scholarly journals Targeting Alcohol Septal Ablation in Patients with Obstructive Hypertrophic Cardiomyopathy Candidates for Surgical Myectomy: Added Value of Three-Dimensional Intracoronary Myocardial Contrast Echocardiography

2021 ◽  
Vol 10 (10) ◽  
pp. 2166
Author(s):  
Giovanni La La Canna ◽  
Iside Scarfò ◽  
Irina Arendar ◽  
Antonio Colombo ◽  
Lucia Torracca ◽  
...  
Keyword(s):  
High Risk ◽  
Three Dimensional ◽  
Added Value ◽  
Alcohol Septal Ablation ◽  
High Risk Type ◽  
Life Threatening ◽  
Surgical Myectomy

Background: Myocardial contrast two-dimensional echocardiography (MC-2DE) is widely used to address alcohol septal ablation (ASA) in obstructive hypertrophic cardiomyopathy (HCM). Owing to its limited cut-planes, MC-2DE may inaccurately identify the contrast misplacement associated with an unsuccessful or complicated ASA outcome. Objective: The aim of this study was to assess the added value of myocardial contrast three-dimensional echocardiography (MC-3DE) compared with MC-2DE to identify the appropriate matching between the target septal zone (TSZ) and coronary artery branch for safe and long-term effective ASA in HCM patients. Methods: A consecutive series of 52 symptomatic obstructive HCM patients referred for isolated surgical myectomy (SM) was analyzed with MC-2DE and MC-3DE following injection of echocontrast into one or more septal branches. MC-2DE and MC-3DE patterns were categorized according to complete (Type 1) or incomplete (Type 2) TSZ covering, high-risk (Type 3) exceeding TSZ, or life-threatening outside TSZ distribution (Type 4). Results: MC-2DE per patient analysis showed a Type 1 pattern in 32 patients and Types 2–4 in the remaining 20 patients; subsequent MC-3DE analysis provided a re-phenotyping of MC-2DE findings in 22 of the 52 patients (42%), showing a high-risk Type 2 pattern in 17 of the 32 patients with Type 1, and a new life-threatening Type 4 in three patients with Type 2, respectively. All patients with MC-3DE Type 1 pattern underwent safe and effective ASA with a long-term uneventful follow-up, while the remaining patients underwent SM. Conclusions: Refining high risk or life-threatening contrast misplacement, MC-3DE is more accurate than conventional MC-2DE to target safe and long-term effective septal reduction with ASA in obstructive HCM patients referred for isolated SM.

Hereditary and acquired angioedema

2019 ◽  
Vol 40 (6) ◽  
pp. 441-445 ◽  
Author(s):  
Gayatri Patel ◽  
Jacqueline A. Pongracic
Keyword(s):  
Factor Xii ◽  
Autosomal Dominant Disorder ◽  
Acquired Angioedema ◽  
Enzyme Replacement ◽  
Life Threatening ◽  
Type 3 ◽  
Esterase Inhibitor

Hereditary angioedema (HAE) is an autosomal dominant disorder defined by a deficiency of functional C1 esterase inhibitor (C1-INH). Acquired angioedema is due to either consumption (type 1) or inactivation (type 2) of CI-INH. Both HAE and acquired angioedema can be life-threatening. Of the three types of HAE, type 1 is most common, occurring in approximately 85% of patients and characterized by decreased production of C1-INH, which results in reduced functional activity to 5‐40% of normal. Type 2 occurs in 15% of cases; C1-INH is detectable in normal or elevated quantities but is dysfunctional. Also, HAE with normal CI-INH (previously called type 3 HAE) is rare and characterized by normal complement studies. Specific genetic mutations have been linked to factor XII, angiopoietin-1, and plasminogen gene. Patients with unknown mutations are classified as unknown. The screening test for types 1 and 2 is complement component C4, which is low to absent at times of angioedema and during quiescent periods. A useful test to differentiate HAE from acquired angioedema is C1q protein, which is normal in HAE and low in acquired angioedema. The management of HAE has been transformed with the advent of disease-specific therapies. On-demand therapy options include plasma and recombinant C1-INH for intravenous infusion; ecallantide, an inhibitor of kallikrein; and icatibant, a bradykinin β2 receptor antagonist, both administered subcutaneously. For long-term prophylaxis, intravenous or subcutaneous C1-INH enzyme replacement and lanadelumab, a monoclonal antibody against kallikrein that is administered subcutaneously, are effective agents.

scholarly journals EXPRESS: Pulmonary Hypertension Associated With Neurofibromatosis Type 2

2021 ◽  
pp. 204589402110295
Author(s):  
Hirohisa Taniguchi ◽  
Tomoya Takashima ◽  
Ly Tu ◽  
Raphaël Thuillet ◽  
Asuka Furukawa ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Neurofibromatosis Type ◽  
Pulmonary Vascular Remodeling ◽  
Life Threatening ◽  
History Of ◽  
Pulmonary Arterial ◽  
First Time

Although precapillary pulmonary hypertension (PH) is a rare but severe complication of patients with neurofibromatosis type 1 (NF1), its association with NF2 remains unknown. Herein, we report a case of a 44-year-old woman who was initially diagnosed with idiopathic pulmonary arterial hypertension (IPAH) and treated with PAH-specific combination therapy. However, a careful assessment for a relevant family history of the disease and genetic testing reveal that this patient had a mutation in the NF2 gene. Using immunofluorescence and Western blotting, we demonstrated a decrease in endothelial NF2 protein in lungs from IPAH patients compared to control lungs, suggesting a potential role of NF2 in PAH development. To our knowledge, this is the first time that precapillary PH has been described in a patient with NF2. The altered endothelial NF2 expression pattern in PAH lungs should stimulate work to better understand how NF2 is contributing to the pulmonary vascular remodeling associated to these severe life-threatening conditions.

scholarly journals Benefit and Harm of Extended Dual Antiplatelet Therapy After PCI in high-risk TWILIGHT-like patients with acute coronary syndrome

2021 ◽  
Vol 10 (Supplement_1) ◽  
Author(s):  
HY Wang ◽  
R Zhang ◽  
ZX Cai ◽  
KF Dou
Keyword(s):  
Acute Coronary Syndrome ◽  
High Risk ◽  
Antiplatelet Therapy ◽  
Cardiovascular Death ◽  
Secondary Outcome ◽  
Short Term ◽  
Bleeding Events ◽  
Coronary Syndrome

Abstract Funding Acknowledgements Type of funding sources: None. Background Recent emphasis on reduced duration and/or intensity of antiplatelet therapy following PCI irrespective of indication for PCI may fail to account for the substantial risk of subsequent nontarget lesion events in acute coronary syndrome (ACS) patients. This study sought to investigate the benefits and risks of extended-term (>12-month) DAPT as compared with short-term DAPT in high-risk "TWILIGHT-like" ACS patients undergoing PCI. Methods All consecutive patients fulfilling the "TWILIGHT-like" criteria undergoing PCI from January 2013 to December 2013 were identified from the prospective Fuwai PCI Registry. High-risk "TWILIGHT-like" patients were defined by at least 1 clinical and 1 angiographic feature based on TWILIGHT trial selection criteria. The present analysis evaluated 4,875 high-risk "TWILIGHT-like" patients with ACS who were event-free at 12 months after PCI. The primary outcome was the composite of all-cause death, myocardial infarction (MI), or stroke at 30 months while BARC type 2, 3, or 5 bleeding was key secondary outcome. Results Extended DAPT compared with shorter DAPT reduced the composite outcome of all-cause death, MI, or stroke by 63% (1.5% vs. 3.8%; HRadj: 0.374, 95% CI: 0.256 to 0.548; HRmatched: 0.361, 95% CI: 0.221-0.590). The HR for cardiovascular death was 0.049 (0.007 to 0.362) and that for MI 0.45 (0.153 to 1.320) and definite/probable stent thrombosis 0.296 (0.080-1.095) in propensity-matched analyses. Rates of BARC type 2, 3, or 5 bleeding (0.9% vs. 1.3%; HRadj: 0.668 [0.379 to 1.178]; HRmatched: 0.721 [0.369-1.410]) did not differ significantly in patients treated with DAPT > 12-month or DAPT ≤ 12-month. The effect of long-term DAPT on primary and key secondary outcome across the proportion of ACS patients with 1-3, 4-5, or 6-9 risk factors showed a consistent manner (Pinteraction > 0.05). Conclusion Among high-risk "TWILIGHT-like" patients with ACS after PCI, long-term DAPT reduced ischemic events without increasing clinically meaningful bleeding events as compared with short-term DAPT, suggesting that extended DAPT might be considered in the treatment of ACS patients who present with a particularly higher risk for thrombotic complications. Abstract Figure.

scholarly journals PRM58 Long-Term Validation of the IMS CORE Diabetes Model in Type 1 and Type 2 Diabetes

2012 ◽  
Vol 15 (7) ◽  
pp. A470 ◽  
Author(s):  
V. Foos ◽  
J.L. Palmer ◽  
D. Grant ◽  
A. Lloyd ◽  
M. Lamotte ◽  
...  
Keyword(s):  
Type 2 Diabetes ◽  
Diabetes Model

Abstract 555: The Universal Clinical Classification of Acute Myocardial Infarction is a Better Predictor of Long Term Outcomes than ST Segment Classification

Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Manuel A Gonzalez ◽  
Dana Eilen ◽  
Rana A Marzouq ◽  
Saed Awadallah ◽  
Hiren R Patel ◽  
...  
Keyword(s):  
Risk Factors ◽  
Cardiovascular Risk ◽  
Inverse Relationship ◽  
Long Term Outcomes ◽  
St Segment ◽  
Vascular Beds

Introduction: The universal classification (UC) of AMI aims to facilitate cross-study analysis, yet the long-term outcomes using UC are largely unknown. Hypothesis: We tested the hypothesis that the long-term outcome of patients with AMI is better predicted by UC than ST segment classification. Methods: We conducted a prospective study of 348 consecutive patients with AMI with mean follow-up of 30.6 months. The primary outcome was the major adverse cardiovascular events (MACE) [composite of all causes of mortality, recurrent AMI, and stroke]. Multivariate and survival analysis of MACE was performed. Results: The study population was STEMI=168, NSTEMI=180, Type 1=278, Type 2=55, Type 3=5, Type 4a=2, Type 4b=5, and Type 5=3. During follow-up 80 patients died, 31 had an AMI, and 7 had a stroke. UC correlates with the ST segment classification (p<0.005). MACE free survival was different for Type 1 and Type 2 (p=0.043), but not for STEMI and NSTEMI. There was a positive association between MACE and the quartile of peak Troponin, number of cardiovascular risk factors, and number of vascular beds affected, and an inverse relationship with the utilization of discharge cardiovascular protective medications (all p≤0.01). No such inverse relationship existed for Type 2. Conclusions: UC of AMI is a better long-term predictor of MACE. The quartile of peak Troponin levels, cardiovascular risk factors, and number of vascular beds affected are independent predictors of MACE, while cardiac medications protect against MACE, except in Type 2 patients.

Keratoprostheses in silicone oil-filled eyes: long-term outcomes

2018 ◽  
Vol 103 (6) ◽  
pp. 781-788 ◽  
Author(s):  
Geetha Iyer ◽  
Bhaskar Srinivasan ◽  
Shweta Agarwal ◽  
Ruchika Pattanaik ◽  
Ekta Rishi ◽  
...  
Keyword(s):  
Silicone Oil ◽  
Vitreoretinal Surgery ◽  
Retrospective Chart Review ◽  
Protective Role ◽  
Visual Rehabilitation ◽  
Two Factors

PurposeTo analyse the functional and anatomical outcomes of different types of keratoprostheses in eyes with retained silicone oil following vitreoretinal surgery.MethodsRetrospective chart review of patients operated with any type of permanent keratoprosthesis (Kpro) in silicone oil-filled eyes between March 2003 and June 2017 were analysed.Results40 silicone oil-filled eyes underwent keratoprostheses, of which 22 were type 1 and 18 were type 2 Kpros (Lucia variant—nine, modified osteo odonto kerato prosthesis (MOOKP)—four, Boston type 2—three and osteoKpro—two) with a mean follow-up of 61.54 , 42.77, 45.25 , 25 and 37 months, respectively. Anatomic retention of the primary Kpro was noted in 33 eyes (82.5%). A best-corrected visual acuity of better than 20/200 and 20/400 was achieved in 26 (65%)+32 (80%) eyes. Retroprosthetic membrane (RPM) was the most common complication noted in 17 eyes (42.5%). Perioptic graft melt was noted in 4 of 22 eyes of the type 1 Kpro (2 (10.5%) without associated ocular surface disorder (OSD)) and in 1 eye each of Boston and Lucia type 2 Kpro. Laminar resorption occurred in one eye each of the MOOKP and OKP groups. Endophthalmitis and glaucoma did not occur in any eye.ConclusionAppropriately chosen keratoprosthesis is a viable option for visual rehabilitation in eyes post vitreoretinal surgery with retained silicone oil-induced keratopathy not amenable to conventional penetrating keratoplasty. Kpro melt among type 1 Kpro did not occur in 89.5% eyes without associated OSD (19 of 22 eyes), despite the lack of aqueous humour and presence of RPM (4 eyes), two factors considered to play a significant role in the causation of sterile melts. Of interest to note was the absence of infection in any of these eyes. The possible protective role of oil from endophthalmitis is interesting, though yet to be ascertained.

scholarly journals Marginating transitional B cells modulate neutrophils in the lung during inflammation and pneumonia

2021 ◽  
Vol 218 (9) ◽  
Author(s):  
John Podstawka ◽  
Sarthak Sinha ◽  
Carlos H. Hiroki ◽  
Nicole Sarden ◽  
Elise Granton ◽  
...  
Keyword(s):  
B Cells ◽  
B Cell ◽  
Regulatory Networks ◽  
Vascular Inflammation ◽  
Transitional B Cells ◽  
Pulmonary Capillaries ◽  
Life Threatening ◽  
B Cell Subsets

Pulmonary innate immunity is required for host defense; however, excessive neutrophil inflammation can cause life-threatening acute lung injury. B lymphocytes can be regulatory, yet little is known about peripheral transitional IgM+ B cells in terms of regulatory properties. Using single-cell RNA sequencing, we discovered eight IgM+ B cell subsets with unique gene regulatory networks in the lung circulation dominated by transitional type 1 B and type 2 B (T2B) cells. Lung intravital confocal microscopy revealed that T2B cells marginate in the pulmonary capillaries via CD49e and require CXCL13 and CXCR5. During lung inflammation, marginated T2B cells dampened excessive neutrophil vascular inflammation via the specialized proresolving molecule lipoxin A4 (LXA4). Exogenous CXCL13 dampened excessive neutrophilic inflammation by increasing marginated B cells, and LXA4 recapitulated neutrophil regulation in B cell–deficient mice during inflammation and fungal pneumonia. Thus, the lung microvasculature is enriched in multiple IgM+ B cell subsets with marginating capillary T2B cells that dampen neutrophil responses.

scholarly journals Patterns of care of subjects with type 1 and type 2 diabetes according to their cardiovascular risk

2021 ◽  
Vol 24 (1) ◽  
pp. 30
Author(s):  
Pintaudi, B.
Keyword(s):  
Risk Factors ◽  
Quality Of Care ◽  
Cardiovascular Risk ◽  
High Risk ◽  
Quality Indicators ◽  
High Cardiovascular Risk ◽  
Very High

AIM OF THE STUDY To explore the distribution by cardiovascular risk groups according to the classification promoted by the ESC (European Society of Cardiology) of subjects with type 1 (T1D) and type 2 (T2D) diabetes cared for by Italian diabetologists and to describe the quality indicators of care, with particular regard to cardiovascular risk factors. DESIGN AND METHODS The study is based on data extracted from electronic medical records of patients treated at the 258 diabetes centers participating in the Annals AMD initiative and active in the year 2018. Patients with T1D or T2D were stratified by cardiovascular risk, in accordance with the recent ESC guidelines. General descriptive indicators and measures of intermediate outcomes, intensity/appropriateness of pharmacological treatment for diabetes and cardiovascular risk factors, presence of other complications and overall quality of care were evaluated. RESULTS Overall, 29,368 adults with T1D and 473,740 subjects with T2D were evaluated. Among subjects with T1D: 64.7% were at very high cardiovascular risk, 28.5% at high risk and the remaining 6.8% at moderate risk. Among subjects with T1D at very high-risk: 54.7% had retinopathy, 29.0% had albuminuria, 7.3% had a history of major cardiovascular event, 47.3% had organ damage, 48.9% had three or more risk factors, and 70.6% had a diabetes duration of over 20 years. Among subjects with T2D: 78.5% were at very high cardiovascular risk, 20.9% at high risk and the remaining 0.6% at moderate risk. Among those with T2D at very high risk: 39.0% had organ damage, 89.1% had three or more risk factors, 18.7% had a previous major cardiovascular event, 26,4% had retinopathy, 39.5% had albuminuria. With regard to the glucose-lowering drugs: the use of DPPIV-i increased markedly as cardiovascular risk increased; the use of secretagogues also increased and, although within low percentages, also the use of GLP1-RA tended to increase. The use of SGLT2-i is also still limited, and only slightly higher in subjects with very high cardiovascular risk. In both types of diabetes, the overall quality of care, as summarized by the Q score values, tended to be lower as the level of cardiovascular riskincreased. CONCLUSIONS The analysis of a large population such as that of the AMD Annals database allowed to highlight the characteristics and quality indicators of care of subjects with T1D and T2D in relation to cardiovascular risk classes. A large proportion of subjects appear to be at high or very high risk. Glucose-lowering drug therapies seem not to be adequately used with respect to the potential advantages in terms of reduction of cardiovascular risk of some drug categories (GLP1-RA and SGLT2-i) and, conversely, with respect to the potential risks related to the use of other pharmacological classes (sulfonylureas). Several actions are necessary to optimize care and improve the quality of care for both subjects with T1D and T2D. KEY WORDS type 1 diabetes; type 2 diabetes; cardiovascular risk; quality indicators of care.

scholarly journals Imaginary Worlds: The Status of Simulation Modeling in Claims for Cost-Effectiveness In Diabetes Mellitus

2016 ◽  
Vol 7 (2) ◽  
Author(s):  
Paul C Langley ◽  
Taeho Greg Rhee
Keyword(s):  
Diabetes Mellitus ◽  
Cost Effectiveness ◽  
Simulation Modeling ◽  
Decision Makers ◽  
The Past ◽  
The Status ◽  
The Impact

Over the past 20 years a number of simulations or models have been developed as a basis for tracking and evaluating the impact of pharmacological and other interventions in type 1 and type 2 diabetes mellitus. These models have typically tracked the natural course of these diseases generating long-term composite claims for cost-effectiveness. These claims can extend over the lifetime of the modeled patient cohort. Set against the standards of normal science, however, these claims lack credibility. The claims presented are all too often either immune to failure or are presented in a form that is non-testable. As such they fail to meet the key experimental requirements of falsification and replication. Unfortunately, there is a continuing belief that long-term or lifetime models are essential to decision-making. This is misplaced. The purpose of this review is to argue that there is a pressing need to reconsider the needs of health system decision makers and focus on modeled or simulated claims that are meaningful, testable, reportable and replicable in evaluating interventions in diabetes mellitus.   Type: Commentary

scholarly journals Neuropeptide Y autoantibodies in patients with long-term type 1 and type 2 diabetes and neuropathy

2013 ◽  
Vol 27 (6) ◽  
pp. 609-617 ◽  
Author(s):  
Hanna Skärstrand ◽  
L.B. Dahlin ◽  
Å. Lernmark ◽  
F. Vaziri-Sani
Keyword(s):  
Type 2 Diabetes ◽  
Neuropeptide Y ◽  
Term Type
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