Construction and Application of National Rare Diseases Registry System of China / 罕见病研究

Rare diseases have been a major challenge for clinical medicine and public health challenge in China. One of the effective measures is to conduct proactive research on rare diseases to deal with the disease burden of the diseases. However, low prevalence, disperse distribution of patients, lack of knowledge about the disease course, and phenotype heterogeneity hamper the development of research for rare diseases. Recently, it has been found that patients registry is effective in understanding the course of the disease and accu- mulating the cases and data of clinical research or clinical trial design. At present, most of developed countries or regions in the world have promoted clinical research and clinical trials of new medications on rare diseases by using the registration of rare disease. In 2016, Peking Union Medical College Hospital established China's first registry system at the national level-National Rare Disease Registry System of China(NRDRS). NRDRS has accumulated 68 137 cases data registered by the researchers from China's 101 collaborating hospitals in 29 provinces/municipalities/autonomous regions, covering 171 different, and forming 188 cohorts. To date, NRDRS complete the initial stage of resources buildup.Nex stage will be focused on clinical research and clinical trials related to rare diseases based on NRDRS. This article is on the process of building NRDRS, the potential support for conducting clinical research and clinical trials related to rare diseases, and the challenges will be faced.

Clinical efficacy and safety of thrombolytic treatment with reteplase in patients with intermediate-risk acute pulmonary embolism / 中华心血管病杂志

Objective@#To assess the efficacy and safety of thrombolytic treatment with reteplase in patients with intermediate-risk acute pulmonary embolism.@*Methods@#Ten consecutive patients with intermediate-risk acute pulmonary embolism who received thrombolytic treatment with reteplase at Thrombosis and Vascular Medicine Center, Fuwai Hospital from March to November in 2016 were included.Vital signs, right ventricular diameter, systolic pulmonary artery pressure, and biochemical markers were assessed before and after thrombolytic therapy with reteplase, and bleeding complications were also observed during 3 months follow up.@*Results@#(1) For the efficacy outcomes: at 48 hours after thrombolytic treatment with reteplase, echocardiography-derived diameter of right ventricular was significant reduced from (27.9±3.8) mm to (24.8±2.6) mm (P=0.03), systolic pulmonary artery pressure decreased from (63.9±21.6) mmHg(1 mmHg=0.133 kPa) to (34.4±19.8) mmHg (P=0.02). Heart rate and breathing rate were also decreased significantly (both P<0.05), blood pressure remained unchanged post therapy.Hypoxemia was quickly corrected with an significant elevation of PaO2 and SaO2 ((65.2±14.3) mmHg vs. (80.0±9.6) mmHg, P=0.006; (90.8±3.5)% vs. (95.2 ±1.6)%, P=0.002 respectively). PaCO2 was also increased significantly (P<0.05). Serum NT-proBNP and cTnI were decreased significantly (both P<0.05). There was no recurrent pulmonary embolism or deep-vein thrombosis during the 3 months follow-up. (2) For the safety outcomes: a thrombolytic relevant hemoptysis (about 70 ml) occurred in 1 patient, and was controlled by PCC therapy.No other clinically relevant events were observed during thrombolytic treatment. Eight patients were followed more than 3 months, there was no major bleeding complication or death during the follow up period.@*Conclusion@#Treatment of intermediate-risk acute pulmonary embolism with reteplase is effective and safe and there are no obvious side effects.

Peri-operative Management and Result of Pulmonary Endarterectomy in 56 Patients / 中国循环杂志

Objective: To summarize the peri-operative management experience of pulmonary endarterectomy (PEA) in patients with chronic thromboembolic pulmonary hypertension (CTEPH). Methods: A total of 56 CTEPH patients received PEA in our hospital from 2015-01 to 2016-11 were retrospectively analyzed. Our study was focused on the medication in respiratory and circulatory system during ICU stay, peri-operative application of vasoactive drug and target drug to pulmonary hypertension (HP), usage of ventilators, mechanical assisted devices and other management experiences. Results: No peri-operative death occurred. There were 2/56 (3.6%) patients with lung reperfusion, 2 (3.6%) with PH crisis. Compared with pre-operation, the post-operative pulmonary artery hemodynamics parameters were improved as right heart catheter measured pulmonary artery systolic pressure (PASP) decreased from (85.05±22.40) mmHg to (36.83 ±17.21) mmHg and pulmonary vascular resistance decreased from (773.84±342.95) dyn·s·cm-5 to (293.59±214.95) dyn·s·cm-5. Post-operative oxygen saturation was maintained at (95-100) % in all patients. Echocardiography found that PASP from pre-operation (85.03±25.78) mmHg decreased to (39.44±19.24) mmHg at follow-up period, P<0.01.Conclusion: A comprehensive peri-operative management of PEA was helpful to improve pulmonary hemodynamics in CTEPH patients; meanwhile, effective prevention and treatment of severe complication could obviously reduce peri-operative mortality.

Establishment of a model of hydrogen peroxide-induced injury in pulmonary artery endothelium cells and relevant mechanisms of oxidative stress / 中华心血管病杂志

Objective@#To establish a hydrogen peroxide (H2O2) induced injury model of pulmonary artery endothelial cells (PAECs) and explore the molecular mechanisms of oxidative stress on the structure and function of PAECs in this model.@*Methods@#Human PAECs were treated with H2O2 at different concentrations (25, 50, 100, 200, 400, 800, 1 600, 3 200, 6 400 μmol/L) for 4 and 24 h, respectively. The PAECs survival curve was obtained according to the cell viability measured by CCK-8 assay. The cell apoptosis of PAECs was detected by flow cytometry. The reactive oxygen species (ROS) generation and mitochondrial activity were measured using small molecule fluorescent probes. Proteins were extracted and the phosphorylation levels of signal molecules in PAECs were detected by Western blot assays.@*Results@#(1) The effect of H2O2 at various concentrations on cell viability of PAECs: cell viability of PAECs decreased in proportion to increasing concentration of H2O2 after incubation for 4 h. The half maximal inhibitory concentration (IC50) of PAECs exposed to H2O2 for 4 and 24 h were 397.00 and 488.77 μmol/L, respectively. (2) The effect of H2O2 on cell apoptosis of PAECs: After H2O2 incubation for 4 h, proportions of PAECs at late-apoptosis ((22.58±3.69) %) and necrotic stage( (11.86±4.27)%) were significantly higher than those of control PAECs at late-apoptosis stage( (3.41±1.44)%, P<0.01) and at necrotic stage ((1.94±1.15) % , P<0.05). The survival rate of PAECs post H2O2 was dramatically lower than that of control PAECs ((7.98±3.21)% vs. (48.89±8.08)%, P<0.01). However, there is no statistical difference between both groups regarding to the early apoptosis. (3) The effect of H2O2 on mitochondrial activity and ROS production of PAECs: the mitochondrial activity and ROS generation of PAECs treated by H2O2 were significantly increased compared to those in control PAECs (P<0.01). (4) The effect of H2O2 on signaling molecules in PAECs: there was a significant increase in phosphorylation level of Akt in PAECs incubated with H2O2 for 30 minutes compared to that in control PAECs (P<0.01), while there was no significant difference in levels of Akt between H2O2 treated PAECs and control PAECs. Phosphorylation level of JNK as well as p38 were also significantly upregulated in H2O2 treated PAECs (P<0.01).@*Conclusion@#H2O2 at the concentration of 400 μmol/L could induce human PAECs injuries via the regulation of Akt and MAPK signaling pathways.

Characteristics and survival of connective tissue disease associated pulmonary arterial hypertension patients with and without interstitial lung disease / 中华风湿病学杂志

Objective To explore the characteristics and survival of connective tissue disease (CTD) patients with both of pulmonary arterial hypertension (PAH) arnd interstitial lung disease (ILD),and to compare with CTD patients with isolated PAH.Methods All adult CTD patients who visited one of the three referral centers in China with a diagnosis of PAH confirmed by right heart catheterization from July 2006 to May 2011 were enrolled.They were then divided into two groups (ILD with and without-ILD group) based on chest CT and then the comparison of baseline characteristics and survival at the endpoint of follow up were made between the two groups.T test,Mann-Whitney U test,x2 test,Kaplan-Meier survival analysis and Cox regression analysis were used for statistical analyses.Results One hundred and twenty-six patients were recruited into the study.Patients with ILD (n=27) were older than those without ILD (n=99).Lung function results including FVC [(75±18)％ vs (83±13)％,t=2.212,P=0.037] and DLCO [(54±22)％ vs (68±20)％,t=2.392,P=0.019] in ILD group were significantly wose than those without-ILD group.Although some important hemodynamic parameters such as mean pulmonary arterial pressure and pulmonary vascular resistance were better in the ILD group than the without-ILD group,Kaplan-Meier analysis showed that the short term survival of ILD group was significantly worse than that of the without-ILD group (72.7％ versus 94.7％ at 1 year and 63.6％ versus 81.1％ at 3 year,P=0.047).In ILD group,Cox regression analysis showed that SvO2 was the only independent factor for the short term survival [HR=0.19,95％CI (0.04,0.83),P=0.027],and Kaplan-Meier analysis showed patients with SvO2＜60％ had significantly lower short term survival than patients with SvO2 ≥60％ (1 and 2 year survival were 60.0％ and 40.0％ versus 92.9％ and 77.4％ respectively,P=0.002).Conclusion Patients with both PAH and ILD is a special subtype in CTD.Although with the superiority of hemodynamics,these patients have significantly worse survival than CTD patients with isolated PAH.Low SvO2 is the independent risk factor for the short term mortality in patients of CTD complicated by both PAH and ILD.More attention should be paid to these patients and the management strategy should be investigated further.

Prethrombotic status and long-time thromboembolic events in primary hypertensive patients with or without elevated homocysteine level / 中华心血管病杂志

<p><b>OBJECTIVE</b>To evaluate the association between homocysteine level and prethrombotic status and long-term thromboembolic events in patients with primary hypertension.</p><p><b>METHODS</b>Results between 110 hypertensive patients with elevated homocysteine (HCY) level were compared with 110 hypertensive patients with normal HCY level which were enrolled from October 2003 to November 2009. Fibrinogen (FIB), viscosity, thrombomodulin (TM), granule membrane protein (GMP-140), prethrombin F1+2 fragment (F1+2), D-dimer fragment (D-Dimer) and antithrombin III (AT-III) were measured and correlated to HCY and prethrombotic state. The endpoints of the study were arterial and venous thromboembolic events. The variables linked with arterial and venous thromboembolic events were included in Cox proportional hazard models. The event-free survival was illustrated with Kaplan-Meier survival curves and compared by the Log-rank test.</p><p><b>RESULTS</b>The patients were followed up for 8-122 months (median follow-up time was 85 months). Compared with hypertensive patients with normal HCY, the plasma level of TM ((4.8±1.2) µg/L vs. (4.5±1.0) µg/L, P = 0.045), GMP-140 ((18.8±3.2) µg/L vs. (17.1±4.3) µg/L, P = 0.001), F1+2 ((1.2±0.4) nmol/L vs. (1.0±0.6) nmol/L, P = 0.004) were significantly higher while the plasma level of AT-III ((95.3±10.4) % vs. (98.6±10.6)%, P = 0.021) was significantly lower in hypertensive patients with elevated HCY level. FIB, viscosity of plasma and D-dimer were similar between the two groups. Multiple regression analyses indicated that HCY level was negatively correlated with AT-III (β = -0.199, P = 0.011) and positively correlated with age (β = 0.217, P = 0.04), female gender (β = 5.667, P = 0.001) and TM (β = 2.341, P = 0.003). Cox multivariate analysis revealed that age and HCY level were independent prognostic risk factors of thromboembolic events (OR 1.046, 95% CI 1.013-1.082, OR 1.052, 95% CI 1.027-1.078, respectively) (all P < 0.05). Kaplan-Meier curves showed that there was a significant difference in the event-free survival between the two groups (Log-rank test, P = 0.027).</p><p><b>CONCLUSIONS</b>Compared with normal HCY hypertensive patients, the levels of plasma prothrombin activators such as TM, GMP-140 and F1+2 were significant increased and anti-thrombin factor such as AT-III was significant decreased in hypertensive patients with elevated HCY. Old age and high HCY level were independent prognostic risk factors of thromboembolic events. The event-free survival in hypertensive patients with elevated HCY is lower than in hypertensive patients with normal HCY level.</p>

Efficacy of inhaled iloprost on top of other targeted therapies for patients with pulmonary hypertension and severe right heart failure / 中华心血管病杂志

<p><b>OBJECTIVE</b>To investigate the efficacy and safety of inhaled iloprost on top of other pulmonary hypertension (PH) specific therapies for patients with PH and severe right heart failure.</p><p><b>METHODS</b>We consecutively enrolled WHO functional class IV patients with PH and chronic thromboembolic pulmonary hypertension (CTEPH) in Shanghai Pulmonary Hospital from January 2011 to January 2013. Inhaled iloprost was administrated to all enrolled patients, oral endothelin antagonist receptors (ERAs) and/or type 5 phosphodiasterase inhibitors (PDE5-I) were also used as basis therapies. The in-hospital outcomes and the changes of right heart functional parameters were observed.</p><p><b>RESULTS</b>Twenty-four patients with PH and 5 patients with CTEPH were enrolled. After a mean treatment duration of (23 ± 13) days, 3 patients dead and significant improvement was observed in the remaining 26 patients. Compared with the baseline, heart rate decreased from (99 ± 14) to (91 ± 12) bpm (P = 0.001), plasma NT-proBNP level decreased from 5 823 (3 029-13 248) to 3 220 (1 678-6 720) ng/L (P < 0.001), tricuspid annular plane systolic excursion (TAPSE) increased from (1.3 ± 0.4) to (1.4 ± 0.3) cm (P = 0.018), right ventricular diameter decreased (left-to-right diameter from (57 ± 11) to (53 ± 10) mm, P = 0.040, and superoinferior diameter from (69 ± 11) to (64 ± 16) mm, P = 0.027), Tbil also decreased from (41 ± 34) to (26 ± 17) µmol/L (P < 0.001). No severe side effects were observed.</p><p><b>CONCLUSION</b>The strategy of inhaled iloprost on top of other PAH-specific target therapy medications is effective and safe for PH patients with severe right heart failure.</p>

Efficacy comparison of 3 rivaroxaban regimen in patients with venous thromboembolism / 中华心血管病杂志

<p><b>OBJECTIVE</b>To compare the efficacy and safety of 3 rivaroxaban regimen in patients with venous thromboembolism (VTE).</p><p><b>METHODS</b>This is a retrospective study. Thirty three inpatients with VTE received rivaroxaban were divided into 3 groups, in which 16 patients were treated with 15 mg rivaroxaban twice daily for 21 days then followed by 20 mg once per day till 3 months (group 1), 9 patients were treated with 20 mg rivaroxaban once daily for 3 months (group 2), 8 patients were treated with 10 mg rivaroxaban once daily for 3 months. The reduction rate of D-Dimer on the third therapy day, the duration of D-Dimer normalization and hospital stay as well as symptom remission, the imaging assessment results after three months treatment, rate of recurrent VTE, bleeding, liver and kidney function were compared among the 3 groups.</p><p><b>RESULTS</b>The reduction rates of D-Dimer on the third therapy day were significantly higher ((46.12 ± 15.42) % vs. (26.59 ± 8.11) % and (25.55 ± 14.00) %, P = 0.02, P = 0.01), and the duration of D-Dimer normalization was significantly shorter ((17.9 ± 7.7) days vs. (24.1 ± 5.1) days and (26.3 ± 6.2) d, P = 0.03, P < 0.01) in group 1 than in group 2 and 3. There was one recurrent deep-vein thrombosis in group 3, one non-major bleeding in group 1 and group 3. Major bleeding or liver and kidney dysfunction were not observed in these patients.</p><p><b>CONCLUSIONS</b>Venous thromboembolism can be safely and effectively treated by rivaroxaban, and does of 15 mg twice daily for 21 days followed by 20 mg once daily for 3 months are superior to the other 2 tested therapy regimen in this patient cohort.</p>

Pulmonary arterial hypertension after operation for congenital heart disease: analysis of baseline clinical characteristics of 122 Chinese patients / 中华心血管病杂志

<p><b>OBJECTIVE</b>To explore the baseline clinical characteristics of patients with pulmonary arterial hypertension after operation for congenital heart disease (AO-CHD-PAH) in China.</p><p><b>METHODS</b>A total of 122 consecutive patients diagnosed as AO-CHD-PAH in Shanghai Pulmonary Hospital from September 1, 2006 to August 31, 2011 were retrospective analyzed.</p><p><b>RESULTS</b>Sixty-seven percent patients were female. The median age at procedure and diagnosis were 14.8 and 24.8 years old, respectively. The median duration from procedure of CHD to diagnosis of PAH was 7.3 years. The most frequent initial symptom (109/122, 89.3%) attributed to PAH was dyspnea on exertion. The frequent sign was accentuation of second heart sound on pulmonary valve area (113/122, 92.6%).Incidence of WHO functional PAH classes I/II was 53.3% (65/122) and mean 6 minutes walk distance was limited to (408.4 ± 103.3) m. Borg dyspnea score was 2.0 (1.0, 3.0).Right heart catheterization demonstrated severe elevated mean pulmonary arterial pressure [(71.6 ± 24.2) mmHg, 1 mmHg = 0.133 kPa] and pulmonary vascular resistance index [(29.1 ± 16.0) Wood U·m(2)] in this patient cohort. Cardiac index was (2.7 ± 0.9) L·min(-1)·m(-2), 90.2% (110/122) patients received PAH-specific therapy. The majority of PAH-specific therapy was phosphodiesterase type 5 inhibitor, which is somehow different from PAH-specific therapy regimen of American-European developed countries.</p><p><b>CONCLUSIONS</b>PAH-specific therapy rate is satisfactory for pulmonary arterial hypertension after operation for congenital heart disease in China and phosphodiesterase type 5 inhibitors are predominant PAH-specific medication in China.</p>

Comparison of clinical characteristics and survival on patients with idiopathic pulmonary arterial hypertension and familial pulmonary arterial hypertension during conventional therapy era and targeted therapy era / 中华心血管病杂志

<p><b>OBJECTIVE</b>To 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.</p><p><b>METHODS</b>IPAH 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.</p><p><b>RESULTS</b>Seventy-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).</p><p><b>CONCLUSION</b>Compared with conventional therapy era, the survival rate of Chinese IPAH and FPAH patients is significantly improved in targeted therapy era.</p>

Long-term effects with ambrisentan monotherapy in patients with pulmonary arterial hypertension / 中华心血管病杂志

<p><b>OBJECTIVE</b>To investigate long-term efficacy and safety of ambrisentan monotherapy in patients with pulmonary arterial hypertension (PAH).</p><p><b>METHODS</b>Patients with PAH who received 2.5 mg or 5 mg of ambrisentan once daily between July 10, 2011 and August 30, 2012 for at least 6 months were enrolled. The efficacy endpoints were change in exercise capacity, World Health Organization (WHO) functional class and N-terminal pro-brain natriuretic peptide (NT-proBNP) level, echocardiographic parameters. The safety endpoint was the safety of long-term ambrisentan administration, as defined by the incidence and severity of adverse events.</p><p><b>RESULTS</b>A total of 18 patients with PAH were enrolled. Mean age was (39 ± 17) years, 8 (55.6%) were female, and 11 (61.1%) patients were in WHO functional class III. The median duration of treatment was 17 months (range: 6-26 months). After treatment, the 6MWD was significantly increased[ (495 ± 97) m vs. (400 ± 91) m, P < 0.001], NT-proBNP was significantly reduced [308 (53-1 645) ng/L vs. 80(22-454) ng/L, P = 0.005], the systolic pulmonary artery was significantly decreased [(62 ± 30) mmHg vs. (82 ± 41) mmHg, P = 0.001] and left ventricular end diastolic diameter was significantly increased [(44 ± 6) mm vs. (40 ± 6) mm, P < 0.004] compared to pre-treatment. WHO functional class was improved compared with baseline in 11(61.1%) patients, stable in 7(38.9%) patients. No patient died during the treatment period. No patient was withdrawn from this study for safety reasons.</p><p><b>CONCLUSIONS</b>Long-term treatment of ambrisentan can effectively improve the exercise capacity, reduce systolic pulmonary artery pressure and NT-proBNP in PAH patients. Ambrisentan is safe and well tolerated in Chinese PAH patients.</p>

Intra- and interobserver reproducibility in the assessment of coronary artery disease: evaluation with invasive coronary angiography and CT coronary angiography / 中华放射学杂志

Objective To investigate the intra- and interobserver repeatability of coronary artery disease (CAD) diagnosis based on invasive coronary angiography (ICA) and CT coronary angiography (CTCA).Methods Two readers with comparable experience ( over 10 years) independently evaluated ICA results of 42 consecutive patients with a blind method. After 30 days,one of them reviewed the same patients again.Another two comparable-experience (over 10 years) readers evaluated the results of CTCA (prospectively ECG-triggering) from the same 42 patients in the same way.The inter-reader and intra-reader repeatability of ICA and CTCA were analyzed by performing Kappa test and calculating the percentage of the segments with agreement on stenotic degree.Using ICA as reference,the accuracy of CTCA in diagnosing CAD was studied by comparing the area under ROC. Results The Kappa between readers for ICA and CTCA were 0.91 and 0.81.Intra-reader Kappa were 0.92 and 0.83 respectively (x2 =509.4 and 432.5,all P ＜0.01 ).The percentage of the segments with agreement between readers on the degree of stenosis were 80.8％ (494/611) in ICA and 75.2％ (469/624) in CTCA ( x2 =2.75,P =0.10),and within the same reader,86.9％ (531/611)in ICA and 81.9％ (511/624) in CTCA(x2 =3.76,P =0.053).With≥ 50％narrowing as a CAD diagnosis criterion,the agreement rates for two readers were 96.6％ (590/611 ) in ICA and 94.4％ (589/624) in CTCA( x2 =3.36,P =0.07),and for the same reader,97.4％ (595/611) in ICA,95.4％ (595/624) in CTCA ( x2 =3.62,P =0.06).Using ICA as reference,two readers of CTCA results achieved a sensitivity and specificity of 84.9％ (530/624)and 98.1％ (612/624).The area under ROC was 0.94 (95％ CI 0.91-0.97).Conclusions Both ICA and CTCA demonstrate good repeatability in diagnosing CAD.The repeatability of ICA is superior to that of CTCA.A certain discrepancy exists in two readings from the same reader or two readers.

Study in correlation of anxiety and depression condition with social support of patients with pulmonary hypertension / 中国实用护理杂志

Objective To investigate the effects of social support intervention on anxiety and depression of pulmonary hypertension(PH)patients,then provide a scientific basis for nursing of patients with pulmonary hypertension.Methods The general condition of PH questionnaire,self-rating anxiety scale (SAS),self-rating depression scale(SDS),social support rating scale(SSRS)were distributed to 131 patients with PH.Then make statistical analysis of patients'anxiety,depression and social support conditions.Results The score of anxiety and depression psychological conditions of patients with pulmonary hypertension was significantly higher than normal population,the difference wag statistically significant.Among 131 patients,16 patients with anxiety,accounting for 12.21% ;21 cases of patients with depression,accounting for 16.03% .28 patients at a high level of social support,92 patients at a medium level of social support,11 patients at a low level of social support,a total of 91.60% of the patients in the middle and higher levels of social support.Anxiety and depression scores had significant negative correlation with social support,objective support points and subjective support points.The anxiety,depression difference among different types of pulmonary hypertension was statistically significant.The difference of anxiety and depression scores between patients with idiopathic pulmonary arterial hypertension and patients with pulmonary hyper tension caused by congenital heart disease were significant.The depression scores between pulmonary hy pertension caused by pulmonary veno-occlusive disease and congenital heart disease were significantly different.Conclusions When nurses care pulmonary hypertension patients.those with different types of PH should be given targeted social support.Attention should be paid to transfer the source of social support to help them adopt a positive attitude to face the diseabe,then improve the treatment and care compliance of patients.

Safety management of 15 patients with severe pulmonary arterial hypertension treated by aerosolized iloprost / 中华护理杂志

This paper summarizes the key points of safety management for 15 patients with severe pulmonary arterial hypertension treated by aerosolized iloprost. All patients achieved significant improvements and none of them suffered any severe side effect. Complete safety management during the therapeutic procedure improved the patients' treatment confidence and compliance,and thereafter strengthened the efficacy of treatment.

Study on the Symptomatic Characteristics of Chinese Medicine of 52 Patients with Pulmonary Hypertension / 中国中医药信息杂志

Objective To study the symptomatic characteristics distribution of Chinese medicine of patients with pulmonary hypertension.Methods Symptomatic information of 52 patients with pulmonary hypertension was collected to find the symptomatic characteristics of Chinese medicine,and analyse the correlation of symptom and pulmonary hypertension with different types and degrees.Results The proportion of TCM symptom of pulmonary hypertension from high to low were syndrome of strong Qi sinking(96.2%),syndrome of blood stasis(75.0%),syndrome of deficiency of lung Qi(42.3%),syndrome of deficiency of heart Qi(30.8%),syndrome of deficiency of spleen Qi(17.3%).Syndrome of blood stasis was the most closely related to the congenital cardiopathy(CC) with pulmonary hypertension.Syndrome of deficiency of heart Qi was the most closely related to the degree of pulmonary hypertension increased pressure.The next one was syndrome of deficiency of lung Qi.Conclusion Syndrome of strong Qi sinking,syndrome of blood stasis,syndrome of deficiency of heart Qi and syndrome of deficiency of lung Qi are the main syndromes of pulmonary hypertension.Syndrome of strong Qi sinking is the most closely related to the pulmonary hypertension.