Clinical Study Of Pulmonary Hypertension

Objectives:To get a good understanding of the clinical features and hemodynamics of patients with Eisenmenger syndrome in congenital heart diseases (CHD).Methods:Patients with Eisenmenger syndrome diagnosed by echocardiogram and right heart catheterization were enrolled from31clinical centers in China during the period from May,2007to December,2013. Age, gender, body mass index (BMI), symptoms and signs, World Health Organization functional class (WHO-FC), six-minute walk distance (6MWD) and hemodynamics were recorded. All the indexes were analyzed and compared.Results:A total of285CHD patients with Eisenmenger syndrome with3types of CHD were enrolled, including atrial septal defect (n=103,36.1%), ventricular septal defect (n=123,43.2%), patent ductus arteriosus (n=59,20.7%). Among them,85were males (29.8%),200were females (70.2%), the ratio of male to female is1:2.35.Mean age of all patients was (30.4±10.9) years, mean BMI was (19.7±3.2) kg/m2. The majority of patients were in WHO-FC Ⅰ±Ⅱ (n=193,67.7%) and only92in WHO-FC Ⅲ-Ⅳ (32.3%). The mean6MWD was (372±68) meters with only13.7%had a6WMD less than300meters. Electrocardiogram of60.7%of these patients indicated a hypertrophic right ventricle, and only6.0%of all patients showed syncope (n=17). Mean pulmonary arterial pressure (mPAP) was (73.8±20.3) mm Hg (1mm Hg=0.133kPa),the pulmonary vascular resistance (PVR)(1429±897) dy·s·cm-5, and the venous oxygen saturation was (67.5±8.0)%. Mean right atrial pressure (mRAP) was (8.8±5.4) mm Hg, with only8.1%patients had a mRAP greater than15mm Hg. Mean cardiac index was (3.6±2.0) L/min/m2, with only14.7%patients had a CI less than2.0L/min/m2.Conclusions:The majority of patients with Eisenmenger syndrome are young females, and ventricular septal defect is the most common underlying cause of Eisenmenger syndrome in CHD in China. The deterioration of heart function and impairment of exercise capacity in CHD patients with Eisenmenger syndrome is not parallel to the elevation of mPAP and PVR. Objectives:To investigate the plasma YKL-40levels in idiopathic pulmonary arterial hypertension (IPAH), PAH associated with congenital heart diseases (CHD-PAH), and PAH associated with connective tissue disease (CTD-PAH); and to investigate the association between YKL-40and clinical characteristics in these three types of PAH.Methods:Patients with IPAH, CHD-PAH and CTD-PAH conformed by right heart catheterization who were admitted to Fuwai hospital from2010October to2012October, and healthy volunteers were enrolled consecutively in this study. Peripheral venous blood samples of PAH patients and healthy volunteers were collected at baseline, and plasma YKL-40levels were determined. Patients’ data from right heart catheterization, echocardiography and laboratory tests were recorded. YKL-40levels were compared between different types of PAH, and correlations between YKL-40and clinical data were analyzed.Results:A total of86IPAH patients,15CHD-PAH patients,17CTD-PAH patients, and54healthy controls. Plasma YKL-40levels (median[interquartile range] were23.75(16.03-39.69) ng/mL in IPAH patients,22.21(20.14-38.13)ng/mL in CHD-PAH patients, and36.27(21.35-70.45) in CTD-PAH patients. After adjusting for age, plasma YKL-40levels were significantly elevated in all these three types of PAH patients compared to healthy controls (16.58[14.20-19.64] ng/mL, all P<0.05). CTD-PAH patients had higher plasma YKL-40than IPAH and CHD-PAH patients though showed no statistical significance.In IPAH patients, YKL-40correlated with age (r=0.409,P<0.001). In terms of PAH severity, YKL-40correlated with cardiac index (r=-0.252, P=0.019),cardiac output (r=-0.276, P=0.010), and NT-proBNP (r=0.248, P=0.021) in IPAH; In CHD-PAH, YKL-40correlated with WHO-functional class (r=0.548,P=0.035); In CTD-PAH, YKL-40correlated positively with mean right atrial pressure (r=0.610, P=0.009) and NT-proBNP (r=0.570, P=0.017).Conclusions:Plasma YKL-40correlated with the right heart function in IPAH and CTD-PAH, and correlated with functional class in CHD-PAH, presenting a potential biomarker in evaluating disease severity in PAH. Whether baseline YKL-40could be a prognostic biomarker for PAH requires follow-up studies. Background and Objectives:To explore a potential role of YKL-40, a marker of tissue remodeling and inflammation, in predicting prognosis in a prospective cohort of patients with idiopathic PAH (IPAH).Methods:Plasma YKL-40levels were measured in82IPAH patients without current or previous PAH-specific treatment during right heart catheterization and in54healthy volunteers. Concurrent data included clinical, hemodynamic, and biochemical variables.Results:Plasma YKL-40levels were increased in IPAH patients compared with control subjects (median, interquartile range:IPAH:24.90,17.68-39.78; controls:16.58,14.20-19.64ng/mL; P<0.001). YKL-40levels correlated with cardiac index (r=-0.244, P=0.027) and N-terminal pro-brain natriuretic peptide (NT-proBNP, r=0.263, P=0.017). After a median follow-up of578days, YKL-40outperformed NT-proBNP, uric acid, and6-minute-walk distance in receiver operating characteristic (ROC) analysis in predicting both clinical worsening (area under the curve (AUC),0.681; sensitivity82.6%, specificity61%) and death (AUC,0.717; sensitivity90.0%; specificity54.2%). Compared to patients with YKL-40below the ROC-derived cut-off point (24.5ng/mL), patients with YKL-40≥24.5ng/mL showed higher pulmonary vascular resistance and uric acid levels, and showed more clinical worsening events and deaths in Kaplan-Meier analysis. Plasma YKL-40was independently associated with clinical worsening in multivariate Cox analyses. After adjusting for potential confounders, YKL-40remained significantly associated with clinical worsening and death (all P<0.05).Conclusions:Plasma YKL-40might serve as a promising indicator of disease severity and prognosis in patients with IPAH.