Idiopathic pulmonary arterial hypertension(IPAH) is a rare disease of unknown etiology.The exact pathogenesis of pulmonary arterial hypertension is still not well known.In the past decades,many protein molecules have ...Idiopathic pulmonary arterial hypertension(IPAH) is a rare disease of unknown etiology.The exact pathogenesis of pulmonary arterial hypertension is still not well known.In the past decades,many protein molecules have been found to be in-volved in the development of IPAH.With proteomic techniques,profiling of human plasma proteome becomes more feasible in searching for disease-related markers.In present study,we showed the protein expression profiles of the serum of IPAH and healthy controls after depleting a few high-abundant proteins in serum.Thirteen spots had changed significantly in IPAH com-pared with healthy controls and were identified by LC-MS/MS.Alpha-1-antitrypsin and vitronectin were down-regulated in IPAH and may be valuable candidates for further explorations of their roles in the development of IPAH.展开更多
Objective Hypoxemia is one of the main factors of pulmonary hypertension,and it can also be a complication of pulmonary hypertension,and it is a risk factor for many cardiovascular diseases,which increases the adverse...Objective Hypoxemia is one of the main factors of pulmonary hypertension,and it can also be a complication of pulmonary hypertension,and it is a risk factor for many cardiovascular diseases,which increases the adverse prognosis of patients.At present,there are some controversies about the diagnosis and treatment of sleep apnea disorder in patients with pulmonary hypertension.展开更多
Idiopathic pulmonary arterial hypertension （IPAH） is a progressive, nearly fatal condition that until recently has had very few treatment options. Median survival time for untreated IPAH was 2.8 years without effect...Idiopathic pulmonary arterial hypertension （IPAH） is a progressive, nearly fatal condition that until recently has had very few treatment options. Median survival time for untreated IPAH was 2.8 years without effective drug intervention. IPAH is characterized by deregulated proliferation of pulmonary arterial endothelial and intimal smooth muscle cells resulting in progressive pulmonary vascular remodeling and an increase in pulmonary arterial pressure. In order to alleviate their symptoms, anticoagulants, diuretics, calcium channel blockers and inotropic agents have been used to treat patients with PAH. Moreover, specific targeted therapies using prostacyclins,展开更多
Objective: To evaluate the treatment and prognostic value of 12-lead electrocardiogram in idiopathic pulmonary hypertension (IPAH). Methods: A total of 200 patients with initial diagnosis of IPAH were included to anal...Objective: To evaluate the treatment and prognostic value of 12-lead electrocardiogram in idiopathic pulmonary hypertension (IPAH). Methods: A total of 200 patients with initial diagnosis of IPAH were included to analyze the correlation between ECG variables and hemodynamics.Univariate and multivariate proportional hazards regression analysis (Cox regression analysis) were used to analyze the changes of ECG parameters and determine the ECG parameters related to IPAH patients. Results: Partial correlation analysis showed that the amplitude of R wave in V1 lead was correlated with mPAP(r=0.343, P<0.001), and the sum of the amplitude of R wave in V1 lead and S wave in V5 lead was correlated with mPAP(r=0.504, P<0.001). The amplitude of R wave in V1 lead was correlated with mortality (HR 3.154, P<0.001), and the sum of the amplitude of R wave in V1 lead and S wave in V5 lead was correlated with mortality (HR 5.056, P<0.001). After 3 months of treatment,ECG parameters and hemodynamic status were improved. Conclusion: The specific 12-lead ECG parameters can reflect the improvement of the treatment of patients with idiopathic pulmonary hypertension and help to evaluate the prognosis of patients with idiopathic pulmonary hypertension.展开更多
Background Previous studies have shown that an echocardiographic right/left ventricular end-diastolic diameter ratio(RV/LV ratio)≥0.9 is an independent predictor of poor prognosis in patients with acute pulmonary emb...Background Previous studies have shown that an echocardiographic right/left ventricular end-diastolic diameter ratio(RV/LV ratio)≥0.9 is an independent predictor of poor prognosis in patients with acute pulmonary embolism. The prognostic value of the RV/LV ratio in patients with idiopathic pulmonary arterial hypertension(IPAH) is still unknown. Methods We retrospectively enrolled 95 consecutive patients with newly diagnosed IPAH and 16 of them were reevaluated by echocardiography at 3-12 months following targeted therapy.Follow-up data were obtained by telephone interviews and review of the patients’ records.Results The RV/LV ratio was in parallel with the severity of World Health Orgnization(WHO) functional class and mean right atrial pressure.The RV/LV ratio was positively correlated with total pulmonary resistance(P P P 2 saturation(P P = 0.001),weight and absence of targeted therapy were independent predictors of death.No significant changes in the RV/LV ratio before and after targeted therapy were observed. A baseline RV/LV ratio≥0.84 or a further increase in the RV/LV ratio during targeted therapy indicated a poor prognosis. Conclusions The RV/LV ratio helps to assess the severity of IPAH and serves as an independent predictor of prognosis in patients with IPAH.展开更多
Background： Pulmonary hypertension （PH） frequently complicates the course of idiopathic pulmonary fibrosis （IPF） patients and is associated with significantly worse outcomes. The aim of the present study was to i...Background： Pulmonary hypertension （PH） frequently complicates the course of idiopathic pulmonary fibrosis （IPF） patients and is associated with significantly worse outcomes. The aim of the present study was to investigate the incidence of PH in IPF patients and evaluate the correlation between clinical parameters and systolic pulmonary artery pressure （sPAP）. Methods： Hospitalized patients with IPF, who were evaluated for sPAP by Doppler echocardiography from January 2004 to December 2011, were enrolled in our study. Patients were defined as PH by an estimated sPAP 〉 50 mmHg and graded as PH likely, PH possible and PH unlikely, based on the 2009 European Society of Cardiology/European Respiratory Society PH Guidelines. The correlations between clinical parameters and sPAP were analyzed by multiple linear regression. Results： Totally, 119 IPF patients were enrolled in our study and 28 （23.5%）, 20 （16.8%） and 71 （59.7%） patients were PH likely, PH possible and PH unlikely, respectively. Borg dyspnea score was positively correlated with sPAP, r = 0.467, P 〈 0.001. Oxygen saturation was negatively correlated with sPAP, r = -0.416, P 〈 0.001. Diffusing capacity of the lung for carbon monoxide percentage predicted was negatively correlated with sPAP, r = -0.424, P = 0.003. N-terminal fragment of pro-brain natriuretic peptide and pulmonary artery width was positively correlated with sPAP, r = 0.452, P = 0.011 and r= 0.513, P 〈 0.001, respectively. Conclusions： The incidence of PH in IPF patients was 23.5% in a single center of China. PH may worsen the dyspnea, right heart dysfunction and decrease the life quality of the patients with IPF.展开更多
基金Project (No. A-007) supported by the Key Medicine Foundation of Zhejiang Province, China
文摘Idiopathic pulmonary arterial hypertension(IPAH) is a rare disease of unknown etiology.The exact pathogenesis of pulmonary arterial hypertension is still not well known.In the past decades,many protein molecules have been found to be in-volved in the development of IPAH.With proteomic techniques,profiling of human plasma proteome becomes more feasible in searching for disease-related markers.In present study,we showed the protein expression profiles of the serum of IPAH and healthy controls after depleting a few high-abundant proteins in serum.Thirteen spots had changed significantly in IPAH com-pared with healthy controls and were identified by LC-MS/MS.Alpha-1-antitrypsin and vitronectin were down-regulated in IPAH and may be valuable candidates for further explorations of their roles in the development of IPAH.
文摘Objective Hypoxemia is one of the main factors of pulmonary hypertension,and it can also be a complication of pulmonary hypertension,and it is a risk factor for many cardiovascular diseases,which increases the adverse prognosis of patients.At present,there are some controversies about the diagnosis and treatment of sleep apnea disorder in patients with pulmonary hypertension.
基金Acknowledgements This research program was supported by the National Natural Science Foundation of China （No. 81000018）, Special Financial Grant from the China Postdoctoral Science Foundation （No. 201104776） and the Major Program of the Chinese PLA General Hospital Funds. （No. 10KMZ04）.
文摘Idiopathic pulmonary arterial hypertension （IPAH） is a progressive, nearly fatal condition that until recently has had very few treatment options. Median survival time for untreated IPAH was 2.8 years without effective drug intervention. IPAH is characterized by deregulated proliferation of pulmonary arterial endothelial and intimal smooth muscle cells resulting in progressive pulmonary vascular remodeling and an increase in pulmonary arterial pressure. In order to alleviate their symptoms, anticoagulants, diuretics, calcium channel blockers and inotropic agents have been used to treat patients with PAH. Moreover, specific targeted therapies using prostacyclins,
基金This study was supported by Jiangsu Provincial Natural Science Foundation Project(BK20151355)Jiangsu Provincial Traditional Chinese Medicine Bureau Project(LB09041)Nanjing University of Chinese Medicine Project(NZYLCZX2015-01)
文摘Objective: To evaluate the treatment and prognostic value of 12-lead electrocardiogram in idiopathic pulmonary hypertension (IPAH). Methods: A total of 200 patients with initial diagnosis of IPAH were included to analyze the correlation between ECG variables and hemodynamics.Univariate and multivariate proportional hazards regression analysis (Cox regression analysis) were used to analyze the changes of ECG parameters and determine the ECG parameters related to IPAH patients. Results: Partial correlation analysis showed that the amplitude of R wave in V1 lead was correlated with mPAP(r=0.343, P<0.001), and the sum of the amplitude of R wave in V1 lead and S wave in V5 lead was correlated with mPAP(r=0.504, P<0.001). The amplitude of R wave in V1 lead was correlated with mortality (HR 3.154, P<0.001), and the sum of the amplitude of R wave in V1 lead and S wave in V5 lead was correlated with mortality (HR 5.056, P<0.001). After 3 months of treatment,ECG parameters and hemodynamic status were improved. Conclusion: The specific 12-lead ECG parameters can reflect the improvement of the treatment of patients with idiopathic pulmonary hypertension and help to evaluate the prognosis of patients with idiopathic pulmonary hypertension.
文摘Background Previous studies have shown that an echocardiographic right/left ventricular end-diastolic diameter ratio(RV/LV ratio)≥0.9 is an independent predictor of poor prognosis in patients with acute pulmonary embolism. The prognostic value of the RV/LV ratio in patients with idiopathic pulmonary arterial hypertension(IPAH) is still unknown. Methods We retrospectively enrolled 95 consecutive patients with newly diagnosed IPAH and 16 of them were reevaluated by echocardiography at 3-12 months following targeted therapy.Follow-up data were obtained by telephone interviews and review of the patients’ records.Results The RV/LV ratio was in parallel with the severity of World Health Orgnization(WHO) functional class and mean right atrial pressure.The RV/LV ratio was positively correlated with total pulmonary resistance(P P P 2 saturation(P P = 0.001),weight and absence of targeted therapy were independent predictors of death.No significant changes in the RV/LV ratio before and after targeted therapy were observed. A baseline RV/LV ratio≥0.84 or a further increase in the RV/LV ratio during targeted therapy indicated a poor prognosis. Conclusions The RV/LV ratio helps to assess the severity of IPAH and serves as an independent predictor of prognosis in patients with IPAH.
基金This work was supported by grants from the National Key Technology R＆D Program, the Ministry of Science and Technology of China （No. 2012BAI05B02） and the Key Program of Beijing Nature Science Foundation （No. 7131008）.
文摘Background： Pulmonary hypertension （PH） frequently complicates the course of idiopathic pulmonary fibrosis （IPF） patients and is associated with significantly worse outcomes. The aim of the present study was to investigate the incidence of PH in IPF patients and evaluate the correlation between clinical parameters and systolic pulmonary artery pressure （sPAP）. Methods： Hospitalized patients with IPF, who were evaluated for sPAP by Doppler echocardiography from January 2004 to December 2011, were enrolled in our study. Patients were defined as PH by an estimated sPAP 〉 50 mmHg and graded as PH likely, PH possible and PH unlikely, based on the 2009 European Society of Cardiology/European Respiratory Society PH Guidelines. The correlations between clinical parameters and sPAP were analyzed by multiple linear regression. Results： Totally, 119 IPF patients were enrolled in our study and 28 （23.5%）, 20 （16.8%） and 71 （59.7%） patients were PH likely, PH possible and PH unlikely, respectively. Borg dyspnea score was positively correlated with sPAP, r = 0.467, P 〈 0.001. Oxygen saturation was negatively correlated with sPAP, r = -0.416, P 〈 0.001. Diffusing capacity of the lung for carbon monoxide percentage predicted was negatively correlated with sPAP, r = -0.424, P = 0.003. N-terminal fragment of pro-brain natriuretic peptide and pulmonary artery width was positively correlated with sPAP, r = 0.452, P = 0.011 and r= 0.513, P 〈 0.001, respectively. Conclusions： The incidence of PH in IPF patients was 23.5% in a single center of China. PH may worsen the dyspnea, right heart dysfunction and decrease the life quality of the patients with IPF.
