Clinical features of POEMS syndrome initially diagnosed as pulmonary hypertension / 中华全科医师杂志

Objective:To analyze the clinical characteristics, diagnosis and treatments of patients with POEMS syndrome initially diagnosed as pulmonary hypertension (PH).Methods:Clinical data of 7 patients who were initially diagnosed as PH and finally diagnosed as POEMS syndrome in Shanghai Pulmonary Hospital from May 2013 to November 2021 were retrospectively reviewed. Clinical manifestations, laboratory tests, echocardiography, hemodynamic findings, treatment and prognosis of patients were analyzed.Results:Seven patients, including 4 males and 3 female, aged (55±9) (44-62) years were presented with elevated pulmonary artery pressure by echocardiography at admission. Chest tightness and shortness of breath (7/7), fatigue (6/7) and lower limb edema (4/7) were the most common symptoms in the first-episode. Meanwhile, patients also presented symptoms associated with POEMS syndrome, including multiple peripheral neuropathy (7/7), multiserosal cavity effusion (6/7), organomegaly (5/7), skin changes (5/7), and endocrine lesions (4/7). Serum levels of vascular endothelial growth factor (VEGF) were significantly increased in all patients. The pulmonary arterial systolic blood pressure was (66±21)mmHg (1 mmHg=0.133 kPa) estimated by echocardiography. Six patients underwent right heart catheterization and significantly increased mean pulmonary artery pressure((35±9) mmHg) was confirmed; and their pulmonary vascular resistance was (4.00±2.10) Wood U. All patients received corresponding treatment for POEMS syndrome. The excise tolerance was improved in 5 patients after successful treatment with stable or reversed WHO functional class. One patient received hemodialysis treatment for uncontrolled POEMS. One patient died during follow-up. The echocardiography was followed up in 4 patients, and 2 of whom had a complete reversal of PH, 1 had a partial reversal, and 1 had not yet reversed.Conclusions:In patients with PH who have multisystem manifestations, such as multiple peripheral neuropathy, multiserosal cavity effusion, organomegaly and skin changes, POEMS syndrome should be considered, and proper and active treatment of POEMS may reverse PH and improve the prognosis of patients.

Clinical features and outcome of Takayasu arteritis associated pulmonary hypertension / 中华急诊医学杂志

Objective To investigate the clinical features, diagnosis, treatment and outcome of patients with Takayasu arteritis associated pulmonary hypertension (TA-PH). Methods Patients diagnosed as TA-PH in Shanghai Pulmonary hospital from 2008 to 2017 were retrospectively reviewed and followed up. Baseline characteristics including hemodynamics were collected. Data were summarized as mean ± standard deviation or frequency (％). Survival analyses were performed using the Kaplan-Meier method. Results Thirteen TA-PH patients (10 female, aged 39±11 years old) were included. The duration from symptoms onset to diagnosis was 2 months to 50 years, and ten patients were diagnosed TA and PH at the same time. Shortness of breath was the most common clinical manifestation (12 cases), followed by chest pain and tightness (8 cases) and palpitation (6 cases). All patients had a moderated WHO functional class and 8 patients were in active phase. Vessel wall thickening, lumen narrowing, occlusion and/or dilation were found in CT pulmonary angiography and angiography. Mean pulmonary arterial pressure (48.0±14.0) mmHg and pulmonary vascular resistant (7.59±4.21) Wood U were increased. All patients received PH-specific therapies, and patients at active status took glucocorticoid. Stentimplantation in pulmonary artery was performed in 4 patients. Three patients died during the follow-up. Conclusions Patients with TA are at risk of PH, and PH can be the first manifestation of TA, which suggest that PH should be screened regularly in patients with TA and shortness of breath. The prognosis of TA-PH is poor. PH-specific therapies and vascular reconstruction therapy may be effective, but need further investigation.

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>

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>

Quantitative assessment of left ventricular torsion in normal subjects using vector velocity imaging / 中华超声影像学杂志

Objective To evaluate the left ventricular(LV) torsion and rotation in normal subjects using vector velocity imaging.Methods LV basal and apical short-axis images were captured in 10 healthy individuals to estimate LV torsion and rotation using routine 2-dimensional echocardiography and vector velocity imaging. Results As viewed from LV apex,the systolic basal rotation was clockwise (negative value),and apical rotation was counterclockwise (positive Value). The apical peak systolic rotational velocity was significantly higher than the basal [endo:(150±62)°/s vs (114±65)°/s;epi:(81±40)°/s vs (55±28)°s,respectively,P＜0.01]. The peak systolic endocardial rotational velocity and rotation was significantly higher than epicardial rotational velocity and rotation[basal:(-114±65)°/s vs (-55±28)°/s,(-12±6)°vs (-4±1)°;apical:(150±62)°s vs (81±40)°s,(10±4)°vs(6±2)°,respectivelv,P＜0.05]. There were no significant differences in the time to peak systolic rotational velocity/rotation between basal endocardium/epicardium and apical endocardium/epicardium. Conclusions Vector velocity imaging can assess LV torsion and rotation non-invasively,and normal LV has a kind of characteristic motion of torsion.

Left atrial function assessed by speckle tracking imaging in patients with chronic heart failure / 中华超声影像学杂志

Obiective To observe the value of speckle tracking imaging(STI)in assessing left atrial(LA) function in patients with chronic heart failure(CHF).Methods Twenty-two patients with CHF[LVEF(37.1± 10.5)%]and twenty normal controls[LVEF(61.94±10.68)%]were studied.CHF group was subdevided into patients with normal or abnormal LV filling pressure.Velocity vector imaging(Siemens Medical Solutions)was used to acquire 2-D images at parasternallong axis and apical 4-chamber views.The LA dimension and volume,LV volume and ejection fraction,ratio of mitral valve E wave and mitral annulus velocity e'E/e')were measured. LA tangential and radial velocity(Vt-As,Vr-As)and time to peak velocity(TVt-As,TVr-As)were analysed on Syngo Workplace offline.Results Vt-As and Vr-As of the CHF group were significantly smaller than those of the controls(P＜0.05),and Vr-As was even decreased in patient with elevated LV filling pressure(P＜0.05), however,TVt-AS and TVr-AS didn't show evident impairment in the CHF group(P＞0.05).Vt-As and Vr-AS were closely correlated with LVEF,EDV and ESV(P＜0.05).Vt-As was negtively correlated with LA volume (P＜0.05).Conclusions STI can quickly and accurately assess LA function,and help to recognize patients with elevated LV filling pressure,which is useful in the risk stratification,treatment evaluation and prognosis prediction of CHF patients.