ABSTRACT
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.
ABSTRACT
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.
ABSTRACT
Objective To investigate the alterations of pulmonary dead space,pulmonary shunt fraction (Qs/Qt) and gas exchange after thromboembolism of lungs in swines and to evaluate the mechanism in respect of pathophysiology and implication.Methods Swine model of pulmonary thromboembolism was made by injection of thrombus into pulmonary artery.Eight swine were used for study of physiologic dead space (VDphy),alveoli dead space (VDalv),airway dead space (VDaw),Qs/Qt,pulmonary arterial pressure (PAP),oxygen partial pressure in arterial blood (PaO2 ),carbon dioxide partial pressure in arterial blood (PaCO2 ),alveolar-arterial O2 gradient (PA-aDO2) determined 30 min before thromboembolism and 0 min,30 min,60 min,120 min after thromboembolism.Results The results showed that VDphy,VDalv,Qs/Qt,PAP,PA-aDO2 increased markedly after thromboembolism in comparison with pre-thromboembolism (P ＜ 0.01 ),while PaO2 declined significantly (P ＜ 0.01 ),and the differences in VDaw and PaCO2 were not significant ( P ＞ 0.05 ).SNK test showed that VDphy and VDalv declined to be smaller at 60 min,120 min than those at 30 min,and PAP declined to be lower at 30 min,60 min,120 min than that at 0 min.Other variables did not change at different intervals after thromboembolism.Hemodynamics did not change significantly after embolization.Conclusions After pulmonary thromboembolism,a various of changes in pathophysiology happened such as increase in dead space,Qs/Qt and PA-aDO2 and decrease in PaO2,but PaCO2 did not change.
ABSTRACT
Objective To investigate the mechanism and significance of low concentration nitric oxide (NO) inhalation in the treatment of pulmonary thromboembelism in swine. Method The pulmonary thromboem-bolism(PTE) model was made in 15 healthy infantile swines which were subsequently assigned to either control group (n = 8) or NO group (n = 7). Swines of the control group were not treated with any medicine, while 10 ppm of NO was administered by continuous inhalation for 2 hours to swines of NO group. Volume of physiological dead space (VDphy), volume of alveolar dead space (VDalv), intrapulmonary shunt (Qs/Qt), mean pulmonary arterial pressure (PAP), systolic blood pressure (SBP), heart rate (HR), cardiac output (CO), arterial blood pH (pH), arterial partial pressure of carbon dioxide (PaCO2) and arterial partial pressure of oxygen (PaO2) were measured 30 min before and 0 min, 30 min, 60 min, 120 min and 180 min after establishment of VIE. Results The post-FIE VDphy, VDalv, Qs/Qt and PAP in both groups increased markedly after PTE compared with the cor-responding pre-PTE measurements (P < 0.01). Post-FIE PaO2 of both groups decreased significandy (P <0.05 and P <0.01), while significance difference was found between pre- and post-PTE HR, SBP, CO, pH or PaCO2 in neither groups (P > 0.05). Both post-PTE PAP and VDalv in NO group were markedly lower(P <0.05 and P <0.01) and beth PaCO2 and PaO2 were much higher than those of the control group (P <0.05). No signi-fieant difference were found in other measurements between two groups. Conclusions Pulmonary arterial pressure may be lowered, alveoli dead space may be reduced and PaCO2 increased by low concentration NO inhalation for the treatment of PIE without decline in haemodynamic status.
ABSTRACT
Objective To investigate the mechanism and significance of low density nitric oxide (NO) in-halation combined with urokinase (UK) in treatment of pulmonary thromboembolism in swine. Method PIE model was estabhshed in 12 healthy infant swines, which were subsequently assigned to UK group or UK+NO ter establishment of the PIE model;in the UK+NO group, swines received continuous NO inhalation of 10 ppm NO for two hours in addition to administration of UK no done in the UK+NO group. Volume of physiological dead space (VDphy), volume of alveolar dead space (VDalv), intrapoulmonary shunt (Qs/Qt), mean ptdmonary arteri-al pressure (PAP), systolic blood pressure (SBP), heart rote (HR), cardiac output (CO), arterial blood pH val-ue, arterial partial pressure of carbon dioxide (PaCO2) and arterial partial pressure of oxygen (PaO2) were mea-sured at 30 min before and 0 min, 30 min, 60 rain, 120 min and 180min after establishment of pulmonary em-bolism.All date were analyzed by ANOVA (SNK-q test),and P<0.05 was considered as significantly differet. Results After PE, VDphy, VDalv, Qs/Qt and PAP of both groups increased markedly compared with the pre-PE values (P<0.01), but the post-PE PAP showed a tendency of decline as time passed. Post-PE PaO2 of both groups decreased significantly (P<0.05 and P<0.01). There were no significant differences in HR, SBP, CO, pH or PaCO2 between pre-PE and post-PE (P>0.05). Both pre- and Post-PE PAP of UK+NO group were markedly less than those of the UK group (P<0.05 and P<0.01). No significant difference was found in other measurements between the two groups. Conclusions UK combined with low density NO inhalation may lower pul-monary arterial pressure promptly to alleviate PIE without distur bance in hemodynamics or gas exchange status and without pulmonary raterial pressure rebound.
ABSTRACT
Objective To study the value of Geneva score assessment combined with simplified rapid plasma D-dimer assay(SRPDDA)for early diagnosis of pulmonary embolism(PE).Methods Clinical data of 658 suspected cases of PE hospitalized at Shanghai Pulmonary Hospital during January 1,1995 to November 30,2007 were retrospectively analyzed.The patients were divided into three categories based on their Geneva score assessment,highly suspected with equal to or greater than 9 scores,intermediately suspected with 5-8 scores and mildly suspected with equal to or less than 4 scores,respectively.Clinical diagnosis for PE of the patients was made based on their probability Geneva score developed by Wicki,SRPDDA,and both Geneva score assessment and SRPDDA,respectively,and compared with results of lung imaging.Results Finally,PE diagnosis was established in 267 cases.Sensitivity for Geneva score and SRPDDA to diagnose PE was 88.4%and 90.3%,with negative predictive value(NPV)of 88.9%and 90.2%and Youden Index(YI)of 51.7%and 51.4%,respectively.And,sensitivity of both Geneva score assessment combined with SRPDDA wag 97.8%,with NPV and YI of 97.3%and 53.8%,respectively,with a likelihood ratio(LR)for both positive test results of 2.62 and a LB for both negative test results of 0.04,respectively.Conclusions As a routine and non-invasive diagnostic test,Geneva score assessment,as well as SRPDDA,play a screening role in early diagnosis for PE,which can help to save unnecessary cost of imping,and complement each other so to improve accuracy of diagnosis and reduce misdiagnosis.