Risk Stratification and Prognosis of Pulmonary Arterial Hypertension Associated with Congenital Heart Disease

Background:Current guidelines for managing pulmonary arterial hypertension(PAH)recommend a risk strati-fication approach.However,the applicability and accuracy of these strategies for PAH associated with congenital heart disease(PAH-CHD)require further validation.This study aims to validate the reliability and predictive accuracy of a simplified stratification strategy for PAH-CHD patients over a three-year follow-up.Additionally,new prognostic variables are identified and novel risk stratification methods are developed for assessing and managing PAH-CHD patients.Methods:This retrospective study included 126 PAH-CHD patients.Clinical and biochemical variables across risk groups were assessed using Kruskal-Wallis and Fisher’s exact tests.Indepen-dent risk factors were identified using ordered logistic regression,while Kaplan-Meier and Cox proportional hazards regression analyses evaluated their impact on all-cause mortality.A new stratification model for the PAH-CHD population was constructed based on these analyses.Results:Significant survival differences across stratified risk groups were observed(p<0.001),validating the effectiveness of the simplified risk stratification method in PAH-CHD patients.Prothrombin activity was a strong independent predictor of adverse outcomes of PAH-CHD patients(Hazard ratio 0.95,p<0.001,C-index 0.70).A model combining N-terminal pro-brain natriuretic peptide,prothrombin activity,albumin,and right atrial area achieved an area under the curve of 0.89 and a C-index of 0.85.Conclusions:The simplified risk stratification method is applicable to PAH-CHD patients.Prothrombin activity is a strong independent predictor of adverse outcomes.A comprehensive risk stratification approach,incorporating both established and novel biomarkers,enhances accessibility and offers predictive efficacy during follow-up for PAH-CHD patients,comparable to established models.

Glycemic and blood pressure control in older patients with hypertension and diabetes： association with carotid atherosclerosis

Backgroud Numerous studies have confirmed the effectiveness of slowing the progression of atherosclerosis by blood pressure （Bp） control in patients with hypertension and several studies also showed the efficacy of intensive glycemic control in decreasing progression of carotid intima-media thickness （CIMT） in patients with type 1 and type 2 diabetes. However, few studies have compared the relative importance of glycemic w＇. Bp control in patients with diabetes and hypertension. We aimed to investigate the association between Bp and glycemic control and subclinical carotid atherosclerosis in older patients with hypertension and type 2 diabetes. Methods In a cross-sectional study, B-mode high-resolution ultrasonography of the carotid artery was performed in 670 subjects （508 males and 162 females） aged 60 years or over who had self-reported hypertension and diabetes but no history of coronary heart disease or stroke. Subjects were categorized by their systolic blood pressure： tight control, 〈 130 mmHg; usual control, 130-139 mmHg; or uncontrolled, 〉 140 mmHg, and by their hemoglobin Alc （HbAlc） level： tight control, 〈 6.5%; usual control, 6.5%-7.5%; or uncontrolled, 〉 7.5%, respectively. Results The mean CIMT was 8.20 ±0.11 mm, and carotid plaque was found in 52.5% （352/670） subjects. Overall, 62.1% of the subjects had subclinical carotid atherosclerosis, defined as having either carotid plaque or elevated CIMT （≥ 1.1 ram）. The mean CIMT was significantly different between Bp control categories （7.60 ± 0.09 mm, 7.90 ±0.08 mm, and 8.60 ± 0.12 mm, respectively, P = 0.03） but not between glycemic control categories （8.20± 0.10 mm, 8.1 ±0.08 mm, and 8.40 ± 0.14 ram, respectively, P = 0.13） using ANCOVA analysis. Multivariable logistic regression adjusting for potential confounding factors showed that usual or uncontrolled Bp control were associated with having carotid plaque （OR = 1.08 and OR=1.42, respectively）, or elevated CIMT [Odd ratio （OR） = 1.17, 95% confidence interval （CI） 1.04-2.24, and OR = 1.54, 95% CI 1.36-2.96, respectively compared to tight Bp control; but did not show glycemic control as independent predictor of either having carotid plaque or elevated CIMT. Conclusions In older patients with hypertension and diabetes, blood pressure control, but not glycemic control is associated with subclinical carotid atherosclerosis.

Endothelial Function and Carotid Artery Wall Thickening in Patients with Early Essential Hypertension

To investigate the relationship between endothelial function and carotid artery wall thickening in patients with early mild essential hypertension, the percent dilatation of humerus diameter and intimal-medial thickening (IMT) of common carotid artery was measured by using high-resolution ultrasound in 20 patients with early mild essential hypertension and 18 patients with normotension.The patients with hypertension had not been treated and their history of increased blood pressure was less than 12 months. In essential hypertension group, the percent dilatation of humerus diameter decreased significantly (2. 65 ± 0. 98 % vs6. 38 ± 1. 61 % ) ; IMT of carotid artery increased (0. 88 ± 0. 16mm vs 0. 58 ±0. 08). There was significant negative correlation between IMT and the percentdilatation of humerus diameter (γ= - 0. 82, P<0. 05), and no correlation be-tween IMT and 24 h mean systolic, diastolic pressure (γ= 0. 12 and γ= 0.07,respectively ; P> 0. 05). Our results suggested that there was endothelial dys-function in early mild essential hypertension. Endothelial dysfunction may not on-ly contribute to the pathogenesis of hypertension but also serve as the most impor-tant inducing factor leading trans formation from hypertension to atherosclerosis.

Relationship between serum inflammatory response cytokines and the change of plaque properties in patients with H-type hypertension complicated by carotid atherosclerosis

Objective:To study the relationship between serum inflammatory response cytokines and the change of plaque properties in patients with H-type hypertension complicated by carotid atherosclerosis.Methods: Patients who were diagnosed with H-type hypertension complicated by carotid atherosclerosis in Yulin Hospital of Traditional Chinese Medicine between May 2014 and December 2016 were selected as group A, patients with H-type hypertension complicated by carotid atherosclerosis and normal Hcy levels were selected as group B, patients with carotid atherosclerosis and normal blood pressure levels and Hcy levels were selected as group C, and healthy volunteers during the same period were selected as control group. Serum levels of inflammatory response cytokines and plaque property-related cytokines were detected.Results: Serum Hcy, sICAM-1, MCP-1, YKL-40, IL-6, PTX3, FGF23, MMP9 and Caspase-3 levels of group A, group B and group C were significantly higher than those of group D, serum Hcy, sICAM-1, MCP-1, YKL-40, IL-6, PTX3, FGF23, MMP9 and Caspase-3 levels of group A and group B were significantly higher than those of group C, and serum Hcy, sICAM-1, MCP-1, YKL-40, IL-6, PTX3, FGF23, MMP9 and Caspase-3 levels of group A were significantly higher than those of group B;serum Hcy, sICAM-1, MCP-1, YKL-40, IL-6, PTX3, FGF23, MMP9 and Caspase-3 levels of group A with vulnerable plaques were significantly higher than those of patients with stable plaques. Serum Hcy, sICAM-1, MCP-1, YKL-40 and IL-6 levels of group A were positively correlated with PTX3, FGF23, MMP9 and Caspase-3 levels.Conclusion: Serum inflammatory response cytokine secretion increases in patients with H-type hypertension complicated by carotid atherosclerosis, and it can result in the change in plaque properties and the decrease in stability.

Accessory renal arteries-a source of hypertension:A case report

BACKGROUND Secondary hypertension is a relatively rare condition most commonly caused by renovascular disease due to atherosclerotic vascular disease or fibromuscular dysplasia.Although accessory renal arteries are frequent,to date,only six cases of secondary hypertension determined by their existence have been reported.CASE SUMMARY We describe a case of a 39-year-old female who came to the emergency department with an urgent hypertensive crisis and hypertensive encephalopathy.Despite normal renal arteries,the computed tomography angiography revealed an inferior polar artery with 50%stenosis of its diameter.Conservative treatment with amlodipine,indapamide and perindopril was adopted,leading to blood pressure control within one month.CONCLUSION To the best of our knowledge,there are controversies regarding accessory renal arteries as a potential etiology for secondary hypertension,but the seven similar cases already described,along with the current case,could reinforce the necessity of more studies concerning this subject.

Correlation analysis of carotid intima media thickness and function in patients with H-type hypertension and acute cerebral infarction

Objective:To explore the correlation of carotid intima media thickness with function in patients with H-type hypertension and acute cerebral infarction.Methods:A total of 150 patients with acute cerebral infarction who were admitted in our hospital from June, 2016 to June, 2017 were included in the study and divided into H-type hypertension group (H-type hypertension merged with acute cerebral infarction, Hcy≥10 μmol/L), non-H-type hypertension group (non- H-type hypertension merged with acute cerebral infarction, Hcy≥10 μmol/L), and the control group (no hypertension but with acute cerebral infarction) according to Hcy level and whether being suffered from hypertension or not with 50 cases in each group. Moreover, 50 healthy individuals who came for physical examinations were served as the healthy group. The morning fasting peripheral venous blood was collected at physical examination time for patients in the healthy group and after admission for patients with acute cerebral infarction. Hcy, sICAM-1, MCP-1, and YKL-40 were detected. The color Doppler ultrasound diagnostic apparatus was used to detect IMT, distensibility, and stiffness. Results: Hcy, MCP-1, sICAM-1, and YKL-40 levels in H-type hypertension group, non-H-type hypertension group, and the control group were significantly higher than those in the healthy group. Hcy, MCP-1, sICAM-1, and YKL-40 levels in H-type hypertension group and non-H-type hypertension group were significantly higher than those in the control group. Hcy, MCP-1, sICAM-1, and YKL-40 levels in H-type hypertension group were significantly higher than those in non-H-type hypertension group. IMT and stiffness in H-type hypertension group, non-H-type hypertension group, and the control group were significantly greater than those in the healthy group, while distensibility was significantly less than that in the healthy group. IMT and stiffness in H-type hypertension group and non-H-type hypertension group were significantly greater than those in the control group, while distensibility was significantly less than that in the control group. IMT and stiffness in H-type hypertension group were significantly greater than those in non-H-type hypertension group, while distensibility was significantly less than that in non-H-type hypertension group.Conclusions: Hcy can directly affect carotid AS, increase carotid IMT, and promote the occurrence of hypertension merged with acute cerebral infarction;therefore, positive monitoring of serum Hcy level and IMT thickness in patients with hypertension and acute cerebral infarction and early intervention are of great significance in reducing the occurrence of carotid AS, delaying the progression of carotid AS, and preventing hypertension and acute cerebral infarction.

Effect of folic acid combined with routine drugs on Hcy metabolism, inflammatory response and plaque properties in patients with H-type hypertension complicated by carotid atherosclerosis

Objective:To explore the effect of folic acid combined with routine drugs on Hcy metabolism, inflammatory response and plaque properties in patients with H-type hypertension complicated by carotid atherosclerosis.Methods: A total of 68 patients with H-type hypertension complicated by carotid atherosclerosis who received treatment in our hospital between January 2014 and September 2016 were collected and divided into control group (n=34) and observation group (n=34) according to random number table. The control group received routine therapy and the observation group received folic acid combined with routine drug therapy. The differences in Hcy metabolism, inflammatory response and plaque properties were compared between the two groups of patients before and after treatment.Results:Before treatment, differences in serum Hcy contents, pro-inflammatory and anti-inflammatory mediator contents, and ultrasound plaque property parameter levels were not statistically significant between two groups of patients. After treatment, serum Hcy content of observation group was lower than that of control group;serum pro-inflammatory factors IL-1, IL-6, IL-8 and CRP contents were lower than those of control group;serum anti-inflammatory factors IL-4, IL-10 and IL-13 contents were lower than those of control group;ultrasound plaque property parameter strain value was lower than that of control group while plaque strain rate was higher than that of control group.Conclusion:Folic acid treatment based on routine treatment can effectively accelerate the Hcy metabolism, reduce systemic inflammatory response and increase the plaque stability in patients with H-type hypertension complicated by carotid atherosclerosis.

Effect of ginkgo capsules combined with secondary preventive drugs on the endothelial injury and plaque properties of patients with hypertension complicated by carotid atherosclerosis

Objective: To study the effect of ginkgo capsules combined with secondary preventive drugs on the endothelial injury and plaque properties of patients with hypertension complicated by carotid atherosclerosis. Methods: A total of 178 patients with hypertension complicated by carotid atherosclerosis who were treated in Chengyue Community Health Service Center of Xisaishan District Huangshi City Hubei Province between February 2015 and January 2017 were collected and randomly divided into two groups. Control group were treated with conventional secondary preventive drugs, and observation group were treated with ginkgo capsules combined with secondary preventive drugs. The differences in serum endothelial injury indexes and lipid metabolism indexes as well as carotid artery ultrasound parameters were compared between the two groups before and after treatment. Results: Before treatment, endothelial injury indexes and lipid metabolism indexes as well as carotid artery ultrasound parameters were not significantly different between two groups. After treatment, serum ET, AngⅡ, TC, LDL-C and LpA contents as well as carotid artery ultrasound parameters DS and AS levels of observation group were lower than those of control group while serum NO and HDL-C contents as well as carotid artery ultrasound parameter MLD level were higher than those of control group. Conclusion: Ginkgo capsule combined with secondary preventive drugs can effectively reduce the endothelial injury and stabilize the plaques in patients with hypertension complicated by carotid atherosclerosis.

Extracorporeal membrane oxygenation as a bridging therapy for patients with thiamine deficiency-induced pulmonary arterial hypertension

Thiamine deficiency is characterized by a deficiency of thiamine(vitamin B1),lactic acidosis,pulmonary arterial hypertension(PAH),right-sided heart failure,and peripheral nerve damage.Thiamine deficiency is easily misdiagnosed and missed during clinical practice and is associated with death in severe cases.[1,2]Here we reported a patient who was admitted to the hospital with severe metabolic acidosis and pulmonary hypertension and finally diagnosed with thiamine deficiency by detecting plasma vitamin B1 levels.Since clinically obvious thiamine deficiency is rare,and cases of thiamine deficiency requiring extracorporeal membrane oxygenation(ECMO)treatment are rarer,we believe that our case will be helpful for emergency clinicians.

The Potential of Circular RNAs as Biomarkers in Pulmonary Arterial Hypertension Related to Congenital Heart Disease

A particular type of endogenous noncoding RNAs known as circular RNAs(circRNAs)has now become possible biomarkers for several diseases because of their stability and tissue-specific expression patterns.CircRNAs might play a role in various of biological processes.The identification of particular circRNAs dysregulated in pulmonary arterial hypertension(PAH)raises the possibility of these molecules serving as biomarkers for the disease’s early diagnosis and treatment.This review mainly summarizes the role and potential of circRNA as a future biomarker in PAH related to congenital heart disease.This study presented several potential circRNA targets as diagnostic biomarkers for PAH,discussed their biological functions,and addressed the challenges that need to be considered for their application in clinical settings.

Mufangji tang ameliorates pulmonary arterial hypertension through improving vascular remodeling,inhibiting inflammatory response and oxidative stress,and inducing apoptosis

Background:Mufangji tang(MFJT)is composed of Ramulus Cinnamomi,Radix Ginseng,Cocculus orbiculatus(Linn.)DC.,and Gypsum.In clinical settings,MFJT has been effectively employed in addressing a range of respiratory disorders,notably including pulmonary arterial hypertension(PAH).However,the mechanism of action of MFJT on PAH remains unknown.Methods:In this study,a monocrotaline-induced PAH rat model was established and treated with MFJT.The therapeutic effects of MFJT on PAH rat model were evaluated.Network pharmacology was conducted to screen the possible targets for MFJT on PAH,and the molecular docking between the main active components and the core targets was carried out.The key targets identified from network pharmacology were tested.Results:Results showed significant therapeutic effects of MFJT on PAH rat model.Analysis of network pharmacology revealed several potential targets related to apoptosis,inflammation,oxidative stress,and vascular remodeling.Molecular docking showed that the key components were well docked with the core targets.Further experimental validation results that MFJT treatment induced apoptosis(downregulated Bcl-2 levels and upregulated Bax levels in lung tissue),inhibited inflammatory response and oxdative stress(decreased the levels of IL-1β,TNF-α,inducible NOS,and malondialdehyde,and increased the levels of endothelial nitric oxide synthase,nitric oxide,glutathione and superoxide dismutase),reduced the proliferation of pulmonary arterial smooth muscle cells(downregulated ET-1 andβ-catenin levels and ERK1/2 phosphorylation,increased GSK3βlevels).Conclusion:Our study revealed MFJT treatment could alleviate PAH in rats via induction of apoptosis,inhibition of inflammation and oxidative stress,and the prevention of vascular remodeling.

Intervention control of aerobic exercise in maintaining quality of life and pulmonary hypertension in hemodialysis patients

BACKGROUND Pulmonary hypertension is a serious complication in the treatment of maintenance hemodialysis patients,which seriously affects the quality of life of patients and threatens their life safety.Prevention,treatment and improvement of pulmonary hypertension are of great significance to improve the quality of life of patients.AIM To investigate the intervention and control of pedal-powered bicycle in maintaining quality of life and pulmonary hypertension in hemodialysis patients.METHODS 73 patients with maintenance hemadialysis combined with pulmonary arterial hypertension at a hemodialysis center in a certain hospital from May 2021 to May 2022 are selected.Patients are divided into two groups,37 cases in the control group(group C)and 36 cases in the intervention group(group I).Patients are divided into two groups,group C is treated with oral administration of betaglandin sodium combined with routine nursing care.Based on group C,group I conducts power cycling exercises.RESULTS After treatment,group I patients had higher muscle strength,36-Item Short Form Health Survey scores,and Kidney Disease Targets Areas scores;The 6-minute walk distance test index level was higher and the Borg score was lower;The group I had lower systolic blood pressure,greater vital capacity,higher positive emotion,lower systolic pulmonary artery pressure index level,higher arterial partial oxygen pressure level,lower pulmonary vascular resistance index level,and higher blood oxygen saturation level[158.91±11.89 vs 152.56±12.81,1795.01±603.18 vs 1907.20±574.15,24.00(22.00,29.00)vs 24.00(22.00,28.00),P<0.001].CONCLUSION Aerobic exercise combined with Western medicine treatment can effectively improve patients'pulmonary hypertension,alleviate their negative emotions,and enable them to achieve a higher level of quality of life.

Arterial Hypertension-Related Factors within Custodial Settings of Southern Benin in 2023

Introduction: Hypertension, a non-communicable disease, is a major public health threat worldwide, accounting for a high level of morbidity and mortality. Although it has been extensively published among the general population, further research is needed to understand the reality of hypertension within the custodial setting. This study aimed to investigate the factors associated with arterial hypertension in custodial settings in southern Benin in 2023. Methods: This was a cross-sectional, descriptive, analytical study held in prisons in southern Benin from March to April 2023, involving inmates selected by two-stage random sampling. In the first stage, four prisons out of the six in the southern region of Benin were selected by simple random sampling. In the second stage, the prisoners were selected by systematic random sampling, with the sampling frame being the numbered list of eligible prisoners in each prison selected. Data collected by observation and questionnaire survey were analyzed using Stata 11 software. Hypertension was defined as systolic blood pressure ≥ 140 mmHg and diastolic blood pressure ≥ 90 mmHg. Overweight was defined by a body mass index (weight/height2 (kg/m2) ≥ 25. Factors associated with hypertension were identified by multiple logistic regression, at a 5% threshold of significance. Results: Altogether 336 inmates aged 37.55 ± 1.72 years were surveyed. The prevalence of hypertension in custodial settings in southern Benin in 2023 was 31.32% (95% CI [17.06;52.57]). Associated factors were inmate age (ORa = 3.36 95% CI: [1.94;5.85]) and abnormal waist circumference (ORa = 2.61 95% CI [1.27;5.40]). Conclusion: The prevalence of arterial hypertension in prisons of southern Benin (31.32%) is high when compared with the national average (25.9% (22.5-29.3)). The ministries of the Interior and Health need to collaborate to involve inmates in preventive strategies for non-communicable diseases, including hypertension.

Serum Calcium Ionised Rate and Materno-Perinatal Prognosis in Arterial Hypertension in Pregnancy at the Reference General Hospital of Panzi

Hypertensive disorders of pregnancy are among the leading causes of severe maternal morbidity and mortality, particularly in developing countries. Hypertensive disorders of pregnancy are among the leading causes of severe maternal morbidity and mortality, particularly in developing countries, maternal hypocalcaemia being a factor favouring the onset of arterial hypertension during pregnancy. The aim was to determine the maternal and perinatal prognosis of patients with hypertensive disorders of pregnancy as a function of serum ionised calcium levels. Material and Methods: A cross-sectional analytical study of 114 patients with arterial hypertension during pregnancy or during pregnancy or in the postpartum period at the HGR/Panzi from 1 January 2021 to 30 June 2022, text was entered using Microsoft Office Word 2010 and the tables were analysed using Excel 2010. The data was analysed using SPSS version 20.0 and Stata 14.0. The associations of the variables were calculated using Pearson's chi-square test, with a significance threshold set at a value of p < 0.05. Study of risk factors, Odds ratios and their confidence intervals were estimated in a univariate analysis. The most determining factors were identified by multivariate analysis using the Forward conditional logistic regression model. Results: The mean gestational age was 34.43 ± 4.327 amenorheas weeks, 46.6% of patients had a vaginal delivery, 66.65% of which were indicated for maternal prognosis, maternal complications were associated with maternal hypocalcaemia in 81, 82% (P = 0.043) and an OR = 3.255 (P = 0.0158) threefold risk that the patient presenting with a complication is likely to be in a state of hypocalcaemia at 95% confidence index, and fetal prognosis was not significantly related to maternal calcaemia. Conclusion: Maternal hypocalcaemia is one of the factors that can influence maternal-foetal complications maternal-fetal complications, early management and prevention of this pathology is pathology is important to reduce maternal-fetal morbidity and mortality.

Anomalous course of the carotid arteries in the retrooesophageal space: An unusual cause of chest pain

The differential diagnosis of chest pain, one of the most frequent symptoms referred in the emergency department, includes cardiac and noncardiac causes and represents a difficult challenge for clinicians. Noncardiac chest pain can be defined as the recurrence of chest pain episodes indistinguishable from coronary events after the exclusion of a cardiac cause. It may be of musculoskeletal, pulmonary, gastroenterological, psychosomatic or neurological aetiology, oesophageal disorders representing the most common detectable cause. In this report we describe a rare case of chest pain which appeared after dinner, due to an oesophageal spasm caused by the anomalous course of the carotid arteries. The case is relevant because it describes a very uncommon variant of the physiological course of the carotid arteries, and because it is unusual that a retro thoracic vascular abnormality can be related to the appearance of chest pain without dysphagia. Chest pain which appears after dinner in our case is probably due to the unusual anatomic connection between oesophagus and carotid arteries that probably begins to make stronger when the patient undertakes the supine position: for that reason nocturnal chest pain promptly relieves after the assumption of orthostatic or semi orthostatic decubitus. In conclusion, in a patient who refers the sudden appearance of nocturnal chest pain certainly unrelated to coronary artery disease, the physicians should consider the involvement of oesophageal system, maybe determined by vascular course abnormalities.

Pulmonary arterial hypertension confirmed by right heart catheterization following COVID-19 pneumonia: A case report and review of literature

BACKGROUND Pulmonary arterial hypertension(PAH)is a disease of the arterioles resulting in an increased resistance in pulmonary circulation with associated high pressures in the pulmonary arteries,causing irreversible remodeling of the pulmonary arterial walls.Coronavirus disease 2019(COVID-19)has been associated with development of new onset PAH in the literature leading to symptoms of dyspnea,cough and fatigue that persist in spite of resolution of acute COVID-19 infection.However,the majority of these cases of COVID related PAH were diagnosed using echocardiographic data or via right heart catheterization in mechanically ventilated patients.CASE SUMMARY Our case is the first reported case of COVID related PAH diagnosed by right heart catheterization in a non-mechanically ventilated patient.Right heart catheterization has been the gold standard for diagnosis of pulmonary hypertension.Our patient had right heart catheterization four months after her initial COVID-19 infection due to persistent dyspnea.CONCLUSION This revealed new onset PAH that developed following her infection with COVID-19,an emerging sequela of the infection.

Genetic analyses in a cohort of 191 Chinese pulmonarv arterial hypertension patients

Objective Pulmonary arterial hypertension(PAH)is mainly characterized by pulmonary artery obstruction,which is diagnosed by a mean pulmonary artery pressure≥25 mm Hg at rest,and excluding other known causes of pulmonary hypertension.To identify genetic mutations and help make a precise diagnosis,we performed genetic testing in 191 probands with invasively confirmed PAH and tried to analyze the genotype-phenotype correlation.

Pulmonary hypertension secondary to seronegative rheumatoid arthritis overlapping antisynthetase syndrome:A case report

BACKGROUND Polyarthritis is the most frequent clinical manifestation in antisynthetase syndrome(ASS)forms of idiopathic inflammatory myositis and may be misdiagnosed as rheumatoid arthritis(RA),particularly in patients with seronegative RA(SNRA).It is unclear whether there is an overlap between ASS and RA,or if ASS sometimes mimics RA.Pulmonary hypertension(PAH)is common in connective tissue diseases(CTDs).However,published reports on CTD-PAH do not include overlapping CTDs,and its incidence and impact on patient prognosis are unclear.CASE SUMMARY We report the case of a 63-year-old woman who presented with a 3-mo history of symptom aggravation of recurrent symmetrical joint swelling and pain that had persisted for over 10 years.The patient was diagnosed with RA and interstitial lung disease.The patient repeatedly presented to the hospital’s respiratory and rhe-umatology departments with arthralgia,plus shortness of breath after activity.Relevant tests indicated that anti-CCP and RF remained negative,while anti-J0-1 and anti-Ro-52 were strongly positive.It was not until recently that we recognized that this could be an unusual case of SNRA with concurrent ASS.Joint pain was relieved after regular anti-rheumatic treatment.Chest computed tomography scans showed that pulmonary interstitial changes did not progress significantly over several years;however,they showed gradual widening of the pulmonary artery,and cardiac ultrasound indicated elevated pulmonary artery systolic pressure.The prescribed treatment of PAH was not effective in improving shortness of breath.CONCLUSION Overlap of RA and ASS may be missed.Further research is necessary to facilitate early diagnosis,effective evaluation,and prognosis.

Abnormal splenic artery diameter/hepatic artery diameter ratio in cirrhosis-induced portal hypertension

AIM:To determine an optimal cutoff value for abnormal splenic artery diameter/proper hepatic artery diameter(S/P) ratio in cirrhosis-induced portal hypertension.METHODS:Patients with cirrhosis and portal hypertension(n = 770) and healthy volunteers(n = 31) underwent volumetric computed tomography threedimensional vascular reconstruction to measure the internal diameters of the splenic artery and proper hepatic artery to calculate the S/P ratio.The cutoff value for abnormal S/P ratio was determined using receiver operating characteristic curve analysis,and the prevalence of abnormal S/P ratio and associations between abnormal S/P ratio and major complications of portal hypertension were studied using logistic regression.RESULTS:The receiver operating characteristic analysis showed that the cutoff points for abnormal splenic artery internal diameter and S/P ratio were > 5.19 mm and > 1.40,respectively.The sensitivity,specificity,positive predictive value,and negative predictive value were 74.2%,45.2%,97.1%,and 6.6%,respectively.The prevalence of an abnormal S/P ratio in the patients with cirrhosis and portal hypertension was 83.4%.Patients with a higher S/P ratio had a lower risk of developing ascites [odds ratio(OR) = 0.708,95%CI:0.508-0.986,P = 0.041] and a higher risk of developing esophageal and gastric varices(OR = 1.483,95%CI:1.010-2.175,P = 0.044) and forming collateral circulation(OR = 1.518,95%CI:1.033-2.230,P = 0.034).After splenectomy,the portal venous pressure and maximum and mean portal venous flow velocities were reduced,while the flow rate and maximum and minimum flow velocities of the hepatic artery were increased(P < 0.05).CONCLUSION:The prevalence of an abnormal S/P ratio is high in patients with cirrhosis and portal hypertension,and it can be used as an important marker of splanchnic hemodynamic disturbances.

Contribution of oxidative stress to pulmonary arterial hypertension

Recent data implicate oxidative stress as a mediator of pulmonary hypertension (PH) and of the associated pathological changes to the pulmonary vasculature and right ventricle (RV). Increases in reactive oxygen species (ROS), altered redox state, and elevated oxidant stress have been demonstrated in the lungs and RV of several animal models of PH, including chronic hypoxia, monocrotaline toxicity, caveolin-1 knock-out mouse, and the transgenic Ren2 rat which overexpresses the mouse renin gene. Generation of ROS in these models is derived mostly from the activities of the nicotinamide adenine dinucleotide phosphate oxidases, xanthine oxidase, and uncoupled endothelial nitric oxide synthase. As disease progresses circulating monocytes and bone marrow-derived monocytic progenitor cells are attracted to and accumulate in the pulmonary vasculature. Once established, these inflammatory cells generate ROS and secrete mitogenic and fibrogenic cytokines that induce cell proliferation and fibrosis in the vascular wall resulting in progressive vascular remodeling. Deficiencies in antioxidant enzymes also contribute to pulmonary hypertensive states. Current therapies were developed to improve endothelial function, reduce pulmonary artery pressure, and slow the progression of vascular remodeling in the pulmonary vasculature by targeting deficiencies in either NO (PDE-type 5 inhibition) or PGI 2 (prostacyclin analogs), or excessive synthesis of ET-1 (ET receptor blockers) with the intent to improve patient clinical status and survival. New therapies may slow disease progression to some extent, but long term management has not been achieved and mortality is still high. Although little is known concerning the effects of current pulmonary arterial hypertension treatments on RV structure and function, interest in this area is increasing. Development of therapeutic strategies that simultaneously target pathology in the pulmonary vasculature and RV may be beneficial in reducing mortality associated with RV failure.