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Clinical Research in Cardiology :... Jul 2022Pulmonary arterial hypertension (PAH) can lead to left main coronary artery compression (LMCo), but data on the impact, screening and treatment are limited. A... (Meta-Analysis)
Meta-Analysis
OBJECTIVE
Pulmonary arterial hypertension (PAH) can lead to left main coronary artery compression (LMCo), but data on the impact, screening and treatment are limited. A meta-analysis of LMCo cases could fill the knowledge gaps in this topic.
METHODS
Electronic databases were searched for all LMCo/PAH studies, abstracts and case reports including pulmonary artery (PA) size. Restricted maximum likelihood meta-analysis was used to evaluate LMCo-associated factors. Specificity, sensitivity and accuracy of PA size thresholds for diagnosis of LMCo were calculated. Treatment options and outcomes were summarized.
RESULTS
A total of five case-control cohorts and 64 case reports/series (196 LMCo and 438 controls) were included. LMCo cases had higher PA diameter (Hedge's g 1.46 [1.09; 1.82]), PA/aorta ratio (Hedge's g 1.1 [0.64; 1.55]) and probability of CHD (log odds-ratio 1.22 [0.54; 1.9]) compared to non-LMCo, but not PA pressure or vascular resistance. A 40 mm cut-off for the PA diameter had balanced sensitivity (80.5%), specificity (79%) and accuracy (79.7%) for LMCo diagnosis, while a value of 44 mm had higher accuracy (81.7%), higher specificity (91.5%) but lower sensitivity (71.9%). Pooled mortality after non-conservative treatment (n = 150, predominantly stenting) was 2.7% at up to 22 months of mean follow-up, with 83% survivors having no angina at follow-up.
CONCLUSION
PA diameter, PA/aorta ratio and CHD are associated with LMCo, while hemodynamic parameters are not. Data from this study support that a PA diameter cut-off between 40 and 44 mm can offer optimal accuracy for LMCo screening. Preferred treatment was coronary stenting, associated with low mid-term mortality and symptom relief. Diagnosis and management of left main coronary artery compression (LMCo) in patients with pulmonary arterial hypertension (PAH).
Topics: Angina Pectoris; Coronary Vessels; Humans; Hypertension, Pulmonary; Pulmonary Arterial Hypertension; Pulmonary Artery
PubMed: 35290496
DOI: 10.1007/s00392-022-01999-z -
American Journal of Cardiovascular... Mar 2021This systematic review and meta-analysis was conducted to identify if long-term bosentan is an effective and safe treatment for pulmonary arterial hypertension (PAH)... (Meta-Analysis)
Meta-Analysis
OBJECTIVE
This systematic review and meta-analysis was conducted to identify if long-term bosentan is an effective and safe treatment for pulmonary arterial hypertension (PAH) regardless of type, including idiopathic PAH (IPAH), and PAH associated with congenital heart disease (APAH-CHD), connective tissue disease (APAH-CTD), and human immunodeficiency virus (APAH-HIV).
METHODS
All relevant observations were systematically searched by two independent investigators and obtained from three databases, including PubMed, EMBASE and the Cochrane Library, from the inception of each database to February 2020. Currently, long-term administration was defined as no less than 12 months. A random-effects or fixed-effects model was selected according to outcomes of the heterogeneity test for meta-analysis, where standardized mean difference (SMD) with 95% confidence intervals (CIs) was used for continuous outcomes, in addition to the estimated effect (ES; 95% CI) for the synthesized survival rate. Furthermore, subgroup analysis was applied to analyze the differences of efficacy and survivals in each type of PAH cohort.
RESULTS
Fifteen studies including a total of 659 subjects undergoing oral bosentan administration for at least 12 months were pooled in this quantitative review. Meta-analysis and subgroup analysis indicated that significant clinical benefits existed, including an improved 6-min walk distance (6MWD) and functional class (FC), in patients with APAH-CHD (6MWD: SMD 0.72, 95% CI 0.52-0.93, p < 0.0001; functional benefits: 50.4%, 95% CI 43.7-57.1%), APAH-HIV (6MWD: SMD 0.83, 95% CI 0.36-1.30, p = 0.001; functional benefits: 80.4%), and IPAH (SMD 0.54, 95% CI 0.28-0.80, p < 0.0001; functional benefits: 61.4%, 95% CI 54.2-68.5%), but a non-significant change in APAH-CTD (6MWD: SMD 0.18, 95% CI - 0.60 to 0.95, p = 0.656; functional benefits: 27.5%). Furthermore, among the hemodynamic parameters, long-term bosentan led to a significant decrease in mean pulmonary artery pressure (SMD - 0.86, p < 0.0001) in APAH-CTD, and a decrease in pulmonary vascular resistance (SMD - 0.65, p < 0.0001) and elevated oxygen saturation (SMD 0.30, p = 0.006) in APAH-CHD. Importantly, in all pooled studies, the overall survival indicated 1-, 2-, and 3-year survival rates of 94.3%, 88.8%, and 81.7%, respectively, in all-cause PAH, and subgroup analysis demonstrated a relative decreasing trend in patients with HIV, from a 2-year survival of 89.8% to a 3-year survival of 66.1%. Adverse drug reactions were relatively mild.
CONCLUSION
In this systematic review and meta-analysis, long-term administration of oral bosentan has been identified as a well-tolerated and effective agent in different types of PAH. In addition, we conclude that long-term oral bosentan should be considered for patients with CTD to achieve a satisfactory exercise capacity, and for those with APAH-HIV to improve survivals, where more attention on adverse events is required.
Topics: Antihypertensive Agents; Bosentan; Connective Tissue Diseases; Exercise; Familial Primary Pulmonary Hypertension; HIV Infections; Heart Defects, Congenital; Hemodynamics; Humans; Observational Studies as Topic; Pulmonary Arterial Hypertension
PubMed: 32918210
DOI: 10.1007/s40256-020-00426-w -
Circulation Mar 2007More than 140 million people worldwide live >2500 m above sea level. Of them, 80 million live in Asia, and 35 million live in the Andean mountains. This latter region... (Review)
Review
More than 140 million people worldwide live >2500 m above sea level. Of them, 80 million live in Asia, and 35 million live in the Andean mountains. This latter region has its major population density living above 3500 m. The primary objective of the present study is to review the physiology, pathology, pathogenesis, and clinical features of the heart and pulmonary circulation in healthy highlanders and patients with chronic mountain sickness. A systematic review of worldwide literature was undertaken, beginning with the pioneering work done in the Andes several decades ago. Original articles were analyzed in most cases and English abstracts or translations of articles written in Chinese were reviewed. Pulmonary hypertension in healthy highlanders is related to a delayed postnatal remodeling of the distal pulmonary arterial branches. The magnitude of pulmonary hypertension increases with the altitude level and the degree of exercise. There is reversal of pulmonary hypertension after prolonged residence at sea level. Chronic mountain sickness develops when the capacity for altitude adaptation is lost. These patients have moderate to severe pulmonary hypertension with accentuated hypoxemia and exaggerated polycythemia. The clinical picture of chronic mountain sickness differs from subacute mountain sickness and resembles other chronic altitude diseases described in China and Kyrgyzstan. The heart and pulmonary circulation in healthy highlanders have distinct features in comparison with residents at sea level. Chronic mountain sickness is a public health problem in the Andean mountains and other mountainous regions around the world. Therefore, dissemination of preventive and therapeutic measures is essential.
Topics: Adaptation, Physiological; Adolescent; Adult; Aged; Aged, 80 and over; Altitude; Altitude Sickness; Animals; Cardiac Catheterization; Cardiac Output; Cattle; Cattle Diseases; Child; Child, Preschool; China; Chronic Disease; Diagnosis, Differential; Exercise; Female; Heart Ventricles; Humans; Hypertension, Pulmonary; Hypertrophy, Right Ventricular; Hypoxia; Infant; Infant, Newborn; Latin America; Male; Middle Aged; Muscle, Smooth, Vascular; Polycythemia; Pulmonary Artery; Pulmonary Circulation; Pulmonary Heart Disease; Vasodilator Agents; Ventricular Function
PubMed: 17339571
DOI: 10.1161/CIRCULATIONAHA.106.624544 -
International Journal of Obstetric... Apr 2024Women with pulmonary hypertension (PH) have increased mortality during pregnancy and the peripartum period. An increasing number of publications suggest improvements in... (Review)
Review
Women with pulmonary hypertension (PH) have increased mortality during pregnancy and the peripartum period. An increasing number of publications suggest improvements in maternal outcomes, so we conducted a systematic review focusing on disease severity and maternal survival. After screening 9097 potential studies from 1967 to 2021, we identified 66 relevant publications. Outcomes improved continuously over time and mortality fell from 11.6% in studies published before 2015 to 8.2% in studies published after 2015. Mortality was lower in patients with mild disease (0.8%) than in those with Eisenmenger syndrome (26.2%) or idiopathic pulmonary arterial hypertension (7.4-24.0%). One major drawback of the published studies is that they define severity using echocardiographic-estimated pulmonary artery pressures, without considering more contemporary parameters. This systematic review provides new insights for preconception counseling on pregnancy risks related to PH and suggests that PH classification and severity should be carefully considered in determining an individual's pregnancy-associated risk.
PubMed: 38781778
DOI: 10.1016/j.ijoa.2024.104210 -
Vascular Medicine (London, England) Oct 2017The formation of a fistula between the internal mammary artery and the pulmonary vasculature (IMA-to-PV) is a rare anomaly. The etiology can be congenital; however, most... (Review)
Review
The formation of a fistula between the internal mammary artery and the pulmonary vasculature (IMA-to-PV) is a rare anomaly. The etiology can be congenital; however, most recent cases have been associated with coronary artery bypass grafting, trauma, inflammatory conditions, chronic infections, or neoplasia. The knowledge base on the formation of these fistulas is derived primarily from case reports. To our knowledge, no systematic reviews or guidelines are available that provide information on how to manage these cases, and the treatment of an IMA-to-PV fistula is controversial. To our knowledge, this report is the first to review 80 cases of IMA-to-PV fistulas reported in the literature. We describe the etiologies, clinical presentation, and management of these fistulas.
Topics: Adult; Arterio-Arterial Fistula; Arteriovenous Fistula; Female; Humans; Iatrogenic Disease; Male; Mammary Arteries; Middle Aged; Pulmonary Artery; Pulmonary Veins; Risk Factors; Treatment Outcome; Vascular System Injuries
PubMed: 28990495
DOI: 10.1177/1358863X17724262 -
The Cochrane Database of Systematic... Mar 2015Most ischaemic strokes are caused by a blood clot blocking an artery in the brain. Clot prevention with anticoagulants might improve outcomes if bleeding risks are low.... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Most ischaemic strokes are caused by a blood clot blocking an artery in the brain. Clot prevention with anticoagulants might improve outcomes if bleeding risks are low. This is an update of a Cochrane review first published in 1995, with recent updates in 2004 and 2008.
OBJECTIVES
To assess the effectiveness and safety of early anticoagulation (within the first 14 days of onset) in people with acute presumed or confirmed ischaemic stroke.
SEARCH METHODS
We searched the Cochrane Stroke Group Trials Register (June 2014), the Cochrane Central Register of Controlled Trials (CENTRAL), the Cochrane Database of Systematic Reviews (CDSR), the Database of Reviews of Effects (DARE) and the Health Technology Assessment Database (HTA) (The Cochrane Library 2014 Issue 6), MEDLINE (2008 to June 2014) and EMBASE (2008 to June 2014). In addition, we searched ongoing trials registries and reference lists of relevant papers. For previous versions of this review, we searched the register of the Antithrombotic Trialists' (ATT) Collaboration, consulted MedStrategy (1995), and contacted relevant drug companies.
SELECTION CRITERIA
Randomised trials comparing early anticoagulant therapy (started within two weeks of stroke onset) with control in people with acute presumed or confirmed ischaemic stroke.
DATA COLLECTION AND ANALYSIS
Two review authors independently selected trials for inclusion, assessed trial quality, and extracted the data.
MAIN RESULTS
We included 24 trials involving 23,748 participants. The quality of the trials varied considerably. The anticoagulants tested were standard unfractionated heparin, low-molecular-weight heparins, heparinoids, oral anticoagulants, and thrombin inhibitors. Over 90% of the evidence relates to the effects of anticoagulant therapy initiated within the first 48 hours of onset. Based on 11 trials (22,776 participants) there was no evidence that anticoagulant therapy started within the first 14 days of stroke onset reduced the odds of death from all causes (odds ratio (OR) 1.05; 95% confidence interval (CI) 0.98 to 1.12) at the end of follow-up. Similarly, based on eight trials (22,125 participants), there was no evidence that early anticoagulation reduced the odds of being dead or dependent at the end of follow-up (OR 0.99; 95% CI 0.93 to 1.04). Although early anticoagulant therapy was associated with fewer recurrent ischaemic strokes (OR 0.76; 95% CI 0.65 to 0.88), it was also associated with an increase in symptomatic intracranial haemorrhages (OR 2.55; 95% CI 1.95 to 3.33). Similarly, early anticoagulation reduced the frequency of symptomatic pulmonary emboli (OR 0.60; 95% CI 0.44 to 0.81), but this benefit was offset by an increase in extracranial haemorrhages (OR 2.99; 95% CI 2.24 to 3.99).
AUTHORS' CONCLUSIONS
Since the last version of the review, no new relevant studies have been published and so there is no additional information to change the conclusions. Early anticoagulant therapy is not associated with net short- or long-term benefit in people with acute ischaemic stroke. Treatment with anticoagulants reduced recurrent stroke, deep vein thrombosis and pulmonary embolism, but increased bleeding risk. The data do not support the routine use of any of the currently available anticoagulants in acute ischaemic stroke.
Topics: Anticoagulants; Brain Ischemia; Humans; Randomized Controlled Trials as Topic; Risk; Stroke
PubMed: 25764172
DOI: 10.1002/14651858.CD000024.pub4 -
Current Cardiology Reviews 2022Reversed Potts shunt has been a prospective approach to treat suprasystemic pulmonary hypertension, particularly when medication treatment fails to reduce right... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Reversed Potts shunt has been a prospective approach to treat suprasystemic pulmonary hypertension, particularly when medication treatment fails to reduce right ventricular afterload.
OBJECTIVE
This meta-analysis aims to review the clinical, laboratory, and hemodynamic parameters after a reversed Potts shunt in suprasystemic pulmonary hypertension patients.
METHODS
Six electronic databases were searched from the date of inception to August 2021, where the obtained studies were evaluated according to the PRISMA statement. The effects of shunt creation were evaluated by comparing preprocedural to postprocedural or follow-up parameters, expressed as a mean difference of 99% confidence interval. Quality assessment was conducted using the STROBE statement.
RESULTS
Seven studies suited the inclusion criteria which were included in this article. A reduction in upper and lower limb oxygen saturation [Upper limb: St. Mean difference -0.55, 99% CI -1.25 to 0.15; P=0.04; I=6%. Lower limb: St. Mean difference -4.45, 99% CI -7.37 to -1.52; P<0.00001; I=65%]. Reversed Potts shunt was shown to improve WHO functional class, 6-minute walk distance, NTpro-BNP level, and hemodynamic parameters including tricuspid annular plane systolic excursion, interventricular septal curvature, and end-diastolic right ventricle/left ventricle ratio.
CONCLUSION
Reversed Potts shunt cannot be said to be relatively safe, although it allows improvement in the clinical and functional status in patients with suprasystemic PAH. Reversed Potts shunt procedure may be the last resort for drug-resistant pulmonary hypertension as it is considered a high-risk procedure performed on patients with extremely poor conditions.
Topics: Humans; Hypertension, Pulmonary; Pulmonary Arterial Hypertension; Pulmonary Artery; Hemodynamics
PubMed: 35538823
DOI: 10.2174/1573403X18666220509203335 -
European Respiratory Review : An... Mar 2020Schistosomiasis-associated pulmonary arterial hypertension (Sch-PAH) is a life-threatening complication of chronic hepatosplenic schistosomiasis. It is suggested to be... (Meta-Analysis)
Meta-Analysis
Schistosomiasis-associated pulmonary arterial hypertension (Sch-PAH) is a life-threatening complication of chronic hepatosplenic schistosomiasis. It is suggested to be the leading cause of pulmonary arterial hypertension (PAH) worldwide. However, pathophysiological data on Sch-PAH are scarce. We examined the hypothesis that there are pronounced similarities in pathophysiology, haemodynamics, and survival of Sch-PAH and idiopathic PAH (iPAH).This systematic review and meta-analysis was registered in the PROSPERO database (identifier CRD42018104066). A systematic search and review of the literature was performed according to PRISMA guidelines for studies published between 01 January 1990 and 29 June 2018.For Sch-PAH, 18 studies evaluating pathophysiological mechanisms, eight studies on haemodynamics (n=277), and three studies on survival (n=191) were identified. 16 clinical registries reporting data on haemodynamics and survival including a total of 5792 patients with iPAH were included for comparison. Proinflammatory molecular pathways are involved in both Sch-PAH and iPAH. The transforming growth factor (TGF)-β signalling pathway is upregulated in Sch-PAH and iPAH. While there was no difference in mean pulmonary artery pressure (54±17 mmHg 55±15 mmHg, p=0.29), cardiac output (4.4±1.3 L·min 4.1±1.4 L·min, p=0.046), and cardiac index (2.6±0.7 L·min·m 2.3±0.8 L·min·m, p<0.001) were significantly higher in Sch-PAH compared to iPAH, resulting in a lower pulmonary vascular resistance in Sch-PAH (10±6 Woods units 13±7 Woods units, p<0.001). 1- and 3-year survival were significantly better in the Sch-PAH group (p<0.001).Sch-PAH and iPAH share common pathophysiological mechanisms related to inflammation and the TGF-β signalling pathway. Patients with Sch-PAH show a significantly better haemodynamic profile and survival than patients with iPAH.
Topics: Animals; Arterial Pressure; Familial Primary Pulmonary Hypertension; Humans; Prognosis; Pulmonary Arterial Hypertension; Pulmonary Artery; Risk Assessment; Risk Factors; Schistosomiasis
PubMed: 32024722
DOI: 10.1183/16000617.0089-2019 -
Annals of Intensive Care Apr 2020The echocardiography working group of the European Society of Intensive Care Medicine recognized the need to provide structured guidance for future CCE research... (Review)
Review
Systematic review and literature appraisal on methodology of conducting and reporting critical-care echocardiography studies: a report from the European Society of Intensive Care Medicine PRICES expert panel.
BACKGROUND
The echocardiography working group of the European Society of Intensive Care Medicine recognized the need to provide structured guidance for future CCE research methodology and reporting based on a systematic appraisal of the current literature. Here is reported this systematic appraisal.
METHODS
We conducted a systematic review, registered on the Prospero database. A total of 43 items of common interest to all echocardiography studies were initially listed by the experts, and other "topic-specific" items were separated into five main categories of interest (left ventricular systolic function, LVSF n = 15, right ventricular function, RVF n = 18, left ventricular diastolic function, LVDF n = 15, fluid management, FM n = 7, and advanced echocardiography techniques, AET n = 17). We evaluated the percentage of items reported per study and the fraction of studies reporting a single item.
RESULTS
From January 2000 till December 2017 a total of 209 articles were included after systematic search and screening, 97 for LVSF, 48 for RVF, 51 for LVDF, 36 for FM and 24 for AET. Shock and ARDS were relatively common among LVSF articles (both around 15%) while ARDS comprised 25% of RVF articles. Transthoracic echocardiography was the main echocardiography mode, in 87% of the articles for AET topic, followed by 81% for FM, 78% for LVDF, 70% for LVSF and 63% for RVF. The percentage of items per study as well as the fraction of study reporting an item was low or very low, except for FM. As an illustration, the left ventricular size was only reported by 56% of studies in the LVSF topic, and half studies assessing RVF reported data on pulmonary artery systolic pressure.
CONCLUSION
This analysis confirmed sub-optimal reporting of several items listed by an expert panel. The analysis will help the experts in the development of guidelines for CCE study design and reporting.
PubMed: 32335780
DOI: 10.1186/s13613-020-00662-y -
Clinical Toxicology (Philadelphia, Pa.) Sep 2018While pulmonary arterial hypertension remains an uncommon diagnosis, various therapeutic agents are recognized as important associations. These agents are typically...
INTRODUCTION
While pulmonary arterial hypertension remains an uncommon diagnosis, various therapeutic agents are recognized as important associations. These agents are typically categorized into "definite", "likely", "possible", or "unlikely" to cause pulmonary arterial hypertension, based on the strength of evidence.
OBJECTIVE
This review will focus on those therapeutic agents where there is sufficient literature to adequately comment on the role of the agent in the pathogenesis of pulmonary arterial hypertension.
METHODS
A systematic search was conducted using PubMed covering the period September 1970- 2017. The search term utilized was "drug induced pulmonary hypertension". This resulted in the identification of 853 peer-reviewed articles including case reports. Each paper was then reviewed by the authors for its relevance. The majority of these papers (599) were excluded as they related to systemic hypertension, chronic obstructive pulmonary disease, human immunodeficiency virus, pulmonary fibrosis, alternate differential diagnosis, treatment, basic science, adverse effects of treatment, and pulmonary hypertension secondary to pulmonary embolism. Agents affecting serotonin metabolism (and related anorexigens): Anorexigens, such as aminorex, fenfluramine, benfluorex, phenylpropanolamine, and dexfenfluramine were the first class of medications recognized to cause pulmonary arterial hypertension. Although most of these medications have now been withdrawn worldwide, they remain important not only from a historical perspective, but because their impact on serotonin metabolism remains relevant. Selective serotonin reuptake inhibitors, tryptophan, and lithium, which affect serotonin metabolism, have also been implicated in the development of pulmonary arterial hypertension. Interferon and related medications: Interferon alfa and sofosbuvir have been linked to the development of pulmonary arterial hypertension in patients with other risk factors, such as human immunodeficiency virus co-infection. Antiviral therapies: Sofosbuvir has been associated with two cases of pulmonary artery hypertension in patients with multiple risk factors for its development. Its role in pathogenesis remains unclear. Small molecule tyrosine kinase inhibitors: Small molecule tyrosine kinase inhibitors represent a relatively new class of medications. Of these dasatinib has the strongest evidence in drug-induced pulmonary arterial hypertension, considered a recognized cause. Nilotinib, ponatinib, carfilzomib, and ruxolitinib are newer agents, which paradoxically have been linked to both cause and treatment for pulmonary arterial hypertension. Monoclonal antibodies and immune regulating medications: Several case reports have linked some monoclonal antibodies and immune modulating therapies to pulmonary arterial hypertension. There are no large series documenting an increased prevalence of pulmonary arterial hypertension complicating these agents; nonetheless, trastuzumab emtansine, rituximab, bevacizumab, cyclosporine, and leflunomide have all been implicated in case reports. Opioids and substances of abuse: Buprenorphine and cocaine have been identified as potential causes of pulmonary arterial hypertension. The mechanism by which this occurs is unclear. Tramadol has been demonstrated to cause severe, transient, and reversible pulmonary hypertension. Chemotherapeutic agents: Alkylating and alkylating-like agents, such as bleomycin, cyclophosphamide, and mitomycin have increased the risk of pulmonary veno-occlusive disease, which may be clinically indistinct from pulmonary arterial hypertension. Thalidomide and paclitaxel have also been implicated as potential causes. Miscellaneous medications: Protamine appears to be able to cause acute, reversible pulmonary hypertension when bound to heparin. Amiodarone is also capable of causing pulmonary hypertension by way of recognized side effects.
CONCLUSIONS
Pulmonary arterial hypertension remains a rare diagnosis, with drug-induced causes even more uncommon, accounting for only 10.5% of cases in large registry series. Despite several agents being implicated in the development of PAH, the supportive evidence is typically limited, based on case series and observational data. Furthermore, even in the drugs with relatively strong associations, factors that predispose an individual to PAH have yet to be elucidated.
Topics: Drug-Related Side Effects and Adverse Reactions; Humans; Hypertension, Pulmonary; Muscle, Smooth, Vascular; Prescription Drugs
PubMed: 29508628
DOI: 10.1080/15563650.2018.1447119