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Vascular Pharmacology Nov 2018A systematic review and meta-analysis was performed to test candidate therapeutic approaches in pulmonary hypertension (PH). The efficacy of 522 interventions with >200... (Meta-Analysis)
Meta-Analysis Review
A systematic review and meta-analysis was performed to test candidate therapeutic approaches in pulmonary hypertension (PH). The efficacy of 522 interventions with >200 unregistered drugs was tested on 7254 animals. We propose a modified formula to assess meta-data that concerns the contribution of PH animal model to the denoted efficacy of tested agents. The measure of efficacy expressed as a response ratio for right ventricle systolic pressure was 0.48 (95% CI, 0.46-0.50; P < 0.00001), mean pulmonary artery pressure was 0.54 (0.52-0.56; P < 0.00001), right ventricle hypertrophy was 0.49 (0.48-0.51; P < 0.00001) and pulmonary artery wall thickness was 0.58 (0.56-0.61; P < 0.00001). Only 41 out of 522 interventions were ineffective. The most potent agents to improve both haemodynamic and hypertrophic parameters were ATP-sensitive potassium channel openers with iptakalim, Rho/ROCK inhibitors with fasudil, RAAS regulators with adenosine and ACE2 activators, and anti-inflammatories with n-3 polyunsaturated fatty acids and NF-кB inhibitors.
Topics: Animals; Antihypertensive Agents; Arterial Pressure; Disease Models, Animal; Hypertension, Pulmonary; Hypertrophy, Right Ventricular; Molecular Targeted Therapy; Pulmonary Artery; Signal Transduction; Vascular Remodeling; Ventricular Function, Right; Ventricular Remodeling
PubMed: 30145225
DOI: 10.1016/j.vph.2018.08.004 -
Congenital Heart Disease 2016Pulmonary artery (PA) aneurysms are rare and their related complications like dissection or rupture have been so far reported in a few reports, and a systematic... (Review)
Review
BACKGROUND
Pulmonary artery (PA) aneurysms are rare and their related complications like dissection or rupture have been so far reported in a few reports, and a systematic description of the disease is lacking. To identify patients with PA aneurysm, at high-risk for complications, is critical. We performed a systematic review of the literature to determine characteristics that could identify high-risk patients.
METHOD
A systematic search strategy was established and executed in Pubmed, Embase, Cochrane Central Register of Controlled Trials and Google scholar. Case reports were included if a minimal set of data were described.
RESULTS
After executing the search strategy and exclusion of non-relevant or duplicate articles, 38 original articles, reviews and 169 case reports could be included. Articles were classified in high and low-pressure PA aneurysms and subdivided in six groups on basis of the causative mechanisms. PA dilatation was most common in association with pulmonary hypertension, but only one dissection was reported in 6 original articles containing 153 patients. Analysis of the case reports suggests that predictors of high-risk patients are: pulmonary hypertension in congenital heart disease, fast PA diameter growth (>2 mm/year), tissue weakness due to infection and possibly pregnancy especially in combination. Except for 2 cases, PA dissection did not occur, when the PA diameter was <75 mm and the PA pressure <50 mmHg.
CONCLUSION
High-risk PA aneurysms maybe identified by evaluating: the causative mechanism(s) for PA dilatation, absolute PA diameter and growth rate and by evaluating the PA systolic pressure.
Topics: Aneurysm; Diagnostic Imaging; Global Health; Humans; Hypertension, Pulmonary; Periodicals as Topic; Prevalence; Pulmonary Artery
PubMed: 26555132
DOI: 10.1111/chd.12316 -
The Cochrane Database of Systematic... Jan 2017Individuals with pulmonary hypertension (PH) have reduced exercise capacity and quality of life. Despite initial concerns that exercise training may worsen symptoms in... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Individuals with pulmonary hypertension (PH) have reduced exercise capacity and quality of life. Despite initial concerns that exercise training may worsen symptoms in this group, several studies have reported improvements in functional capacity and well-being following exercise-based rehabilitation in PH.
OBJECTIVES
To assess the efficacy and safety of exercise-based rehabilitation for people with PH. Primary outcomes were exercise capacity, adverse events during the intervention period and health-related quality of life (HRQoL). Secondary outcomes included cardiopulmonary haemodynamics, functional class, clinical worsening during follow-up, mortality and changes in B-type natriuretic peptide.
SEARCH METHODS
We searched the Cochrane Airways Specialised Register of Trials up to August 2016, which is based on regular searches of CINAHL, AMED, Embase, PubMed, MEDLINE, PsycINFO and registries of clinical trials. In addition we searched CENTRAL and the PEDro database up to August 2016 and handsearched relevant journals.
SELECTION CRITERIA
All randomised controlled trials (RCTs) focusing on exercise-based rehabilitation programmes for PH.
DATA COLLECTION AND ANALYSIS
Two reviewers extracted data independently. For binary outcomes, we calculated odds ratios and their 95% confidence interval (CI), on an intention-to-treat basis. For continuous data, we estimated the mean difference (MD) between groups and its 95% CI. We employed a random-effects model for analyses. We assessed risk of bias for included studies and created 'Summary of findings' tables using GRADE.
MAIN RESULTS
We included six RCTs and were able to extract data from five studies. The majority of participants were Group I pulmonary artery hypertension (PAH). Study duration ranged from three to 15 weeks. Exercise programmes included both inpatient- and outpatient-based rehabilitation that incorporated both upper and lower limb exercise. The mean six-minute walk distance following exercise training was 60.12 metres higher than control (30.17 to 90.07 metres, n = 165, 5 RCTs, low-quality evidence; minimal important difference was 30 metres), the mean peak oxygen uptake was 2.4 ml/kg/minute higher (1.4 to 3.4 ml/kg/min, n = 145, 4 RCTs, low-quality evidence) and the mean peak power in the intervention groups was 16.4 W higher (10.9 to 22.0 higher, n = 145, 4 RCTs, low-quality evidence). The mean change in HRQoL for the SF-36 physical component score was 4.63 points higher (0.80 to 8.47 points, n = 33, 2 RCTs, low-quality evidence) and for the SF-36 mental component score was 4.17 points higher (0.01 to 8.34 points; n = 33; 2 RCTs, low-quality evidence). One study reported a single adverse event, where a participant stopped exercise training due to lightheadedness.
AUTHORS' CONCLUSIONS
In people with PH, exercise-based rehabilitation results in clinically relevant improvements in exercise capacity. Exercise training was not associated with any serious adverse events. Whilst most studies reported improvements in HRQoL, these may not be clinically important. Overall, we assessed the quality of the evidence to be low. The small number of studies and lack of information on participant selection makes it difficult to generalise these results across the spectrum of people with PH.
Topics: Exercise Therapy; Exercise Tolerance; Hemodynamics; Humans; Hypertension, Pulmonary; Middle Aged; Oxygen Consumption; Quality of Life; Randomized Controlled Trials as Topic; Selection Bias; Walk Test
PubMed: 28099988
DOI: 10.1002/14651858.CD011285.pub2 -
European Spine Journal : Official... Jul 2022Systematic review. (Review)
Review
STUDY DESIGN
Systematic review.
PURPOSE
Adolescent idiopathic scoliosis (AIS) is a deformity of the trunk and chest and can cause a spectrum of pulmonary symptoms. However, no standardized measurement instrument exists. The aim of this systematic review is to identify and describe patient-reported and clinical measurement instruments used to evaluate pulmonary symptoms in patients with AIS.
METHODS
Studies published after 01.01.2000 were included in a systematic search. Patient-reported outcome measures (PROMs) and clinical measurement instruments for pulmonary symptoms were extracted as well as their measurement properties (floor-ceiling effects, validity, reliability, responsivity and interpretability). The Risk of Bias (RoB) was evaluated.
RESULTS
Out of 3146 studies, 122 were eligible for inclusion. Seven clinical measurement instruments, measuring 50 measurement parameters, were identified. Five PROMs for pulmonary symptoms were identified. Studies assessing the quality of measurement properties in the AIS population were not identified. As such, the RoB could not be determined.
CONCLUSION
No available adequate patent centric instruments were identified that measure pulmonary functioning and symptoms. Although clinical measurement instruments are regularly used, their use in routine practice does not seem feasible. The measurement properties of some identified PROMs seem promising; however, they have not been validated in an AIS population. As pulmonary symptoms in patients with AIS are still poorly understood, the development of such a construct and potentially a subsequent PROM to routinely measure pulmonary functioning and patient experience is recommended.
Topics: Adolescent; Humans; Kyphosis; Patient Reported Outcome Measures; Quality of Life; Reproducibility of Results; Scoliosis
PubMed: 35438343
DOI: 10.1007/s00586-022-07204-z -
EClinicalMedicine Aug 2023Right ventricular dysfunction (RVD) is associated with adverse outcomes of acute pulmonary embolism (PE). However, there are no studies describing the long-term,...
BACKGROUND
Right ventricular dysfunction (RVD) is associated with adverse outcomes of acute pulmonary embolism (PE). However, there are no studies describing the long-term, full-spectrum right ventricular parameters on morphology, pressure and function at certain follow-up time points after PE onset. More exploration of right ventricular function would provide useful clues for long-term management of patients with PE.
METHODS
For this systematic review and meta-analysis, we completed a literature search in Pubmed, EMBASE and WebofScience (from Jan 1st, 1998 to April 20th, 2023). Studies of patients with acute PE followed-up longer than 3 months with right ventricle assessment and written in English-language were included. Right ventricular function was assessed by either echocardiography or computed tomographic pulmonary angiography (CTPA). The primary outcome was structural and functional parameters of the right ventricle, and the secondary outcomes were functional assessments [New York Heart Association (NYHA) functional classification and 6-min walk test distance (6 MWD)], at each follow-up time points. Random effect meta-analyses were performed using R software (PROSPERO: CRD42023433332).
FINDINGS
A total of 33 studies (3920 patients) were included in the final analysis. The 3-month, 6-month and 1-year prevalence of right ventricular dysfunction (RVD) was 0.34 [95% confidence interval (CI) 0.21-0.48, I = 96%], 0.26 (95% CI 0.17-0.36, I = 93%) and 0.34 (95% CI 0.19-0.48, I = 94%), respectively. Pooled tricuspid annulus plane systolic excursion (TAPSE), right ventricular to left ventricular diameter (RV/LV) ratio and pulmonary artery systolic pressure (PASP) at 1-year was 21.80 mm (95% CI 20.08-23.52, I = 93%), 0.64 (95% CI 0.48-0.81, I = 92%) and 27.33 mmHg (95% CI 18.88-35.78) (I = 96%), respectively. The proportion of NYHA III-IV was 0.06 (95% CI 0.0-0.12) and the pooled 6 MWD was 462.98 m (95% CI 447.55-478.41) over 1 year. Patients treated with thrombolysis had lower prevalence of RVD (1-year 0.17 and 0.07 in systemic thrombolysis and catheter-directed thrombolysis, respectively) than those treated with anticoagulation therapy alone (1-year 0.24) but the pooled risk ratio (RR) was not statistically significant.
INTERPRETATION
Although the conclusion of this study may be limited by its high heterogeneity from varied study designs, inclusion criteria and definition of RVD of each study, our findings suggested that persistent RVD and functional impairment were of considerable high prevalence during long-term follow-up after acute PE. Treatment strategy may influence the prevalence of long-term RVD.
FUNDING
This study is supported by CAMS Innovation Fund for Medical Sciences (CIFMS) (2021-I2M-1-061). The National Key Research and Development Program of China (2016YFC0905600). National High Level Hospital Clinical Research Funding (2022-NHLHCRF-LX-01-02-03). CAMS Institute of Respiratory Medicine Grant for Young Scholars (2023-ZF-8).
PubMed: 37638233
DOI: 10.1016/j.eclinm.2023.102153 -
JACC. Cardiovascular Interventions Apr 2023Balloon pulmonary angioplasty (BPA) was introduced as a treatment modality for patients with inoperable, medically refractory chronic thromboembolic pulmonary...
BACKGROUND
Balloon pulmonary angioplasty (BPA) was introduced as a treatment modality for patients with inoperable, medically refractory chronic thromboembolic pulmonary hypertension decades ago; however, reports of high rates of pulmonary vascular injury have led to considerable refinement in procedural technique.
OBJECTIVES
The authors sought to better understand the evolution of BPA procedure-related complications over time.
METHODS
The authors conducted a systematic review of original articles published by pulmonary hypertension centers globally and performed a pooled cohort analysis of procedure-related outcomes with BPA.
RESULTS
This systematic review identified 26 published articles from 18 countries worldwide from 2013 to 2022. A total of 1,714 patients underwent 7,561 total BPA procedures with an average follow up of 7.3 months. From the first period (2013-2017) to the second period (2018-2022), the cumulative incidence of hemoptysis/vascular injury decreased from 14.1% (474/3,351) to 7.7% (233/3,029) (P < 0.01); lung injury/reperfusion edema decreased from 11.3% (377/3,351) to 1.4% (57/3,943) (P < 0.01); invasive mechanical ventilation decreased from 0.7% (23/3,195) to 0.1% (4/3,062) (P < 0.01); and mortality decreased from 2.0% (13/636) to 0.8% (8/1,071) (P < 0.01).
CONCLUSIONS
Procedure-related complications with BPA, including hemoptysis/vascular injury, lung injury/reperfusion edema, mechanical ventilation, and death, were less common in the second period (2018-2022), compared with first period (2013-2017), likely from refinement in patient and lesion selection and procedural technique over time.
Topics: Humans; Pulmonary Artery; Hypertension, Pulmonary; Pulmonary Embolism; Hemoptysis; Lung Injury; Vascular System Injuries; Treatment Outcome; Angioplasty, Balloon; Pulmonary Edema; Edema; Chronic Disease
PubMed: 37100561
DOI: 10.1016/j.jcin.2023.01.361 -
Global Heart 2023This systematic review and meta-analysis aimed to determine the efficacy of macitentan in patients with pulmonary hypertension (PH). (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
This systematic review and meta-analysis aimed to determine the efficacy of macitentan in patients with pulmonary hypertension (PH).
METHODS
A systematic search was made of PubMed, Embase, Cochrane Library, and clinicaltrials.gov, without language restrictions. Randomized controlled trials (RCTs) on treatment of PH with macitentan, compared with placebo or blank, were reviewed. Studies were pooled to weighted mean differences (WMDs) and risk ratios (RRs), with 95% confidence intervals (CIs).
RESULTS
Six RCTs (enrolling 1,003 participants) met the inclusion criteria. Macitentan showed significant effects on 6-min walk distance (6MWD) (WMD 12.06 m, 95% CI 2.12 to 21.99 m), pulmonary vascular resistance (PVR) (WMD -186.51 dyn·s/cm, 95% CI -232.72 to -140.29 dyn·s/cm), mean pulmonary artery pressure (mPAP) (WMD -3.20 mmHg, 95% CI -5.93 to -0.47 mmHg), N-terminal pro-brain natriuretic peptide (NT-proBNP) (WMD -232.47 ng/L, 95% wCI -318.22 to -146.72 ng/L), and cardiac index (WMD 0.39 L/min/m, 95% CI 0.20 to 0.58 L/min/m).
CONCLUSION
Macitentan significantly improved 6MWD, PVR, mPAP, NT-proBNP, and cardiac index in patients with PH. Macitentan should be further validated in patients with PH.
Topics: Humans; Hypertension, Pulmonary; Treatment Outcome; Randomized Controlled Trials as Topic; Pyrimidines
PubMed: 37901601
DOI: 10.5334/gh.1274 -
Pediatric Research May 2019Congenital diaphragmatic hernia (CDH) is a severe birth defect that is characterized by pulmonary hypoplasia and pulmonary hypertension (PHTN). PHTN secondary to CDH is... (Review)
Review
Congenital diaphragmatic hernia (CDH) is a severe birth defect that is characterized by pulmonary hypoplasia and pulmonary hypertension (PHTN). PHTN secondary to CDH is a result of vascular remodeling, a structural alteration in the pulmonary vessel wall that occurs in the fetus. Factors involved in vascular remodeling have been reported in several studies, but their interactions remain unclear. To help understand PHTN pathophysiology and design novel preventative and treatment strategies, we have conducted a systematic review of the literature and comprehensively analyzed all factors and pathways involved in the pathogenesis of pulmonary vascular remodeling secondary to CDH in the nitrofen model. Moreover, we have linked the dysregulated factors with pathways involved in human CDH. Of the 358 full-text articles screened, 75 studies reported factors that play a critical role in vascular remodeling secondary to CDH. Overall, the impairment of epithelial homeostasis present in pulmonary hypoplasia results in altered signaling to endothelial cells, leading to endothelial dysfunction. This causes an impairment of the crosstalk between endothelial cells and pulmonary artery smooth muscle cells, resulting in increased smooth muscle cell proliferation, resistance to apoptosis, and vasoconstriction, which clinically translate into PHTN.
Topics: Animals; Animals, Newborn; Disease Models, Animal; Endothelial Cells; Female; Hernias, Diaphragmatic, Congenital; Humans; Hypertension, Pulmonary; Infant, Newborn; Myocytes, Smooth Muscle; Phenyl Ethers; Pregnancy; Pulmonary Artery; Rats; Risk Factors; Vascular Remodeling
PubMed: 30780153
DOI: 10.1038/s41390-019-0345-4 -
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 -
Heart & Lung : the Journal of Critical... 2020Aorto-cardiac fistulae are a rare but increasingly reported entity, and data are scarce. (Review)
Review
BACKGROUND
Aorto-cardiac fistulae are a rare but increasingly reported entity, and data are scarce.
METHOD
The authors performed a systematic review of ACFs to characterize the underlying etiology, clinical presentation, and compare outcomes of treatment strategies.
RESULTS
3,733 publications were identified in the search. Of those, 292 studies including 300 patients were included. Etiology of ACFs was 38% iatrogenic, 25% infectious, 14% traumatic, and 15% due to other causes. Most patients (74%) presented with heart failure. Common locations were aortic-right atrium (37%), and aortic-pulmonary artery (25%). The majority of patients (71%) were treated surgically, while 13% were treated percutaneously, and 16% were treated conservatively. Patients who were managed conservatively had a higher mortality than those treated with invasive closure (53% vs. 12% vs. 3%, p = <0.00001).
CONCLUSIONS
This systematic review sheds light on this highly morbid condition. Once recognized, fistula closure appears to be superior to conservative management.
Topics: Aortic Diseases; Fistula; Heart Atria; Humans; Pulmonary Artery; Vascular Fistula
PubMed: 31735456
DOI: 10.1016/j.hrtlng.2019.11.002