Clinical Characteristics And Prognosis Analys On Adult Idiopathic Pulmonary Hypertension Patients

Objective:To investigate the clinical characteristics and prognostic factors of adult patients with idiopathic pulmonary arterial hypertension(IPAH).Methods: A total of 24 adult patients diagnosed as IPAH in the First Affiliated Hospital of Guangxi Medical University from October 2009 to February 2016 were retrospective analyzed.Clinical and hemodynamic data were analyzed and followed up to April 2017.The risk factors influencing prognosis in adult with IPAH were identified by COX survival analysis.Result: 1.The average of the patients was(35.33±13.93)years and the male to female ratio was 1:3.8.2.Almost all of patients had symptoms with exertional dyspnea,the incidence of syncope,chest pain,hemoptysis and hoarseness were33.3%,8.3%,20.8%,and 12.5%,respectively.3.An accentuated pulmonary component of the second heart sound was heard in all patients,and 50% patients had systolic murmur of tricuspid regurgitation.4.About70.8% patients were World Health Organization Functional Class(WHO-FC)III-IV group.Six-minute walking test distances(6MWD)was(396.91±53.14)m,and the range of Brog dyspnea score was 0-5(median 3).5.The average of the N-terminalpro-brain natriuretic peptide(NT-proBNP),red cell distribution width(RDW)and uric acid(UA)was(447.40±151.11)umol/L,(2548.40±1256.10)pg/ml and(15.50±2.30)%,respectively.UA and NT-proBNP was higher in WHO-FC III/IV group than WHO-FCI/II group.6.Electrocardiogram with right ventricular hypertrophy were common.Transthoracic echocardiography showed the significantly enlarge of right ventricular,and the increase of pulmonary arterial pressure was also significant.7.The mean pulmonary pressure and pulmonary vascular resistance by right catheterization were(58.6±12.9)mmHg and(16.1±6.5)WU.8.The average of follow-up times was(29.2±21.3)months,8patients were died,the survival rate of 1,2,3and 4 was 79.1%,70.8%,70.8%and 66.6%,respectively.These following variables could influence the survival of IPAH: 6MWD(P=0.025,RR=7.7*1015),NT-proBNP(P=0.011,RR=1.001)and RDW(P=0.011,RR=1.001)? UA(P=0.09,RR=1.002).In multivariate analysis,NT-proBNP(P=0.010,RR=1.002)and RDW(P=0.032,RR=10*1018)were the independent prognostic factor of IPAH.Conclusion:The IPAH is mostly found in young and middle-aged females.The most common symptom was exertional dyspnea,and some patients had syncope,chest pain,hemoptysis and hoarseness.An accentuated pulmonary component of the second heart sound was heard in all patients,and 50% patients had systolic murmur of tricuspid regurgitation.All patients had the enlargement of right ventricular,as well as the significantly increased of mPAP and PVR.Patients had poor prognosis and high fatality.NT-proBNP and RDW were independently predictive factors for adult IPAH patients.