The clinical characteristics and long-term prognosis of pulmonary arterial hypertension associated with hereditary hemorrhagic telangiectasia.

Pulmonary arterial hypertension (PAH) is a severe complication of hereditary hemorrhagic telangiectasia (HHT); however, little is known about its clinical characteristics and prognosis. Nine newly diagnosed HHT-PAH patients were prospectively recruited between October 2007 and January 2016 and were followed up every half-year. Eighteen idiopathic pulmonary arterial hypertension (IPAH) patients, matched with HHT-PAH patients on mean pulmonary arterial pressure, pulmonary capillary wedge pressure, pulmonary vascular resistance, cardiac index, and World Health Organization (WHO) functional class (FC), were recruited.

Prognostic Value of Pulmonary Artery Compliance in Patients with Pulmonary Arterial Hypertension Associated with Adult Congenital Heart Disease.

In congenital heart disease (CHD), the presence of pulmonary arterial hypertension (PAH) is associated with a poor prognosis. In this study, we aim to investigate the role of pulmonary artery compliance (Cp) in predicting the mortality of PAH associated with adult congenital heart disease (APAH-CHD). One-hundred and seventy-five patients of APAH-CHD who underwent a comprehensive clinical evaluation were included in this study.

The Value of the Electrocardiogram for Evaluating Prognosis in Patients with Idiopathic Pulmonary Arterial Hypertension.

Background: Association between electrocardiography (ECG) features and right ventricular anatomy and physiology has been established. This study is aimed to identify the value of 12-lead ECG in evaluating prognosis of patients with idiopathic pulmonary arterial hypertension (IPAH).

Method: 194 patients with newly diagnosed IPAH were included in this study.

Prognostic value of right ventricular ejection/filling parameters in IPAH using cardiac magnetic resonance: A prospective pilot study.

Background And Objective: The potential prognostic value of cardiovascular magnetic resonance (CMR)-derived right ventricular (RV) ejection/filling parameters in the assessment of RV function remains to be fully established. The goal of this study was to explore the prognostic value of these parameters in idiopathic pulmonary arterial hypertension (IPAH) patients.

Methods: In this prospective investigation, newly diagnosed IPAH patients without targeted therapy were recruited.

Pulmonary Vascular Capacitance is Associated with Vasoreactivity and Long-Term Response to Calcium Channel Blockers in Idiopathic Pulmonary Arterial Hypertension.

Purpose: This study aimed to identify the relationship between pulmonary vascular capacitance (PVC) and vasoreactivity in patients with idiopathic pulmonary arterial hypertension (IPAH), and the value of PVC in predicting long-term response to CCB treatment.

Methods: Pulmonary vasodilator testing with inhaling iloprost was performed in 308 newly diagnosed IPAH patients. Acute vasodilator-responsive patients accepted CCBs treatment.

The Prognostic Value of 18F-FDG Uptake Ratio Between the Right and Left Ventricles in Idiopathic Pulmonary Arterial Hypertension.

Purpose: Metabolic changes occur in the right ventricle (RV) under increased afterload in pulmonary arterial hypertension. FDG PET imaging has potential to assess RV function. In this study, we aimed to determine the prognostic value of metabolic changes of RV using FDG PET imaging in idiopathic pulmonary arterial hypertension (IPAH).

High levels of serum lactate dehydrogenase correlate with the severity and mortality of idiopathic pulmonary arterial hypertension.

Liver dysfunction reflects the status of heart failure, and previous studies have demonstrated that serum lactate dehydrogenase (S-LDH) levels are increased in patients exhibiting heart failure and liver dysfunction. Right heart failure is a main characteristic of idiopathic pulmonary arterial hypertension (IPAH). The aim of the present study was to assess the prognostic significance of S-LDH levels in patients with IPAH.

Survival advantages of excess body mass index in patients with idiopathic pulmonary arterial hypertension.

Objective: An obesity paradox, a "paradoxical" decrease in morbidity and mortality with increasing body mass index (BMI), has been shown in patients with heart failure. However, the impact of BMI in patients with idiopathic pulmonary arterial hypertension (IPAH) has not been studied. This study aims to find out whether BMI is a prognostic factor in IPAH.

Platelet distribution width and mean platelet volume in idiopathic pulmonary arterial hypertension.

Background: Previous studies have demonstrated that platelet activation occurs in patients with pulmonary arterial hypertension (PAH). Mean platelet volume (MPV) and platelet distribution width (PDW) are two markers of platelet activation, and have recently been recognised as risk predictors of cardiovascular diseases. This study aimed to investigate whether MPV and PDW would be useful to reflect disease severity and predict prognosis in idiopathic PAH (IPAH).

Plasma soluble ST2 levels correlate with disease severity and predict clinical worsening in patients with pulmonary arterial hypertension.

Background: Soluble suppression of tumorigenicity (sST2) has been proposed to be a marker for biomechanical strain and a possible predictor of mortality in patients with chronic heart failure. The use of sST2 in pulmonary arterial hypertension (PAH) has not been well defined.

Hypothesis: Plasma sST2 levels may correlate with the disease severity and predict clinical worsening in PAH.

Increased levels of plasma CXC-Chemokine Ligand 10, 12 and 16 are associated with right ventricular function in patients with idiopathic pulmonary arterial hypertension.

Objective: To investigate plasma levels of CXC-Chemokine Ligand 10 (CXCL10), CXC-Chemokine Ligand 12 (CXCL12) and CXC-Chemokine Ligand 16 (CXCL16) in patients with idiopathic pulmonary arterial hypertension (IPAH).

Methods: Plasma levels of biomarkers were measured by enzyme-linked immunosorbent assay in 61 patients with IPAH and 20 healthy volunteers.

Results: Plasma CXCL10, CXCL12 and CXCL16 concentrations were increased significantly in IPAH patients compared with controls, and significantly correlated with N-terminal pro-brain natriuretic peptide, tricuspid annulus plane systolic excursion and right ventricular ejection fraction.

Elevated plasma YKL-40 as a prognostic indicator in patients with idiopathic pulmonary arterial hypertension.

Background And Objective: Pulmonary vascular remodelling and inflammation have been implicated in pulmonary arterial hypertension (PAH). YKL-40, a marker of tissue remodelling and inflammation, has recently been recognized as a risk predictor of cardiovascular and inflammatory diseases. The study aimed to investigate a potential role of YKL-40 in predicting prognosis in idiopathic PAH (IPAH).

The ratio of (18)F-FDG activity uptake between the right and left ventricle in patients with pulmonary hypertension correlates with the right ventricular function.

Purpose: It is known that patients with pulmonary hypertension (PH) can have elevated F-FDG uptake in the right ventricle (RV) on PET imaging. This study was designed to assess possible relationship between FDG uptake of ventricles and the function/hemodynamics of the RV in patients with PH.

Patients And Methods: Thirty-eight patients with PH underwent FDG PET imaging in both fasting and glucose-loading conditions.

Red blood cell distribution width predicts survival in patients with Eisenmenger syndrome.

Background: Previous studies identified an independent relationship between red blood cell distribution width (RDW) and prognosis in patients with pulmonary hypertension of mixed etiologies and idiopathic pulmonary arterial hypertension. This study aimed to investigate the significance of RDW for predicting survival in patients with Eisenmenger syndrome (ES).

Methods: We retrospectively reviewed the clinical records and collected baseline data for patients newly diagnosed with ES in our hospital between January 2005 and October 2009.

[Application of cardiopulmonary exercise testing in patients with chronic thromboembolic pulmonary hypertension].

Objective: To evaluate the application value of cardiopulmonary exercise testing in patients with chronic thromboembolic pulmonary hypertension (CTEPH).

Methods: A total of 116 consecutive patients admitted into the Cardiology Department of Fuwai Hospital.They were divided into 3 groups of CTEPH (n = 44), CPE (without pulmonary hypertension in chronic pulmonary embolism) (n = 24) and control (without pulmonary embolism or pulmonary hypertension) (n = 48) respectively.

[Clinical observation of cardiopulmonary exercise test in patients with idiopathic pulmonary arterial hypertension].

Objective: To explore the exercise characteristics of patients with idiopathic pulmonary arterial hypertension (IPAH).

Methods: From November 2010 to September 2012 , 76 consecutive IPAH patients and 24 healthy controls from Fuwai Cardiovascular Hospital were enrolled to undergo cardiopulmonary exercise testing. The exercise parameters were compared.

[Clinical features of adult patients with Eisenmenger syndrome associated with different types of congenital heart disease].

Objective: To explore the clinical features and hemodynamics of adult patients with Eisenmenger syndrome in different types of congenital heart diseases (CHD).

Methods: Patients with Eisenmenger syndrome with different types of CHD diagnosed by right heart catheterization were enrolled from 31 clinical centers in China during the period from May 2007 to October 2010. Age, gender, body mass index (BMI), symptoms and signs, World Health Organization functional class (WHO-FC) of pulmonary hypertension, six-minute walk distance (6MWD) and hemodynamics were recorded.

Echocardiographic parameters in patients with pulmonary arterial hypertension: correlations with right ventricular ejection fraction derived from cardiac magnetic resonance and hemodynamics.

Background: Echocardiography is the most convenient method used to evaluate right ventricular function, and several echocardiographic parameters were studied in previous studies. But the value of these parameters to assess the right ventricular function in patients with pulmonary arterial hypertension (PAH) has not been well defined.

Methods: Patients with PAH were observed prospectively.

Impact of sildenafil on survival of patients with Eisenmenger syndrome.

The favorable effects of short-term use of sildenafil on patients with Eisenmenger syndrome have been reported. We further studied the impact of long-term use of sildenafil on survival of these patients. In this study, the baseline data of patients newly diagnosed as Eisenmenger syndrome in our hospital between January 2005 and December 2009 were retrospectively collected.

[Relationship between serum uric acid levels and patient conditions and prognosis in idiopathic pulmonary arterial hypertension].

Objective: To explore the relationship between serum uric acid levels and patient conditions and prognosis in idiopathic pulmonary arterial hypertension (IPAH).

Methods: A total of 76 IPAH patients confirmed by right heart catheterization were enrolled consecutively and followed up until the endpoint of all-cause death. Their baseline data were recorded and analyzed by Spearman's rank test and independent t-test.

[Clinical analysis of 150 patients with idiopathic pulmonary arterial hypertension].

Objective: To explore the demographic characteristics and clinical features of patients with idiopathic pulmonary arterial hypertension (IPAH) in China.

Methods: Between March 2007 and September 2010, IPAH diagnosis was confirmed by right heart catheterization in 150 adult patients from 31 clinical centers in China. Clinical and hemodynamic data were analyzed and patients were divided into WHO functional class I/II and WHO functional class III/IV group.

Comparison of 18F-FDG uptake by right ventricular myocardium in idiopathic pulmonary arterial hypertension and pulmonary arterial hypertension associated with congenital heart disease.

This study measured glucose uptake in the right ventricle (RV) of patients with pulmonary hypertension and investigated the relationship to hemodynamics and survival. Myocardial 18F-fluorodeoxy-glucose (FDG) uptake was measured using single-photon positron emission tomography (SPECT) in 24 patients with idiopathic pulmonary arterial hypertension (IPAH) and 43 patients with congenital heart disease (CHD). In both IPAH and CHD-PAH, RV FDG uptake (RV/LV ratio) was associated with pulmonary vascular resistance (PVR).

Acute responses to inhalation of Iloprost in patients with pulmonary hypertension.

Background: Iloprost has been used to test acute pulmonary vasoreactivity in idiopathic pulmonary arterial hypertension (PAH). We aimed to investigate the acute hemodynamic and oxygenation responses and tolerability to 20 µg aerosolized Iloprost in Chinese patients with pulmonary hypertension.

Methods: Between March 2005 and May 2010, 212 pulmonary hypertension patients inhaled a single dose of 20 µg Iloprost over 10 - 15 minutes for vasoreactivity testing.

[Multicenter study of disease attributes in adult patients with pulmonary hypertension].

Objective: To understand the demographic, hemodynamic and clinical features of adult patients with pulmonary hypertension (PH) in China.

Methods: Between May 2007 and October 2010, a total of 551 adult PH patients were recruited from 31 clinical centers all over China. All fulfilled the traditional hemodynamic criteria diagnosed through right heart catheterization (RHC).