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. Patients underwent right heart catheterization (RHC), 6-min walk test and CMR imaging within 1 week. Cardiopulmonary haemodynamics derived from RHC, and RV ejection/filling parameters derived from CMR were collected. RESULTS: A total of 41 IPAH patients were recruited with the median follow-up time of 824 days. During this period, 13 patients significantly deteriorated and among them, 7 patients died. CMR-derived RV peak ejection rate (PER) correlated significantly with CMR-derived RV stroke volume index (r = 0.574, P < 0.001) and RV cardiac index (r = 0.611, P < 0.001), but had no significant correlation with RV end diastolic volume index (EDVI, r = 0.201, P = 0.208) and pulmonary vascular resistance (r = -0.134, P = 0.403). Multivariate Cox regression analysis showed that RV time to PER (TPER, hazard ratio (HR) = 1.010, 95% CI: 1.003-1.017, P = 0.005) and RV TPER/time of a cardiac cycle (TPER/TCC, HR = 1.085, 95% CI: 1.031-1.141, P = 0.002) were predictors of clinical deterioration after adjusting for N-terminal pro-brain natriuretic peptide (NT-proBNP) and RV EDVI. Kaplan-Meier survival analyses showed that patients with RV TPER ≥ 199.01 ms (log rank: P = 0.036) or RV TPER/TCC ≥ 20.02% (log rank: P = 0.007) had worse prognosis. CONCLUSION: CMR-derived RV TPER and TPER/TCC can reflect RV early systolic function, and may be promising predictors for long-term prognosis in IPAH patients.

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. Correlations between electrocardiography variables and hemodynamics were assessed. Univariate and multivariable cox regression analysis were performed to identify ECG variables for predicting all-cause mortality in IPAH. RESULTS: Partial correlation analysis showed that P wave amplitude in lead II correlated with the mean pulmonary arterial pressure (mPAP, r = 0.349, p ≤ 0.001) and cardiac index (CI, r = -0.224, p = 0.002); R wave amplitude in V1 correlated with mPAP (r = 0.359, p ≤ 0.001); S wave amplitude in V6 correlated with mPAP (r = 0.259, p = 0.030) and CI (r = -0.220, p = 0.003). P wave amplitude in lead II (HR 1.555, p = 0.033) and R wave amplitude in lead aVR (HR 5.058, p < 0.001) were the independent predictors of all-cause mortality. Kaplan-Meier survival curves showed patients with a p ≥ 0.25 mv in lead II, and R ≥ 0.4 mv in lead aVR had lower 3-year survival (55 vs. 91%, p < 0.001). CONCLUSION: Specific lead-12 ECG features could reflect right ventricular overload hemodynamics, and are useful to evaluate prognosis of patients with IPAH.

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. All patients were followed up in a 6-month interval and the primary end point was all cause of death. The duration of mean follow-up was 67±26 months, of which there were twenty-three death. Cp had an inverse correlation with pulmonary artery resistance (PVR), regardless of the clinical phenotype (Eisenmenger syndrome, PAH with small defect and PAH after defect correction). Patients in the lowest Cp group (Cp ≤ 0.84 mL/mmHg) had advanced WHO function class, worse exercise tolerance, liver function, and status of oxygen saturation. In univariate cox regression analysis, Cp (HR = 1.359, P < 0.001), PVR (HR = 0.972, P = 0.001), pulmonary blood flow (HR = 1.092, P = 0.001), heart rate (HR = 1.038, P = 0.028) and 6-minute walking distance (HR = 1.003, P = 0.037) were predictors of survival. After adjustment by bivariate analysis, Cp was the independent predictor. Kaplan-Meier survival curves showed that patients with Cp < 1.04 mL/mmHg had worse prognosis. In conclusion, Cp possibly reflects disease severity and decreased Cp was associated with poor prognosis in patients with APAH-CHD.

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. Patients who benefit from long-term CCB were defined as those being in World Health Organization (WHO) functional class II or I after at least 1 year on CCB monotherapy. RESULTS: PVC had significant correlations with WHO function class, 6-min walk distance, mean pulmonary arterial pressure, and pulmonary vascular resistance (r = -0.363, p < 0.001; r = 0.333, p < 0.001; r = -0.514, p < 0.001; r = -0.739, p < 0.001). Thirty-five acute vasodilator-responsive IPAH patients (11.4 %) displayed less severe disease and a higher baseline PVC (1.5 ± 0.6 vs. 1.1 ± 0.7 ml/mmHg, p = 0.003). During acute vasodilator testing, PVC increased significantly by mean of 79 ± 48 % and reached to a higher absolute value of 2.6 ± 1.5 ml/mmHg compared with non-responsive patients (1.4 ± 1.5 ml/mmHg, p < 0.001). Furthermore, PVC increased more during acute vasodilator testing in the 24 patients who benefit from long-term CCB treatment (1.4 ± 1.3 vs. 0.5 ± 0.4 ml/mmHg, p = 0.004). The OR of increased PVC during vasodilator testing for predicting patients with long-term response to CCB was 1.24 (95 % CI 1.02-1.50, p = 0.031) as assessed by multivariable logistic regression analysis. CONCLUSIONS: PVC was higher in acute vasodilator-responsive IPAH patients and may be a predictor of long-term response to CCBs therapy.

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). METHODS: MPV and PDW levels were measured in 82 IPAH patients without antiplatelet or anticoagulant treatment on admission and 82 healthy controls. Concurrent collected data included clinical, haemodynamic and biochemical variables. All patients were followed-up from the date of blood testing. The endpoint was all-cause mortality. RESULTS: MPV and PDW were significantly higher in patients with IPAH than in age and sex-matched control subjects (11.4±0.9fl vs. 10.3±0.9fL and 14.3±2.9% vs. 11.9±1.9%, respectively; p=0.000). Pearson's correlation analysis revealed that MPV and PDW correlated positively with right ventricular systolic pressure, mean pulmonary arterial pressure and pulmonary vascular resistance. After a mean follow-up of 14±8 months, 12 patients died of right heart failure. Receiver operating characteristic analysis showed that MPV and PDW could not predict all-cause mortality. Multivariate Cox regression analysis suggested that right/left ventricular end-diastolic diameter ratio and NT-proBNP were independent predictive parameters of all-cause mortality. CONCLUSIONS: Our results suggest that MPV and PDW were elevated in patients with IPAH. They could partly reflect disease severity, but did not predict prognosis.

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. Follow-up data were collected periodically using a specifically designed network database until December 31, 2012. The end point was all-cause death. RESULTS: A total of 109 patients with ES were included in the study. Twenty-one patients (19.3%) died during a median follow-up period of 4.2 years (interquartile range 3.7-5.0 years). Baseline RDW was significantly correlated with mixed venous oxygen saturation (r=-0.286, p=0.003), arterial oxygen saturation (r=-0.423, p<0.001), mean pulmonary arterial pressure (r=0.271, p=0.004) and total pulmonary resistance (r=0.465, p<0.001). The 1-, 3- and 5-year survival rates for all 109 patients were 94%, 87% and 78%, respectively. Kaplan-Meier analysis showed that patients with RDW ≥13.9% had a lower survival rate than patients with RDW <13.9% (p=0.001). Multivariate Cox regression analysis showed that RDW was an independent prognostic marker in ES, with a hazard ratio of 1.162 (95% CI 1.036-1.302; p=0.010). CONCLUSIONS: Baseline RDW correlates with hemodynamics and is an independent prognostic marker in ES.

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). METHODS: Plasma YKL-40 levels were measured in 82 IPAH patients without current or previous PAH-specific treatment during right heart catheterization and in 54 healthy volunteers. Concurrent data included clinical, haemodynamic and biochemical variables. RESULTS: Plasma YKL-40 levels were increased in IPAH patients compared with control subjects (median, interquartile range: IPAH: 24.90, 17.68-39.78 ng/mL; controls: 16.58, 14.20-19.64 ng/mL; P < 0.001). YKL-40 levels 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 of 578 days, YKL-40 outperformed NT-proBNP, uric acid, and 6-min walk distance in receiver operating characteristic (ROC) analyses in predicting both clinical worsening (area under the curve (AUC) 0.681) and death (AUC 0.717). Compared with patients with YKL-40 below the ROC-derived cut-off point (24.5 ng/mL), the high YKL-40 group showed higher pulmonary vascular resistance and serum uric acid levels, and showed more clinical worsening events and deaths in Kaplan-Meier analyses. Plasma YKL-40 was independently associated with clinical worsening in univariate and multivariate Cox analyses (all P < 0.05). CONCLUSIONS: Plasma YKL-40 might serve as a promising indicator of disease severity and prognosis 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. METHODS AND RESULTS: We analysed 173 patients with IPAH. The patients were subclassified into categories of BMI defined as: under-weight (< 18.5 kg/m2), normal weight (18.5 to 24.9 kg/m2), overweight and obese (25 to 34.9 kg/m2). The three BMI groups had similar profiles in terms of haemodynamic parameters assessed by right heart catheterization and level of NT-proBNP. The overweight and obese group had higher age, and lower WHO functional class, larger left ventricular end-diastolic dimensions (LVEDDs) than the other two groups.The Kaplan-Meier survival curves for the three BMI categories demonstrated that the overweight and obese group had a significantly higher survival rate than the normal weight and underweight groups (log-rank test, P = 0.027, P = 0.000, respectively). In a stepwise forward regression, lower BMI, higher WHO functional class, lower cardiac index, smaller LVEDDs and absence of targeted medication remained independent predictors of mortality. CONCLUSIONS: Excess body mass is a protective factor for death in patients with IPAH.

[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. All the above indices were analyzed and compared. RESULTS: A total of 224 patients with Eisenmenger syndrome with 3 kinds of CHD were enrolled, including atrial septal defect (n = 67), ventricular septal defect (n = 104) and patent ductus arteriosus (n = 53). Among them, there were 67 males (29.9%) and 157 females (70.1%) with a mean age of (29.6 ± 9.9) years (range: 15-63). Mean BMI was (19.9 ± 4.0) kg/m(2) and mean 6MWD (371 ± 75) m. The majority of patients were in WHO-FC II (n = 158, 70.5%) and III (n = 64, 28.6%). Electrocardiogram of 77.2% of them indicated hypertrophic right ventricle. Mean right atrial pressure was (8.9 ± 5.7) mm Hg (1 mm Hg = 0.133 kPa), mean pulmonary arterial pressure (mPAP) (77.2 ± 19.1) mm Hg, cardiac index (3.03 ± 1.35) L·min(-1) · m(-2) and pulmonary vascular resistance (PVR) (1621 ± 887) dyn · s · cm(-5). CONCLUSIONS: The majority of patients with Eisenmenger syndrome with different types of CHD are young females and ventricular septal defect is the most frequent underlying cause. The deterioration of heart function in patients with Eisenmenger syndrome is non-parallel to mPAP and PVR in CHD.

[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. Correlations among peak oxygen consumption, anaerobic threshold, peak oxygen pulse, New York Heart Association (NYHA) class, N-terminal pro-brain natriuretic peptide (NT-proBNP), 6-minute walking distance (6 MWD) and cardiac index are analyzed in IPAH. RESULTS: There were 21 males and 55 females in IPAH and 8 males and 16 females in controls. Their mean ages were (31.5 ± 10.6) and (35.5 ± 6.4) years respectively. Significant differences (P = 0.000) existed between two groups in peak oxygen consumption ((12.7 ± 3.3) vs (25.6 ± 5.8) ml·min(-1)·kg(-1)), anaerobic threshold ((9.8 ± 2.5) vs (16.7 ± 3.9) ml·min(-1)·kg(-1)), peak oxygen pulse ((5.3 ± 1.6) vs (9.9 ± 2.5) ml/bpm) and ventilator efficiency (slope of minute ventilation in relation to CO2 produced) ((42.6 ± 2.0) vs (25.5 ± 3.5)). In IPAH, peak oxygen consumption was significantly correlated with NYHA class (r = -0.509, P = 0.000), 6 MWD (r = 0.443, P = 0.002) and NT-proBNP levels (r = -0.423, P = 0.011). And anaerobic threshold was significantly correlated with NYHA class (r = -0.362, P = 0.002), 6MWD (r = 0.343, P = 0.004) and NT-proBNP levels (r = -0.275, P = 0.017). Peak oxygen pulse and ventilator efficiency were both correlated well with total pulmonary vascular resistance. Partial correlation analysis demonstrated that there were significant correlations among peak oxygen consumption, anaerobic threshold, NYHA class, NT-proBNP levels and 6MWD after adjusting for age, gender and weight. CONCLUSIONS: Peak oxygen consumption and anaerobic threshold decrease ventilator efficiency in IPAH patients. Cardiopulmonary exercise testing is an invasive tool of assessing safely the function of IPAH patients.

[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. Their levels of N-terminal pro-brain natriuretic peptide (NT-proBNP) were measured. Incremental cardiopulmonary exercise testing was performed to compare its differential results among 3 groups and evaluate the correlation between NT-proBNP and its parameters. RESULTS: The body mass index (BMI) in the CTEPH group was lower than those in the CPE and control groups ((23.8 ± 3.9) vs (26.1 ± 3.6) and (26.7 ± 3.2) kg/m(2) ), both P < 0.05); the medical history in the CTEPH group was longer than those in the CPE and control groups ((58 ± 48) vs (12 ± 10) and (29 ± 25) months, both P < 0.05). The plasma concentrations of NT-proBNP in the CTEPH group were higher than those in the CPE and control groups ((1678 ± 1255) vs (577 ± 167) and (608 ± 247) pmol/L, both P < 0.05). All of them completed the test and there were no severe complications such as syncope or exacerbation of disease. Maximum oxygen consumption (VO2max), percentage of predicted maximum oxygen consumption (VO2 %), oxygen consumption in relation to body weight (VO2/kg), anaerobic threshold and O2 pulse in the CTEPH group were significantly lower than those in the CPE and control groups (P < 0.05). The ratios of dead space volume (VD) to tidal volume (VT) in the CTEPH and CPE groups were higher than those in the control group (P < 0.05). The plasma concentrations of NT-proBNP were inversely correlated with right ventricular internal diameter (r = -0.690, P = 0.000) and VO2/kg (r = -0.496, P = 0.000). The right ventricular internal diameter (ß = 0.583, P = 0.000) and VO2/kg (ß = 0.233, P = 0.032) were strong independent determinants of NT-proBNP. CONCLUSION: As a reliable pathophysiological indictor of CTEPH, cardiopulmonary exercise testing may be used objectively and safely to evaluate the cardiopulmonary function of CTEPH patients.

Atorvastatin in pulmonary arterial hypertension (APATH) study.

Statins have been shown to both prevent and attenuate pulmonary hypertension in animal models. This study investigates the potential therapeutic benefits of atorvastatin as an affordable treatment for pulmonary hypertension patients. 220 patients with pulmonary arterial hypertension (PAH) or chronic thromboembolic pulmonary hypertension (CTEPH) were randomised, double-blind, to receive atrovastatin 10 mg daily or matching placebo in addition to supportive care. At 6 months, 6-min walk distance decreased by 16.6 m in the atorvastatin group and 14.1 m in the placebo group. The mean placebo-corrected treatment effect was -2.5 m (95% CI: -38-33; p=0.96), based on intention to treat. A small nonsignificant increase in pulmonary vascular resistance and fall in cardiac output was seen in both treatment groups. There was no significant difference in the proportion of patients who improved, remained stable or showed a deterioration in World Health Organization functional class between atorvastatin and placebo treatments. Nine patients died in the atorvastatin group and 11 in the placebo group. Serum cholesterol levels fell significantly on atorvastatin treatment. Discontinuation rates were 23.2% and 26.9% on atorvastatin and placebo, respectively. Atorvastatin 10 mg daily has no beneficial effect on the natural history of PAH or CTEPH over 6 months.

[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. And the follow-up outcomes were analyzed with Kaplan-Meier plots. RESULTS: There were 27 males and 49 females with a mean age of 29.7 ± 9.7 years. They were classified into World Health Organization functional class (WHO-FC)II (n = 28), class III (n = 45) and class IV (n = 3). Their baseline mean pulmonary artery pressure was (65 ± 16) mm Hg, pulmonary vascular resistance (1677 ± 669) dyn×s(-1)×cm(-5), pulmonary capillary wedge pressure (9.6 ± 5.0) mm Hg, mean right atrial pressure (9.8 ± 6.1) mm Hg, cardiac index (2.07 ± 0.57) L ×min(-1)× m(-2) and serum uric acid (391 ± 103) µmol/L. The correlation analysis indicated that the serum level of uric acid correlated positively with right ventricular diameter (r = 0.28, P = 0.018) and negatively with CI (r = -0.34, P = 0.003). Independent t-test results indicated that the patients with a higher level of uric acid were apt to have a worse WHO-FC, and the higher level uric acid group (serum uric acid > 416.5 µmol/L) had a relative higher level of WHO-FC, NT-proBNP and endothelin-1. A lower level of CI denoted more severe conditions and prognosis. Survival analysis indicated that the serum level of uric acid could strongly predict survival in IPAH patients with over time and those with a high level of uric acid had a worse prognosis. CONCLUSION: The serum level of uric acid correlates significantly with patient conditions and prognosis in IPAH. And a higher serum level of uric acid predicts worse conditions and prognosis.

[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). The relevant data of demographic, clinical and hemodynamic features of all patients, analyzed the similarities and differences of demographic characteristics between different subtypes. They were divided into 2 groups: WHO functional class I/II and III/IV. And their hemodynamic and clinical features were compared. RESULTS: There were 165 males and 386 females with a mean age of (35 ± 12) years. The mean body mass index (BMI) was (21 ± 4) kg/m(2). There were pulmonary arterial hypertension (PAH, n = 487) and chronic thromboembolic pulmonary hypertension (CTEPH, n = 64). Fatigue (421, 76.4%) and dyspnea (398, 72.2%) were the most common symptoms; Physical examination revealed such a common sign as an accentuated pulmonic second sound (P(2)) in 510 patients (92.6%). Over half (325, 59.0%) of them were of WHO functional class II and 213 (38.6%) patients functional class III. The 6-minute walking distance (6MWD) and Borg dyspnea score were (352 ± 91) m and 3.0 (2.0 - 4.0) respectively. ECG of 497 (90.2%) patients showed right ventricular hypertrophy. Mean right atrial pressure was (9 ± 6) mm Hg (1 mm Hg = 0.133 kPa), pulmonary arterial pressure (67 ± 20) mm Hg, cardiac index (2.7 ± 1.2) Lmin(-1)m(-2) and pulmonary vascular resistance (1496 ± 783) dyn.sec.cm(-5). CONCLUSIONS: Young females with a low BMI are predominantly affected by PH. Severe functional and hemodynamic compromises often appear on presentation. And hemodynamic impairment is correlated with 6MWD and WHO functional class.

[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. RESULTS: The mean age of 150 patients were 36 ± 13 years with female patient/male patient ratio of 2:1, and mean BMI was (21.3 ± 3.5) kg/m(2). Fatigue (n = 123, 82.0%) and dyspnea (n = 112, 74.7%) are the most common symptoms. Accentuated pulmonic second sound (P(2)) was detected in 92.0% (n = 138) of patients during physical examination, which was also the most common sign. About 49.0% (n = 73) patients were WHO functional class I/II patients and 46.0% (n = 68) patients were WHO functional class III/IV patients. Six minutes walking distance (6MWD) and Borg dyspnea score was (337 ± 101) m and 2.0 (2.0, 4.0), respectively. Right ventricular hypertrophy was suggested by ECG in 93.1% (n = 140) patients. Right atrial pressure was (10 ± 6) mm Hg, mean pulmonary artery pressure was (61 ± 16) mm Hg, cardiac index was (2.3 ± 0.8) L×min(-1)×m(-2) and pulmonary vascular resistance (1484 ± 699) dyn×s(-1)×cm(-5) in this cohort. 6 MWD (305 m ± 89 m vs. 377 m ± 88 m) was significantly shorter while Borg dyspnea score [3.0 (3.0, 5.0) vs. 2.0 (2.0, 3.0)] was significantly higher in WHO functional class III/IV patients than in WHO functional class I/II patients. Similarly hemodynamic parameters were also worse in WHO functional class III/IV patients than in WHO functional class I/II patients (all P < 0.05). CONCLUSION: Idiopathic pulmonary arterial hypertension patients in this cohort affect mostly young adults, dominated by female gender and lower body mass index. Fatigue and dyspnea are the most common symptoms and accentuated pulmonic second sound (P(2)) is the most common sign. IPAH patients are often displaying severe functional and hemodynamic disturbance at first visit to hospitals. Dyspnea and hemodynamic impairment are related to 6MWD and WHO functional class.

[Preliminary study on the efficacy and safety of oral sildenafil in pulmonary arterial hypertension in China].

OBJECTIVE: Sildenafil has been shown to be effective in pulmonary arterial hypertension (PAH). However, the impact of sildenafil on PAH has been under-investigated in China. The aim of the present study was to evaluate the efficacy and safety of oral sildenafil in PAH patients in China. METHODS: In this prospective, open-label and multi-center study, 90 patients were recruited from 14 centers to receive oral sildenafil (75 mg/d) for 12 weeks. They underwent a six-minute walk test (SMWT) and cardiac catheterization at the beginning and the end of 12 weeks. The primary endpoint was the changes in exercise capacity as assessed by SMWT. And the secondary endpoints included assessment of functional class, evaluation of cardiopulmonary hemodynamics and clinical deterioration (defined as death, transplantation and re-hospitalization for PAH). Drug safety and tolerability were also examined. RESULTS: There were 19 males and 71 females with an average age of 32.5 ± 12.1 years old (range: 18 - 61). Their etiologies were idiopathic (n = 15), related with congenital heart disease (n = 60), or related with connective tissue disease (n = 9) and chronic thromboembolic pulmonary hypertension (n = 6). Oral sildenafil significantly increased the SMWT distances [(342 ± 93) m vs. (403 ± 88) m, P < 0.001]. There was also remarkable improvement in Borg dyspnea score (2.9 ± 2.6 vs. 2.4 ± 2.0, P = 0.005). Furthermore, significant improvements in World Healthy Organization (WHO) functional class and cardiopulmonary hemodynamics were also found (mean pulmonary artery pressure, P < 0.001; cardiac index, P < 0.001; pulmonary vascular resistance, P < 0.001). Side effects were mild and consistent with other reports. CONCLUSION: This study confirms and extends previous studies. Oral sildenafil is both safe and effective for the treatment of adult PAH patients in China.

[Sildenafil therapy for pulmonary hypertension: a prospective study].

OBJECTIVE: To explore the safety and efficacy of oral sildenafil therapy for pulmonary arterial hypertension (PAH), and to provide evidence for sildenafil treatment for Chinese patients with PAH. METHODS: In this 12-week, prospective, open-label, uncontrolled study, 56 patients with PAH were given oral sildenafil (25 mg, tid). The primary end point was change from baseline to 12 weeks in exercise capacity assessed by 6 min walk (6MW) test. Secondary end points included changes in WHO class and cardiopulmonary hemodynamics. Clinical worsening was defined as death, transplantation, hospitalization for PAH, or initiation of additional therapies for PAH, such as intravenous epoprostenol or oral bosentan. RESULTS: After 12 weeks, the compliance was good in 56 patients. Significant improvement was seen in NYHA heart function class and WHO class as compared to baseline (P < 0.01): from class IV to class III in 2, from class III to class II in 8 and to class I in 2 cases, and from class II to class I in 5 cases. No NYHA heart function class and WHO PAH function class deterioration were observed. Oral sildenafil increased 6MW distance, from (352 ± 80) m to (396 ± 78) m, with a change of (44 ± 70) m (P < 0.01). Significant improvement was seen in hemodynamics (mean pulmonary artery pressure, P < 0.01; cardiac index, P < 0.01; pulmonary vascular resistance, P < 0.01) at week 12 as compared with baseline. Mean right atrial pressure decreased (3 ± 11) mm Hg (1 mm Hg = 0.133 kPa), mean pulmonary arterial pressure decreased (6 ± 14) mm Hg, cardiac output increased (1.1 ± 2.0) L/min, cardiac index increased (0.7 ± 1.1) L×min(-1)×m(-2), and total pulmonary resistance decreased (490 ± 831) Dys×s×cm(-5). Side effects were mild and consistent with those reported with sildenafil treatment. No statistically significant clinical worsening was observed with sildenafil therapy for PAH patients. CONCLUSIONS: Sildenafil improves exercise capacity, WHO functional class, and hemodynamics in patients with pulmonary arterial hypertension.

[The prognosis study of 108 idiopathic pulmonary arterial hypertension patients].

OBJECTIVE: To analyze the characteristics of idiopathic pulmonary arterial hypertension (IPAH) and predict the prognosis of IPAH by Echocardiogram and Right Heart Catheterization. METHODS: 108 IPAH patients who was diagnosed as IPAH in Fuwai hospital from Jan 2003 to Jun 2007 were retrospectively analyzed and followed up to June 2008, the statistical analysis software was SPSS 13.0. RESULT: The mean age of the 108 IPAH patients was (32.5 +/- 12.6) years, the ratio of female and male was 2.86:1, the average time from appearing symptom to diagnosis was (34.9 +/- 36.3) months, the incidence of syncope, hepatomegaly, hemoptysis and Edema of lower extremity was 32.4%, 27.8%, 15.7% and 14.8% respectively. A total of 94 patients (87.0%) were followed up for (27.6 +/- 14.7) months, forty-two patients (38.9%) died of right heart failure, the mean death time was (11.6 +/- 9.4) months after diagnosis, the survival rate of 1, 2 and 3 years was 74%, 58% and 44% respectively. These following variables could predict survival in univariate analysis: Systolic Blood Pressure (SBP) (P < 0.01, RR = 0.937), pulse Pressure (PP) (P < 0.01, RR = 0.930), left ventricular End-Diastolic Dimension (LVEDD) (P = 0.004, RR = 0.924), right ventricular End-Diastolic Dimension (RVEDD) (P = 0.029, RR = 1.045), RVEDD/LVEDD (P = 0.003, RR = 3.222), Pulmonary Arterial Systolic Pressure (PASP) measured by echocardiogram (P = 0.002, RR = 1.022), PASP (P = 0.030, RR = 1.026), mean Pulmonary Arterial Pressure (mPAP) (P = 0.031, RR = 1.037) and Pulmonary Vascular Resistance (PVR) measured by right heart catheter (P = 0.019, RR = 1.001). in multivariate analysis, PP (P < 0.01, RR = 0.923), RVEDD/LVEDD (P = 0.006, RR = 3.126) and PASP measured by Echocardiogram (P = 0.002, RR = 1.022), PASP, mPAP, PVR measured by right heart catheter (P = 0.017, RR = 1.001) were predictive. CONCLUSION: IPAH was prone to attack young women with a poor prognosis, the diagnosis of IPAH at an early stage was difficult because of nonspecific symptom, syncope, hepatomegaly and Edema of lower extremity can predict the severity of IPAH, PVR measured by right heart catheterization, PP and PASP were independently predictive factors for IPAH.

[The relationship between plasma BNP level and the left ventricular dysfunction parameters in patients with acute myocardial infarction and it's value in diagonosing heart failure.].

OBJECTIVE: To explore the correlation between plasma BNP level and left ventricular dysfunction parameters in patients with acute myocardial infarction (AMI). METHODS: Plasma BNP level was determined in 230 consecutive inpatients with AMI and 111 normal controls. Patients were grouped according Killip grades, LVEF and LVEDd, respectively. BNP was transformed into lnBNP for the normal distribution. The receiver operator characteristic curve (ROC curve) was drawn to determine the best threshold and criteria for diagnosing heart failure. RESULTS: After AMI, lnBNP levels increased significantly in proportion with increasing Killip grades (I-III), and decreasing LVEF (all P < 0.05). lnBNP level was significantly higher in LVEDd > 55 mm group than in the LVEDd < 55 mm group (P < 0.01). lnBNP, LVEDd and LVEF all linearly correlated with Killip grades (P < 0.05) and the best correlation was shown between lnBNP and Killip grades (r = 0.53, P < 0.05). lnBNP also positively correlated with LVEDd (r = 0.17, P < 0.05) and negatively correlated with LVEF (r = -0.41, P < 0.01). Among the parameters, lnBNP level presented the largest AUC in their ROC curves (P < 0.01) for diagnosing decompensated heart failure and cardiogenic shock. The sensitivity, specifiticity and accuracy rates for diagnosing decompensated heart failure were 84.9%, 45.0% and 70.0% respectively by lnBNP at the cut point of 140 ng/L. The sensitivity, negative predicting value and accuracy rate for diagnosing cardiac shock were 82.8%, 66.7% and 67.4% respectively by BNP at the cut point of 400 ng/L. CONCLUSION: lnBNP level in hospitalized patients with AMI was positively correlated with Killip grades and LVEDd, negatively correlated with LVEF and could serve as a parameter for diagnosing the decompensated heart failure and excluding the cardiac shock.

[Value of radionuclide lung scintigraphy in the diagnosis and quantitative analysis of chronic thromboembolic pulmonary hypertension].

OBJECTIVE: This study was to assess the reliability of perfusion/ventilation (Q/V) lung scintigraphy in the diagnosis and quantitative analysis of chronic thromboembolic pulmonary hypertension (CTEPH). METHODS: A total of 78 in-patients with pulmonary hypertension who had no history of congenital heart disease, valvular disease and acute pulmonary embolism were included in this study. All patients underwent Q/V scintigraphy for detecting CTEPH. The sensitivity, specificity and accuracy of Q/V scintigraphy were defined by comparing with the results of pulmonary angiography. Percentage of Perfusion Defect score (PPDs%) was calculated in patients with CTEPH confirmed by pulmonary angiography. The correlations between PPDs% and mPAP, PPDs% and SPAP were analyzed. RESULTS: The sensitivity, specificity and accuracy of a high-probability Q/V scintigraphy interpretation were 96.0%, 81.1% and 86.9%, respectively, compared with 100%, 69.8% and 79.5% for the combination of high- and intermediate- probability Q/V scintigraphy interpretation. PPDs% was significantly correlated with mPAP and SPAP (r = 0.538 for mPAP, P < 0.01 and r = 0.456 for SPAP, P < 0.05). CONCLUSION: Perfusion/ventilation lung scintigraphy is a valuable technique for diagnosis and quantitative analysis of CTEPH.