scholarly journals Single Stage Endovascular Treatment of a Type 2 Abernethy Malformation: Successful Nonsurgical Outcome in a Case Report

2015 ◽  
Vol 2015 ◽  
pp. 1-3 ◽  
Author(s):  
Carl Kraus ◽  
Vladimir Sheynzon ◽  
Robert Hanna ◽  
Joshua Weintraub

Abernethy malformations are a rare collection of congenital hepatic portosystemic shunts. Our 19-year-old patient is with a type 2 Abernethy malformation elected permanent shunt closure following worsening dyspnea. This report details a single stage endovascular technique wherein shunt closure was achieved immediately by placement of an aortic endograft. At 5-month follow-up, the patient reported decreased shortness of breath. Furthermore, ultrasound investigation demonstrated a patent portal vein and right heart catheterization 6 months after procedure revealed decreased pulmonary hypertension relative to preprocedure values. This one step method may serve as an alternative treatment strategy to multistage endovascular closure techniques of type 2 Abernethy malformations.

2020 ◽  
Vol 11 ◽  
Author(s):  
Jean Pierre Sibomana ◽  
Aloma Campeche ◽  
Roberto J. Carvalho-Filho ◽  
Ricardo Amorim Correa ◽  
Helena Duani ◽  
...  

Pulmonary arterial hypertension (PAH) is a disease of the lung blood vessels that results in right heart failure. PAH is thought to occur in about 5% to 10% of patients with hepatosplenic schistosomiasis, particularly due to S. mansoni. The lung blood vessel injury may result from a combination of embolization of eggs through portocaval shunts into the lungs causing localized Type 2 inflammatory response and vessel remodeling, triggering of autonomous pathology that becomes independent of the antigen, and high cardiac output as seen in portopulmonary hypertension. The condition is likely underdiagnosed as there is little systematic screening, and risk factors for developing PAH are not known. Screening is done by echocardiography, and formal diagnosis requires invasive right heart catheterization. Patients with Schistosoma-associated PAH show reduced functional capacity and can be treated with pulmonary vasodilators, which improves symptoms and may improve survival. There are animal models of this disease that might help in understanding disease pathogenesis and identify novel targets to screen and treatment. Pathogenic mechanisms include Type 2 immunity and activation and signaling in the TGF-β pathway. There are still major uncertainties regarding Schistosoma-associated PAH development, course and treatment.


2011 ◽  
Vol 38 (10) ◽  
pp. 2172-2179 ◽  
Author(s):  
MONIQUE HINCHCLIFF ◽  
ARYEH FISCHER ◽  
ELENA SCHIOPU ◽  
VIRGINIA D. STEEN

Objective.Pulmonary arterial hypertension (PAH) increases mortality in systemic sclerosis (SSc). The multicenter PHAROS registry (Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma) prospectively follows subjects with SSc at high risk for or with incident pulmonary hypertension (PH). We describe the registry design and baseline characteristics of subjects enrolled during the first 18 months since the start of the study.Methods.High-risk subjects are enrolled and classified as Pre-PAH if they have (1) carbon monoxide diffusing capacity (DLCO) < 55% predicted; (2) percentage of predicted forced vital capacity/DLCO ratio ≥ 1.6; or (3) an estimated right ventricular systolic pressure > 35 mm Hg on echocardiography. Subjects with right heart catheterization (RHC)-confirmed incident PH (mean pulmonary artery pressure ≥ 25 mm Hg within previous 6 months) are subclassified into PAH, pulmonary venous hypertension secondary to left-side heart disease (PVH), and PH due to interstitial lung disease (PH-ILD). Baseline and biannual demographic, clinical, and laboratory data and patient-reported health questionnaires are collected.Results.There are 237 subjects enrolled in PHAROS. The majority are white (73%) and women (87%). There are 166 Pre-PAH and 71 Definite PH subjects (49 PAH, 7 PVH, and 15 PH-ILD).Conclusion.PHAROS is the largest US and Canadian cohort of subjects with SSc at high risk for or with incident PAH. PAH-specific therapies are approved for 49/71 subjects with RHC-confirmed PAH. Analyses of PHAROS registry data will permit identification of risk factors for development of PAH among SSc patients at high risk for PAH and enhance understanding of the course of SSc-PAH.


Pneumologie ◽  
2015 ◽  
Vol 69 (05) ◽  
Author(s):  
V Foris ◽  
G Kovacs ◽  
P Douschan ◽  
X Kqiku ◽  
C Hesse ◽  
...  

1968 ◽  
Vol 07 (02) ◽  
pp. 125-129
Author(s):  
J. Měštan ◽  
V. Aschenbrenner ◽  
A. Michaljanič

SummaryIn patients with acquired and congenital valvular heart disease correlations of the parameters of the radiocardiographic curve (filling time of the right heart, minimal pulmonary transit time, peak-to-peak pulmonary transit time, and the so-called filling time of the left heart) with the mean pulmonary artery pressure and the mean pulmonary “capillary” pressure were studied. Further, a regression equation was determined by means of which the mean pulmonary “capillary” pressure can be predicted.


Diabetes ◽  
2018 ◽  
Vol 67 (Supplement 1) ◽  
pp. 1096-P
Author(s):  
RUTH E. BROWN ◽  
ALEXANDER ABITBOL ◽  
HARPREET S. BAJAJ ◽  
HASNAIN KHANDWALA ◽  
RONALD GOLDENBERG ◽  
...  

Diabetes ◽  
2019 ◽  
Vol 68 (Supplement 1) ◽  
pp. 2327-PUB ◽  
Author(s):  
ANN A. VERHAEGEN ◽  
ANDRÉ SCHEEN ◽  
KATHY C. ALEXANDRE ◽  
JACQUES BRUHWYLER ◽  
IDES M. COLIN

2021 ◽  
Vol 10 (Supplement_1) ◽  
Author(s):  
MD Lyhne ◽  
SJ Dragsbaek ◽  
JV Hansen ◽  
JG Schultz ◽  
A Andersen ◽  
...  

Abstract Funding Acknowledgements Type of funding sources: Foundation. Main funding source(s): Laerdal Foundation for Acute Medicine, Novo Nordisk Foundation Background/Introduction: Acute pulmonary embolism (PE) is a frequent condition in acute cardiac care and is potentially fatal. Cause of death is right ventricular (RV) failure due to increased RV afterload from both pulmonary vascular obstruction and vasoconstriction. Inodilators are interesting drugs of choice as they may improve RV function and lower its afterload. Purpose We aimed to investigate the cardiovascular effects of three clinically relevant inodilators: levosimendan, milrinone and dobutamine in acute PE. Methods We conducted a randomized, blinded, animal study using 18 female pigs. Animals received large autologous PE until doubling of baseline mean pulmonary arterial pressure and were randomized to four logarithmically increasing doses of each inodilator. Effects were evaluated with bi-ventricular pressure-volume loop recordings, right heart catheterization and blood gas analyses. Results Induction of PE increased RV afterload and pulmonary pressure (p &lt; 0.05) causing RV dysfunction. Levosimendan and milrinone showed beneficial hemodynamic profiles by lowering RV pressures and volume (p &lt; 0.001) and improved RV function and cardiac output (p &lt; 0.05) without increasing RV mechanical work. Dobutamine increased RV pressure and function (p &lt; 0.01) but at a cost of increased mechanical work at the highest doses, showing an adverse hemodynamic profile. See Figure. Conclusion(s): In a porcine model of acute PE, levosimendan and milrinone reduced RV afterload and improved RV function, whereas dobutamine at higher doses increased RV afterload and RV mechanical work. The study motivates clinical testing of inodilators in patients with acute PE and RV dysfunction. Abstract Figure. Inodilators in acute pulmonary embolism


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1063.2-1063
Author(s):  
J. Wang ◽  
Y. Feng ◽  
Y. Lei ◽  
X. Zhang

Background:Based on the characteristics of systemic lupus erythematosus-associated pulmonary arterial hypertension (SLE-PAH), Sunet alhas put forward a scoring system to distinguish two clinical phenotypes as vasculitic and vasculopathic subtypes[1]. A weighted score ≥2 suggested a vasculitic subtype by combining two factors: The time interval between SLE and PAH diagnosis <2 years and ≥2 years were 1 and 0 point; SLE Disease Activity Index (SLEDAI) >9, 5-9 and <5 were 2, 1, 0 point, respectively. While the vasculitic subtype seemed to have poorer prognosis in Sun’s research, other study has shown controversial result[2].Objectives:To find out the prognosis of two distinct clinical phenotypes of SLE-PAH.Methods:Between 2008 and 2019, a SLE-PAH cohort confirmed by right heart catheterization (RHC) from Guangdong Provincial People’s Hospital was included. Other groups of pulmonary hypertension were excluded. Based on the scoring system, patients were divided into vasculitic (weighted score≥2) and vasculopathic subtypes (weighted score<2). The endpoint was PAH-related mortality. Survival status were confirmed by clinic follow-up data or phone call.Results:A total of 53 SLE-PAH patients were enrolled. The cases of vasculitic and vasculopathic subtype were 14 and 39, respectively. Ten endpoint events occurred. Eight attributed to PAH and the cause could not be traced in two which were still included in study. The pooled 1-, 3-, 5-year survival rates were 85.7%, 78.6%, 65.5% in vasculitic subtype, and 93.9%, 87.5%, 87.5% in vasculopathic subtype, respectively. Kaplan-Meier analysis showed vasculitic subtype tended to have a poorer prognosis than vasculopathic subtype (p=0.16, HR 2.4, 95%CI 0.5-13.8, figure 1).Figure 1.Survival curves for patients with systemic lupus erythematosus-pulmonary arterial hypertension (SLE-PAH) in two distinct subtypes. RHC, Right Heart Catheterization.Conclusion:The prognosis of the two phenotypes of SLE-PAH was statistically indifferent while the vasculitic subtype showed a trend of worse prognosis. Further studies are needed.References:[1]F. Sun, Y. Lei, W. Wu, L. Guo, K. Wang, Z. Chen, W. Xu, X. Wang, T. Li, X. Zhang, S. Ye, Two distinct clinical phenotypes of pulmonary arterial hypertension secondary to systemic lupus erythematosus, Ann Rheum Dis 78(1) (2019) 148-150.[2]J. Qian, M. Li, J. Zhao, Q. Wang, Z. Tian, X. Zeng, Inflammation in SLE-PAH: good news or not?, Ann Rheum Dis (2018).0:1–2. doi:10.1136/annrheumdis-2018-214605Disclosure of Interests:None declared


2021 ◽  
Vol 49 (3) ◽  
pp. 030006052110023
Author(s):  
Yang Huang ◽  
Zuo-Gang Wang ◽  
Liang Tang ◽  
Su-Gang Gong ◽  
Yuan-Yuan Sun ◽  
...  

Objective To determine if plasma exosomal microRNAs (miRNAs) can predict survival in patients with idiopathic pulmonary arterial hypertension (IPAH). Methods The study enrolled patients with IPAH that underwent right heart catheterization. Plasma was collected and exosomal miRNAs were extracted. Exosomes were evaluated using transmission electron microscopy, Western blot analysis and particle size distribution analysis. MiRNAs were evaluated using a miRNA microarray and validated using real-time polymerase chain reaction. Results This study included 12 patients with IPAH in the study group and 48 patients with IPAH in the validation group. The mean ± SD follow-up duration was 60.3 ± 35.4 months in the overall cohort. The levels of miR-596 were higher in the nonsurvivors compared with the survivors. The levels of miR-596 significantly correlated with survival time, mean right atrial pressure, pulmonary vascular resistance (PVR) and cardiac index. High levels of miR-596 and PVR were significantly associated with poor overall survival. Multivariate analysis demonstrated that exosomal miR-596 (hazard ratio [HR] = 2.119; 95% confidence interval [CI] 1.402, 3.203) and PVR (HR = 1.146; 95% CI 1.010, 1.300) were independent predictors of survival. Conclusions High levels of plasma exosomal miR-596 were significantly associated with disease severity and poor prognosis of patients with IPAH.


BMJ Open ◽  
2021 ◽  
Vol 11 (3) ◽  
pp. e044888
Author(s):  
Rita McMorrow ◽  
Barbara Hunter ◽  
Christel Hendrieckx ◽  
Dominika Kwasnicka ◽  
Leanne Cussen ◽  
...  

IntroductionType 2 diabetes is a global health priority. People with diabetes are more likely to experience mental health problems relative to people without diabetes. Diabetes guidelines recommend assessment of depression and diabetes distress during diabetes care. This systematic review will examine the effect of routinely assessing and addressing depression and diabetes distress using patient-reported outcome measures in improving outcomes among adults with type 2 diabetes.Methods and analysisMEDLINE, Embase, CINAHL Complete, PsycInfo, The Cochrane Library and Cochrane Central Register of Controlled Trials will be searched using a prespecified strategy using a prespecified Population, Intervention, Comparator, Outcomes, Setting and study design strategy. The date range of the search of all databases will be from inception to 3 August 2020. Randomised controlled trials, interrupted time-series studies, prospective and retrospective cohort studies, case–control studies and analytical cross-sectional studies published in peer-reviewed journals in the English language will be included. Two review authors will independently screen abstracts and full texts with disagreements resolved by a third reviewer, if required, using Covidence software. Two reviewers will undertake risk of bias assessment using checklists appropriate to study design. Data will be extracted using prespecified template. A narrative synthesis will be conducted, with a meta-analysis, if appropriate.Ethics and disseminationEthics approval is not required for this review of published studies. Presentation of results will follow the Preferred Reporting Items for Systematic reviews and Meta-Analyses guidance. Findings will be disseminated via peer-reviewed publication and conference presentations.PROSPERO registration numberCRD42020200246.


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