries.There tended to be a shorter diagnostic delay,more standard adoption of targeted therapy and better survival after 2015.Utilized in the current cohort,the current main risk stratification strategies can significantly discriminate patients at different risk levels.At follow-up,approximately half of the re-evaluated patients did not achieve lowrisk profiles,indicating more aggressive treatment should be implemented to optimize the goal-oriented treatments.Taken together,to further improve the prognosis of PAH,continuous efforts are still warranted to improve the diagnosis and management of PAH,strengthen the health systems and introduce more sufficient treatments.PART ⅡIncorporation of noninvasive assessments in risk prediction for pulmonary arterial hypertensionBackground Risk assessment for pulmonary arterial hypertension(PAH)utilizing noninvasive prognostic variables could be more practical in real-world scenarios,especially at follow-up re-evaluations.Objectives To explore prognostic noninvasive variables in patients with PAH,and to incorporate those predictive variables in the established risk assessment tool to investigate their incremental prognostic value.Methods From a national prospective multicenter observational registry study of PAH in China,patients who underwent comprehensive noninvasive evaluations both at baseline and at follow-up visits were enrolled.The primary endpoint was all-cause mortality.Predictive variables identified by Cox analyses were further incorporated with the French noninvasive risk prediction approach.Results A total of 580 PAH patients were enrolled.During a median follow-up time of 47.0 months,112 patients(19.3%)died.By multivariate Cox analyses,tricuspid annular plane systolic excursion(TAPSE),TAPSE/pulmonary arterial systolic pressure(PASP),and peak oxygen consumption(VO2)remained independent predictors for survival.Three low-risk criteria(i.e.,peak VO2≥ 44%predicted,TAPSE≥ 17 mm and TAPSE/PASP>0.17 mm/mmHg)were newly derived.Regarding the French noninvasive risk prediction method,substituting the criterion for N-terminal pro b-type natriuretic peptide(NT-proBNP)with the newly derived low-risk criteria for TAPSE or TAPSE/PASP,or alternating six-minute walking distance>440 m with a peak VO2≥ 44%predicted retained the discrimination power.When recombining the low-risk criteria,the combination of World Health Organization functional class(WHO FC),TAPSE and peak VO2 at baseline,and the combination of WHO FC,NT-proBNP and peak VO2 at followup showed better discriminative ability than the other combinations[C-index:0.660(95%confidence interval:0.613-0.707)and 0.778(0.739-0.817),respectively].Conclusions Peak VO2,TAPSE and TAPSE/PASP are significant prognostic predictors for survival in PAH,with incremental prognostic value when incorporated with the French noninvasive risk prediction approach.For better risk prediction,WHO FC,at least one measurement of exercise capacity and one measurement of right ventricular function should be considered. |
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