OBJECTIVETo investigate the current practice of pulmonary hypertension including current epidemiology, diagnosis and treatment.

DATA SOURCESThe review was based on data obtained from the published articles and guidelines.

STUDY SELECTIONArticles with high level of evidence or current best evidence in each issue were selected to be reviewed.

RESULTSOverall prevalence of pulmonary hypertension was 0.3% to 6% with left heart disease occupying the most proportion, followed by pulmonary disease, pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension. In diagnosis, a flow diagram of diagnosis of pulmonary hypertension, differential diagnosis of pulmonary hypertension and how to determine the severity of pulmonary hypertension are explained including recent development of magnetic resonance imaging and gene abnormality study on bone morphogenetic protein receptor II. In treatment, newly-developed pulmonary vasodilators and the way to use them are shown to treat pulmonary hypertension.

CONCLUSIONSafer and more effective treatment algorithm and basic researches and clinical trials are warranted to be explored.

Humans ; Hypertension, Pulmonary ; diagnosis ; drug therapy ; epidemiology

Epidemiologic patterns of pulmonary arterial hypertension differ by era and region and may shed light on the pathophysiology and treatment of the disease. New efforts to target one or more of the recently studied therapies could establish personalized medicine as standard care in pulmonary arterial hypertension.
Humans ; Hypertension, Pulmonary ; epidemiology ; physiopathology ; therapy ; Risk Factors

Child ; Humans ; Hypertension, Pulmonary - epidemiology ; Hypertension, Pulmonary - etiology ; Papua New Guinea - epidemiology ; Urban Population - statistics & ; numerical data

Objective: To analyze the classification, diagnosis and treatment status of patients with pulmonary hypertension (PH) in Yunnan province. Methods: This was a retrospective study. Hospitalized patients with PH at Yan'an Affiliated Hospital of Kunming Medical University from January 2012 to December 2019 were enrolled. The clinical data of enrolled patients, including demographic data, comorbidities, targeted drug therapy, echocardiography and right heart catheterization results, were obtained through the electronic medical record system. The composition ratio of PH, diagnosis and treatment were analyzed. Results: A total of 13 590 patients with PH were enrolled, accounting for 3.09% (13 590/440 056) of the total number of hospitalizations during the same period. The composition of PH was predominantly pulmonary arterial hypertension (PAH) (55.50% (7 542/13 590)), followed by pulmonary hypertension (PH) caused by left heart disease (24.16% (3 284/13 590)). Among them, PAH could be subdivided into four types: idiopathic pulmonary arterial hypertension (IPAH), PAH associated with connective tissue disease, PAH associated with portal hypertension, and PAH associated with congenital heart disease (CHD-PAH), with CHD-PAH as the predominating type (98.09% (7 398/7 542). Patients with PAH were predominantly adolescents. In hospitalized patients with PH, from 2012 to 2019, the proportion of children and adolescents showed a decreasing trend from year to year, and the proportion of middle-aged and older adults showed a significant increasing trend, and the proportion of female patients showed a gradual decreasing trend, and the proportion of patients with comorbid hypertension, diabetes mellitus, coronary artery disease, arrhythmia, and pneumonia showed an increasing trend. A total of 1 034 patients (7.61% (1 034/13 590)) underwent right heart catheterization. The concordance rate between echocardiographic and right heart catheterization findings was (86.98% (875/1 006)). A total of 2 574 (18.94%) of PH patients were treated with PAH targeted drugs, of which 58.16% (1 497/2 574) were treated with monotherapy. Among the PH patients treated with PAH targeted drugs, the majority of patients were PAH patients (86.44% (2 225/2 574)), and 83.53% (2 150/2 574) patients treated with PAH targeted drugs were CHD-PAH. Conclusions: Hospitalized PH patients in our center between 2012 and 2019 are predominantly CHD-PAH, and the proportion of patients receiving right heart catheterization and targeted drug therapy is relatively low. The percentage of middle-aged and elderly PH patients shows an increasing trend from year to year, as well as the percentage of those with concomitant comorbidities.
Child ; Aged ; Adolescent ; Middle Aged ; Humans ; Female ; Hypertension, Pulmonary/therapy* ; Retrospective Studies ; China/epidemiology* ; Familial Primary Pulmonary Hypertension ; Pulmonary Arterial Hypertension/complications* ; Heart Defects, Congenital

Objective: To analyze the classification, diagnosis and treatment status of patients with pulmonary hypertension (PH) in Yunnan province. Methods: This was a retrospective study. Hospitalized patients with PH at Yan'an Affiliated Hospital of Kunming Medical University from January 2012 to December 2019 were enrolled. The clinical data of enrolled patients, including demographic data, comorbidities, targeted drug therapy, echocardiography and right heart catheterization results, were obtained through the electronic medical record system. The composition ratio of PH, diagnosis and treatment were analyzed. Results: A total of 13 590 patients with PH were enrolled, accounting for 3.09% (13 590/440 056) of the total number of hospitalizations during the same period. The composition of PH was predominantly pulmonary arterial hypertension (PAH) (55.50% (7 542/13 590)), followed by pulmonary hypertension (PH) caused by left heart disease (24.16% (3 284/13 590)). Among them, PAH could be subdivided into four types: idiopathic pulmonary arterial hypertension (IPAH), PAH associated with connective tissue disease, PAH associated with portal hypertension, and PAH associated with congenital heart disease (CHD-PAH), with CHD-PAH as the predominating type (98.09% (7 398/7 542). Patients with PAH were predominantly adolescents. In hospitalized patients with PH, from 2012 to 2019, the proportion of children and adolescents showed a decreasing trend from year to year, and the proportion of middle-aged and older adults showed a significant increasing trend, and the proportion of female patients showed a gradual decreasing trend, and the proportion of patients with comorbid hypertension, diabetes mellitus, coronary artery disease, arrhythmia, and pneumonia showed an increasing trend. A total of 1 034 patients (7.61% (1 034/13 590)) underwent right heart catheterization. The concordance rate between echocardiographic and right heart catheterization findings was (86.98% (875/1 006)). A total of 2 574 (18.94%) of PH patients were treated with PAH targeted drugs, of which 58.16% (1 497/2 574) were treated with monotherapy. Among the PH patients treated with PAH targeted drugs, the majority of patients were PAH patients (86.44% (2 225/2 574)), and 83.53% (2 150/2 574) patients treated with PAH targeted drugs were CHD-PAH. Conclusions: Hospitalized PH patients in our center between 2012 and 2019 are predominantly CHD-PAH, and the proportion of patients receiving right heart catheterization and targeted drug therapy is relatively low. The percentage of middle-aged and elderly PH patients shows an increasing trend from year to year, as well as the percentage of those with concomitant comorbidities.
Child ; Aged ; Adolescent ; Middle Aged ; Humans ; Female ; Hypertension, Pulmonary/therapy* ; Retrospective Studies ; China/epidemiology* ; Familial Primary Pulmonary Hypertension ; Pulmonary Arterial Hypertension/complications* ; Heart Defects, Congenital

OBJECTIVETo compare the clinical characteristics and survival on Chinese patients with idiopathic pulmonary arterial hypertension (IPAH) and familiar pulmonary arterial hypertension (FPAH) during conventional therapy era and targeted therapy era.

METHODSIPAH and FPAH patients who were referred between Jan 1999 and Oct 2004 in Fuwai Hospital were defined as conventional therapy era group (before 2005 no PAH-specific drug was available in China). All patients in this group were followed up till Jun 2005. IPAH and FPAH patients who were referred between Sep 2006 and Aug 2011 were defined as targeted therapy era group (new PAH-specific drugs were available in China since 2006) were analyzed. All patients in this group were followed up till Dec 2013. The primary endpoints were death and therapy medicine.

RESULTSSeventy-two patients were enrolled in conventional therapy era group, 375 were enrolled in targeted therapy era group. The mean age was (35.9 ± 12.2) years and (34.5 ± 17.4) years respectively (P = 0.67), and women was predominant in both groups. There was no difference in WHO functional class and hemodynamic data between the two groups. About 90.3% patients were treated by calcium-channel blockers (CCB) in conventional therapy era group. In targeted therapy era group, almost all patients were treated by at least one PAH-specific drug, only 3.2% patients who had a positive response to acute pulmonary vasodilator testing were treated by CCB. The median survival time was 30.4 months in conventional therapy era group and 66.2 months in targeted therapy era group. The 1-, 2-, 3- and 5- year survival rates of IPAH and FPAH patients were 68.0%, 56.9%, 38.9% and 20.8% in conventional therapy era group, and 89.3%, 78.1%, 68.2% and 53.7% in targeted therapy era group respectively (P < 0.000 1).

CONCLUSIONCompared with conventional therapy era, the survival rate of Chinese IPAH and FPAH patients is significantly improved in targeted therapy era.

Adult ; Aged ; China ; epidemiology ; Familial Primary Pulmonary Hypertension ; epidemiology ; therapy ; Female ; Follow-Up Studies ; Humans ; Hypertension, Pulmonary ; epidemiology ; therapy ; Male ; Middle Aged ; Survival Rate ; Young Adult

OBJECTIVETo investigate the inpatient pulmonary hypertension (PH) surveillance in a single center of cardiovascular hospital during last ten years.

METHODSIn this retrospective analysis, data from patients with discharge diagnosis as PH from Jan. 1996 to Dec. 2005 were collected.

RESULTSA total of 7085 out of 106 640 patients (6.63%) were documented as PH during the survey period and 3.77% PH was idiopathic, 65.93% PH originated from congenital heart diseases, 22.61% from left heart diseases, 5.66% from thrombotic diseases, 0.89% from respiratory diseases, 0.61% from connective tissue diseases, 0.51% from pulmonary vasculitis and 0.03% from portal hypertensive diseases. Both total inpatient number and patients with PH increased year by year during the last 10 years in our hospital. The number of in-hospital patients with PH was significantly higher in 2004 - 2005 than that in 1996 - 2003 (P < 0.0001) and more PH was originated from cardiomyopathy and valvular heart diseases. Idiopathic pulmonary hypertension also tended to increase and PH due to congenital heart diseases was significantly reduced during 2004 - 2005.

CONCLUSIONThe data from a single center of cardiovascular hospital shows a tendency for increased in-hospital prevalence of pulmonary hypertension during the last ten years.

Humans ; Hypertension, Pulmonary ; epidemiology ; Inpatients ; statistics & numerical data ; Prevalence ; Retrospective Studies

Objective: To identify the risk factors related to perinatal complications in patients with pulmonary hypertension underwent cesarean section. Methods: We retrospectively analyzed the medical records of all pregnant women with pulmonary hypertension hospitalized in 4 different hospitals in Shandong province and underwent cesarean section between May 2010 and May 2020. Patients were divided into perinatal complication group and control group according to the presence or absence of perinatal complications. Perinatal complications included aggravated heart function, new onset arrythmias, sudden cardiac arrest, all-cause death within 42 days post cesarean section, postpartum bleeding and thrombotic events. Risk factors of perinatal complications were analyzed. Results: A total of 167 patients (47 cases in the perinatal complication group and 120 cases in the control group) were included in this study. The average age of this cohort was 28(24, 32) years, and 75(44.9%) patients suffered newly diagnosed pulmonary hypertension during pregnancy. The main cause of pulmonary hypertension was congenital heart disease (137(82.0%)). Age, pregnant weeks, percent of primipara, intra-cardiac shunt, and receiving targeted medication therapy, cardiac dimensions were similar between the two groups. A total of 62 complications were recorded in the complication group including 28 cases of aggravated heart function, 4 cases of new onset arrythmias, 2 cases of cardiac arrest, 11 cases of bleeding or thrombotic events and 17 patients were dead. Prevalence of idiopathic pulmonary hypertension and general anesthesia was significantly higher, functional capacity was significantly lower in perinatal complication group than in control group (all P<0.05). The estimated systolic pulmonary artery pressure, serum N-terminal pro-B type natriuretic peptide and total bilirubin (TBIL) levels were significantly higher in perinatal complication group than in control group (all P<0.05). Logistic analysis demonstrated WHO Function Class(FC) Ⅲ/Ⅳ (OR=2.416,95%CI 1.016-5.743, P=0.046) and TBIL level (OR=6.874,95%CI 1.643-28.757, P=0.008) were the independent risk factors of perinatal complications. Conclusion: TBIL and WHO FC are independent risk factors of perinatal complications in pregnant women with pulmonary hypertension underwent cesarean section.
Cesarean Section/adverse effects* ; China/epidemiology* ; Female ; Humans ; Hypertension, Pulmonary/epidemiology* ; Pregnancy ; Pregnancy Outcome ; Retrospective Studies ; Risk Factors

Objective: To understand the status, attitude and related risk factors on smoking among 18-65 years old patients with hypertension, diabetes, dyslipidemia, chronic obstructive pulmonary disease (COPD) or asthma in Beijing. Methods: Data was gathered from the 2014 Beijing Non-communicable and Chronic Disease Surveillance Program. Multiple classified cluster sampling method was used and 19 815 participants aged 18-65 were sampled from 16 districts in Beijing. Results: Among all the 18 405 participants, male hypertensive patients showed a higher rate on current smoking than the other groups (χ(2)=17.695, P<0.001). Male patients with dyslipidemia had higher current smoking rate than the other groups (χ(2)=39.292, P<0.001). However, female patients with COPD or with asthma showed higher rate on current smoking than the other groups (χ(2)=6.276, P=0.012), (χ(2)=8.245, P=0.004). Among the smokers, hypertensive patients presented lower rate (χ(2)=20.487, P<0.001) on intention of smoking concession, than the other groups. Patients with COPD showed greater intention in quitting smoking (χ(2)=6.085, P=0.048), than the other groups. Male patients with diabetes (χ(2)=9.219, P=0.010) or dyslipidemia (χ(2)=13.513, P=0.001) who had stopped smoking tobacco appeared having higher rates in keeping the current status. Results from logistic regression analyses showed that smoking was the risk factor for hypertension (OR=1.17), dyslipidemia (OR=1.25), COPD (OR=1.78), and asthma (OR=1.57). Conclusions: Patients with certain kinds of chronic diseases showed higher rate of current smoking and lower rate of quitting. Cigarette consumption appeared an important risk factor for patients with hypertension, dyslipidemia, COPD, or asthma in Beijing.
Adolescent ; Adult ; Aged ; Asthma/epidemiology* ; Beijing/epidemiology* ; Chronic Disease/epidemiology* ; Diabetes Mellitus/epidemiology* ; Female ; Humans ; Hypertension/epidemiology* ; Intention ; Male ; Middle Aged ; Pulmonary Disease, Chronic Obstructive/epidemiology* ; Risk Factors ; Smokers ; Smoking/psychology* ; Smoking Cessation ; Nicotiana/adverse effects*

BACKGROUNDPulmonary 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).

METHODSHospitalized 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.

RESULTSTotally, 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.

CONCLUSIONSThe 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.

Aged ; Echocardiography, Doppler ; Female ; Humans ; Hypertension, Pulmonary ; blood ; epidemiology ; physiopathology ; Idiopathic Pulmonary Fibrosis ; blood ; epidemiology ; physiopathology ; Incidence ; Male ; Middle Aged ; Natriuretic Peptide, Brain ; blood ; Peptide Fragments ; blood ; Pulmonary Artery ; physiopathology ; Smoking