| Objective:To analyze clinical phenotype and hemodynamics characteristics of patients with different subtypes of pulmonary arterial hypertension(PAH)and to improve diagnosis and treatment.To investigate the reliability of Two-Step Cluster method used for classification of patients with pulmonary arterial hypertension associated with congenital heart disease(CHD-PAH),and to compare the difference of clinical phenotype and hemodynamics among different groups of patients.Methods:From January 2005 to April 2017,556 patients with idiopathic pulmonary arterial hypertension,heritable pulmonary arterial hypertension,and associated pulmonary arterial hypertension(connective tissue disease,portal hypertension and congenital heart disease)were enrolled at Wuhan Asia Heart Hospital.Clinical phenotype and hemodynamics characteristics were compared among different subtypes groups of PAH.A total of 436 patients with pulmonary arterial hypertension associated with congenital heart disease were graded according to the pulmonary arterial pressure/systemic arterial pressure(Pp/Ps),and the patients were classified according to the Two-Step Cluster for mPAP and PVR,then we compared the clinical phenotypes of different grades and categories.Results:1.Among the overall 556 cases,there were 367(66.0%)females and the median age was 28.2 years.The number of patients in CHD-PAH group(448 cases,93.9%)was the overwhelming majority.2.Dyspnea/shortness of breath was the most common symptom in PAH patients.3.IPAH group had higher PVRI(F=7.504,P=0.001)、ALT(F=5.833,P=0.007),SUA(F=6.659,P=0.001)and NT-proBNP(F=4.199,P=0.027).Moreover,there was a high proportion of functional classⅢ/Ⅳ(44.4%)in IPAH group(?~2=23.099,P=0.001).4.CHD-PAH group(448 cases)had the significantly highest in mPAP(F=7.319,P=0.001)and Pp/Ps(F=8.089,P<0.001).Compared with other groups,NT-proBNP of CHD-PAH group was relatively lower(F=5.960,P=0.003).5.Clustering analyses for mPAP and PVR showed five specific clusters inCHD-PAH,lowest mPAP and lowest PVR(cluster 1),low mPAP and low PVR(cluster 2),moderately high mPAP and moderately high PVR(cluster 3),high mPAP and high PVR(cluster 4),highest mPAP and highest PVR(cluster 5),accounting for 7.8%,21.3%,41.3%,24.1%,5.5%of the total cases respectively.6.There were significant difference of mPAP(F=290.280,P<0.001),PVR(F=300.528,P<0.001),Pp/Ps(F=304.531,P<0.001),RDW(F=9.841,P=0.043),AST(F=9.663,P=0.047),SUA(F=4.998,P=0.001),WHO-FC(?~2=20.336,P<0.001),6MWD(F=3.543,P=0.009)and the rate of surgery(?~2=86.964,P<0.001)among five clusters.There were significant difference of mPAP(F=232.017,P<0.001),PVR(F=131.453,P<0.001),Pp/Ps(F=275.802,P<0.001),AST(F=7.058,P=0.029)the rate of surgery among three grades,and there were no difference of RDW(F=1.379,P=0.253),SUA(F=2.881,P=0.057),WHO-FC(?~2=50.968,P<0.001),6MWD(F=1.024,P=0.362)among three grades.Conclusions:1.Patients with pulmonary arterial hypertension in central China are younger and frequently in female than in previous descriptions.CHD-PAH is the most common type.2.The liver function and kidney function in IPAH patients was damaged and they were already at the severity condition when seeing to doctor firstly.3.In evaluating the prognosis of CHD-PAH patients,the right ventricular function and CI should be combined,rather than relying solely on mPAP.4.There is no difference between Pp/Ps and Two-Step Cluster method in hemodynamic indexes and the rate of surgery,biochemical indexes and heart function using two classification methods are slightly different,which implies that clustering analyses for mPAP and PVR for classification of CHD-PAH is reliable and can provide more clinical significance. |
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