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50th Anniversary of the first Human Heart Transplant-How is it seen today?

Wed, 12/13/2017 - 13:45
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50th Anniversary of the first Human Heart Transplant-How is it seen today?

Eur Heart J. 2017 Dec 07;38(46):3402-3404

Authors: Sliwa K, Zilla P

PMID: 29232446 [PubMed - in process]

Christiaan Barnard's defining moment: The epic first human heart transplant performed 50 years ago by Chris Barnard is discussed by David Cooper MD who was with Barnard at Groote Schuur Hospital in Cape Town.

Wed, 12/13/2017 - 13:45
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Christiaan Barnard's defining moment: The epic first human heart transplant performed 50 years ago by Chris Barnard is discussed by David Cooper MD who was with Barnard at Groote Schuur Hospital in Cape Town.

Eur Heart J. 2017 Dec 07;38(46):3400-3401

Authors: Cooper DKC

PMID: 29232445 [PubMed - in process]

Chris Barnard 50th Heart Transplant Anniversary.

Wed, 12/13/2017 - 13:45
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Chris Barnard 50th Heart Transplant Anniversary.

Eur Heart J. 2017 Dec 07;38(46):3399

Authors:

PMID: 29232444 [PubMed - in process]

Complex Decision-Making in Heart Failure: A Systematic Review and Thematic Analysis.

Wed, 12/13/2017 - 13:45
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Complex Decision-Making in Heart Failure: A Systematic Review and Thematic Analysis.

J Cardiovasc Nurs. 2017 Dec 11;:

Authors: Hamel AV, Gaugler JE, Porta CM, Hadidi NN

Abstract
BACKGROUND: Heart failure follows a highly variable and difficult course. Patients face complex decisions, including treatment with implantable cardiac defibrillators, mechanical circulatory support, and heart transplantation. The course of decision-making across multiple treatments is unclear yet integral to providing informed and shared decision-making. Recognizing commonalities across treatment decisions could help nurses and physicians to identify opportunities to introduce discussions and support shared decision-making.
OBJECTIVE: The specific aims of this review are to examine complex treatment decision-making, specifically implantable cardiac defibrillators, ventricular assist device, and cardiac transplantation, and to recognize commonalities and key points in the decisional process.
METHODS: MEDLINE, CINAHL, PsycINFO, and Web of Science were searched for English-language studies that included qualitative findings reflecting the complexity of heart failure decision-making. Using a 3-step process, findings were synthesized into themes and subthemes.
RESULTS: Twelve articles met criteria for inclusion. Participants included patients, caregivers, and clinicians and included decisions to undergo and decline treatment. Emergent themes were "processing the decision," "timing and prognostication," and "considering the future." Subthemes described how participants received and understood information about the therapy, making and changing a treatment decision, timing their decision and gauging health status outcomes in the context of their decision, the influence of a life or death decision, and the future as a factor in their decisional process.
CONCLUSIONS: Commonalities were present across therapies, which involved the timing of discussions, the delivery of information, and considerations of the future. Exploring this further could help support patient-centered care and optimize shared decision-making interventions.

PMID: 29232275 [PubMed - as supplied by publisher]

Patient-reported outcomes enhance the survival prediction of traditional disease risk classifications: An international study in patients with myelodysplastic syndromes.

Wed, 12/13/2017 - 13:45
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Patient-reported outcomes enhance the survival prediction of traditional disease risk classifications: An international study in patients with myelodysplastic syndromes.

Cancer. 2017 Dec 12;:

Authors: Efficace F, Cottone F, Abel G, Niscola P, Gaidano G, Bonnetain F, Anota A, Caocci G, Cronin A, Fianchi L, Breccia M, Stauder R, Platzbecker U, Palumbo GA, Luppi M, Invernizzi R, Bergamaschi M, Borin L, Di Tucci AA, Zhang H, Sprangers M, Vignetti M, Mandelli F

Abstract
BACKGROUND: Current prognostic systems for myelodysplastic syndromes (MDS) are based on clinical, pathologic, and laboratory indicators. The objective of the current study was to develop a new patient-centered prognostic index for patients with advanced MDS by including self-reported fatigue severity into a well-established clinical risk classification: the International Prognostic Scoring System (IPSS).
METHODS: A total of 469 patients with advanced (ie, IPSS intermediate-2 or high-risk) MDS were analyzed. Untreated patients (280 patients) were recruited into an international prospective cohort observational study to create the index. The index then was applied to an independent cohort including pretreated patients with MDS from the Dana-Farber Cancer Institute in Boston, Massachusetts (189 patients). At baseline, patients completed the European Organization for Research and Treatment of Cancer Quality of Life Questionnaire-Core 30 (EORTC QLQ-C30).
RESULTS: A new prognostic index was developed: the FA-IPSS(h), in which FA stands for fatigue and h for higher-risk. This new risk classification enabled the authors to distinguish 3 subgroups of patients with distinct survival outcomes (ie, risk-1, risk-2, and risk-3). Patients classified as FA-IPSS(h) risk-1 had a median overall survival (OS) of 23 months (95% confidence interval [95% CI], 19-29 months), whereas those with risk-2 had a median OS of 16 months (95% CI, 12-17 months) and those with risk-3 had a median OS of 10 months (95% CI, 4-13 months). The predictive accuracy of this new index was higher than that of the IPSS alone in both the development cohort as well as in the independent cohort including pretreated patients.
CONCLUSIONS: The FA-IPSS(h) is a novel patient-centered prognostic index that includes patients' self-reported fatigue severity. The authors believe its use might enhance physicians' ability to predict survival more accurately in patients with advanced MDS. Cancer 2017. © 2017 American Cancer Society.

PMID: 29231969 [PubMed - as supplied by publisher]

Practice patterns to improve pre and post-transplant medication adherence in heart transplant centres: a secondary data analysis of the international BRIGHT study.

Wed, 12/13/2017 - 13:45
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Practice patterns to improve pre and post-transplant medication adherence in heart transplant centres: a secondary data analysis of the international BRIGHT study.

Eur J Cardiovasc Nurs. 2017 Dec 01;:1474515117747577

Authors: Senft Y, Kirsch M, Denhaerynck K, Dobbels F, Helmy R, Russell CL, Berben L, De Geest S, BRIGHT study team

Abstract
BACKGROUND: As medication non-adherence is a major risk factor for poor post-transplant outcomes, we explored how adherence is assessed, enhanced and integrated across the transplant continuum.
AIM: The aim of this study was to study practice patterns regarding pre- and post-transplant medication adherence assessment and interventions in international heart transplant centres.
METHODS: We used data from the Building Research Initiative Group: chronic illness management and adherence in heart transplantation (BRIGHT) study, a cross-sectional study conducted in 36 heart transplant centres in 11 countries. On a 27-item questionnaire, 100 clinicians (range one to five per centre) reported their practice patterns regarding adherence assessment and intervention strategies pre-transplant, immediately post-transplant, less than one year, and one or more year post-transplant. Educational/cognitive, counselling/behavioural and psychosocial/affective strategies were assessed. Clinicians' responses (intervention present vs. absent; or incongruence in reporting intervention) were aggregated at the centre level.
RESULTS: The adherence assessment method most commonly used along the transplant continuum was questioning patients (range 75-88.9%). Pre-transplant, all three categories of intervention strategy were applied. Providing reading materials (82.9%) or instructions (68.6%), involving family or support persons in education (91.4%), and establishing partnership (91.4%) were used most frequently. Post-transplant, strategies closely resembled those employed pre-transplant. Training patients (during recovery) and cueing were more often applied during hospitalisation (74.3%). After the first year post-transplant, except for motivational interviewing (25.7-28.6%), the number of strategies decreased.
CONCLUSIONS: Across the transplant continuum, diverse adherence interventions are implemented; however, post-transplant, the frequency of adherence interventions decreases. Therefore, increased investment is necessary in long-term adherence interventions.

PMID: 29231747 [PubMed - as supplied by publisher]

Temporary Biventricular Assist Device to Bridge a Patient With Cardiogenic Shock Due to AL Amyloidosis to Heart Transplantation.

Wed, 12/13/2017 - 13:45
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Temporary Biventricular Assist Device to Bridge a Patient With Cardiogenic Shock Due to AL Amyloidosis to Heart Transplantation.

Artif Organs. 2017 Dec;41(12):1183-1184

Authors: Al-Lawati K, Lim HS

PMID: 29230849 [PubMed - in process]

Unusual Pathology in a Kidney from a Heart-Transplant Patient.

Wed, 12/13/2017 - 13:45
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Unusual Pathology in a Kidney from a Heart-Transplant Patient.

Case Rep Transplant. 2017;2017:1084718

Authors: Larcher M, Delas A, Delmas C, Cointault O, Dambrin C, Del Bello A, Kamar N

Abstract
Acute kidney injury (AKI) is often observed after heart transplantation. In this setting, acute tubular necrosis is the main histological finding on kidneys. We report the unusual pathology found in a kidney from a heart-transplant patient. The patient experienced several hemodynamic insults, massive transfusion, and implantation of a mechanical circulatory-support device before heart transplantation: there was prolonged AKI after transplantation. A kidney biopsy revealed acute tubular necrosis and renal hemosiderosis, which was probably related to the transfusion and to mechanical circulatory-support device-induced intravascular hemolysis. Assessment of iron during resuscitation could have prevented, at least partly, AKI.

PMID: 29230343 [PubMed]

Invasive and noninvasive hemodynamic assessment in adults with Fontan palliation.

Wed, 12/13/2017 - 13:45
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Invasive and noninvasive hemodynamic assessment in adults with Fontan palliation.

Int J Cardiol. 2017 Dec 05;:

Authors: Egbe AC, Connolly HM, Taggart NW, Al-Otaibi M, Borlaug BA

Abstract
BACKGROUND/OBJECTIVES: Although echocardiographic-Doppler cardiac index (CI) assessment is widely used to guide heart failure management in patients with biventricular circulation, this application has not been studied in the Fontan population. The objective of this study was to: (1) determine the correlation between Doppler and cardiac catheterization CI calculation; (2) determine the association between Doppler CI and the occurrence of Fontan failure.
METHODS: Retrospective review of adult Fontan patients followed at Mayo Clinic Adult Congenital Heart Disease program, 1994-2015. Inclusion criteria were: systemic left ventricle and echocardiogram and cardiac catheterization performed within the same week. Fontan failure was defined as a composite of all-cause mortality, heart transplantation listing, and palliative care.
RESULTS: 59 patients (age 29±6years; men 32[54%]) underwent 97 studies. Of the 59, 41[69%] had atriopulmonary Fontan and 12 (20%) had cirrhosis. Compared to patients without cirrhosis, patients with cirrhosis had higher Doppler CI (3.6±0.6 vs 2.8±0.4L/min∗m2, p=0.039); Fick CI (3.3 [2.5-3.7] vs 2.4 [1.6-3.1] L/min/m2, p=0.028); lower systemic vascular resistance (20±3 vs 25±4 WU∗m2, p=0.04). There was a positive correlation between Doppler and Fick CI (r=0.52; p<0.0001). Fontan failure occurred in 13 patients (22%) within 7.5±2.1years. In patients without cirrhosis, Fick CI and Doppler CI <2.5L/min/m2 were associated with Fontan failure (odds ratio [OR] 1.58, p=0.046) and (OR 1.43, p=0.051) respectively.
CONCLUSIONS: Doppler CI assessment in feasible in a selected group of Fontan patients and it is predictive of clinical outcomes. The application of this concept in systemic right ventricles deserves further research.

PMID: 29229372 [PubMed - as supplied by publisher]

Pretransplant coronary artery disease is a predictor for myocardial infarction and cardiac death after liver transplantation.

Wed, 12/13/2017 - 13:45
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Pretransplant coronary artery disease is a predictor for myocardial infarction and cardiac death after liver transplantation.

Eur J Intern Med. 2017 Dec 08;:

Authors: Darstein F, Hoppe-Lotichius M, Vollmar J, Weyer-Elberich V, Zimmermann A, Mittler J, Otto G, Lang H, Galle PR, Zimmermann T

Abstract
BACKGROUND: Cardiovascular disease is a serious problem of liver transplant (LT) recipients because of increased cardiovascular risk due to immunosuppressive therapy, higher age, intraoperative risk and comorbidities (such as diabetes and nicotine abuse). Reported frequency of cardiovascular events after LT shows a high variability between different LT cohorts. Our aim was to analyze a cohort of LT recipients from a single center in Germany to evaluate frequency of the cardiovascular endpoints (CVE) myocardial infarction and/or cardiac death after LT and to investigate correlations of CVE post LT with pretransplant patient characteristics.
PATIENTS: In total, data from 352 LT patients were analyzed. Patients were identified from an administrative transplant database, and all data were retrieved from patients' charts and reports.
RESULTS: During the median follow-up of 4.0 (0-13) years, 10 cases of CVE were documented (six myocardial infarctions and four coronary deaths). The frequency of CVE did not differ according to classic cardiovascular risk factors such as body mass index (p=0.071), total cholesterol (p=0.533), hypertension (p=0.747), smoking (p=1.000) and pretransplant diabetes mellitus (p=0.146). In patients with pretransplant coronary heart disease (n=24; 6.8%) CVE were found more frequently (p=0.024).
CONCLUSION: In summary, we found a rate of 2.8% CVE after LT in a German transplant cohort. Pretransplant CHD was the only risk factor for CVE, but showed no significant impact on overall survival.

PMID: 29229303 [PubMed - as supplied by publisher]

Intratracheal instillation of alveolar type II cells enhances recovery from acute lung injury in rats.

Wed, 12/13/2017 - 13:45
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Intratracheal instillation of alveolar type II cells enhances recovery from acute lung injury in rats.

J Heart Lung Transplant. 2017 Nov 08;:

Authors: Guillamat-Prats R, Puig F, Camprubí-Rimblas M, Herrero R, Serrano-Mollar A, Gómez MN, Tijero J, Matthay MA, Blanch L, Artigas A

Abstract
BACKGROUND: Acute lung injury (ALI) and acute respiratory distress syndrome (ARDS) are characterized by excess production of inflammatory factors. Alveolar type II (ATII) cells help repair damaged lung tissue, rapidly proliferating and differentiating into alveolar type I cells after epithelial cell injury. In ALI, the lack of viable ATII favors progression to more severe lung injury. ATII cells regulate the immune response by synthesizing surfactant and other anti-inflammatory proteins and lipids. Cross-talk between ATII and other cells such as macrophages may also be part of the ATII function. The aim of this study was to test the anti-inflammatory and reparative effects of ATII cells in an experimental model of ALI.
METHODS: In this study ATII cells (2.5 × 106 cells/animal) were intratracheally instilled in rats with HCl and lipopolysaccharide (LPS)-induced ALI and in healthy animals to check for side effects. The specific effect of ATII cells was compared with fibroblast transplantation.
RESULTS: ATII cell transplantation promoted recovery of lung function, decrease mortality and lung inflammation of the animals with ALI. The primary mechanisms for benefit were paracrine effects of prostaglandin E2 (PGE2) and surfactant protein A (SPA) released from ATII cells that modulate alveolar macrophages to an anti-inflammatory phenotype. To our knowledge, these data are the first to provide evidence that ATII cells secrete PGE2 and SPA, reducing pro-inflammatory macrophage activation and ALI.
CONCLUSION: ATII cells and their secreted molecules have shown an ability to resolve ALI, thereby highlighting a potential novel therapeutic target.

PMID: 29229270 [PubMed - as supplied by publisher]

The impact of screening method on HLA antibody detection before and after lung transplantation: A prospective pilot study.

Wed, 12/13/2017 - 13:45
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The impact of screening method on HLA antibody detection before and after lung transplantation: A prospective pilot study.

J Heart Lung Transplant. 2017 Nov 22;:

Authors: Cao S, Courtwright AM, Lamattina AM, Guleria I, Burkett P, El-Chemaly S, Goldberg HJ

PMID: 29229269 [PubMed - as supplied by publisher]

Heart Transplantation in Patients ≥60 Years: Importance of Relative Pulmonary Hypertension and Right Ventricular Failure on Midterm Survival.

Wed, 12/13/2017 - 13:45
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Heart Transplantation in Patients ≥60 Years: Importance of Relative Pulmonary Hypertension and Right Ventricular Failure on Midterm Survival.

J Cardiothorac Vasc Anesth. 2017 Sep 18;:

Authors: Bianco JC, Mc Loughlin S, Denault AY, Marenchino RG, Rojas JI, Bonofiglio FC

Abstract
OBJECTIVES: To determine the impact of recipient age and perioperative risk factors on midterm survival after orthotopic heart transplantation (OHT). The authors hypothesized that perioperative variables are more important as predictors of mortality than is a recipient's age.
DESIGN: Retrospective study.
SETTING: Tertiary care university hospital.
PARTICIPANTS: The study comprised 126 consecutive adults who underwent OHT.
INTERVENTIONS: After Institutional Review Board approval, the authors analyzed 126 consecutive adult patients who underwent OHT between January 2009 and December 2015 and followed-up with them up until June 2016. Patients were divided into the following 2 groups according to the recipient's age at the time of transplantation: older group (≥60 y old) and younger group (18 to 59 y).
MEASUREMENTS AND MAIN RESULTS: Actuarial survival rates for all patients were 88.1%, 78.6%, and 72.2% at 30 days, 1 year, and after a median follow-up of 18.9 months (midterm survival) (1st quartile: 8.1; 3rd quartile: 37.4), respectively. In the unadjusted analysis, the older group demonstrated a significant increase in 1-year mortality (p = 0.005) and a trend toward worse midterm mortality (p = 0.087). Multivariable analysis was performed using Cox proportional hazards regression analysis. Independent risk factors related to midterm mortality after OHT were as follows: preoperative relative pulmonary hypertension using the mean arterial-to-mean pulmonary artery pressure ratio ≤3 (hazard ratio [HR] 5.39, 95% confidence interval [CI] 1.64-17.74, p = 0.006); cardiopulmonary bypass duration (per each 10-min increment) (HR 1.14, 95% CI 1.08-1.22, p < 0.001); and postoperative right ventricular dysfunction (HR 3.50, 95% CI 1.52-8.05, p = 0.003). Neither recipients ≥60 years old (HR 2.15, 95% CI 0.98-4.67, p = 0.054) nor donor/recipient body surface area ratio (HR 1.01, 95% CI 0.98-1.04, p = 0.463) was an independent risk factor for midterm mortality.
CONCLUSIONS: In patients undergoing heart transplantation, survival was related more to preoperative relative pulmonary hypertension, cardiopulmonary bypass duration, and postoperative right ventricular failure than to recipient age. Older patients should be selected for OHT carefully, taking into consideration preoperative factors other than age.

PMID: 29229262 [PubMed - as supplied by publisher]

Preliminary Experience in Combined Somatic and Cerebral Oximetry Monitoring in Liver Transplantation.

Wed, 12/13/2017 - 13:45
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Preliminary Experience in Combined Somatic and Cerebral Oximetry Monitoring in Liver Transplantation.

J Cardiothorac Vasc Anesth. 2017 Jul 20;:

Authors: Hu T, Collin Y, Lapointe R, Carrier FM, Massicotte L, Fortier A, Lambert J, Vandenbroucke-Menu F, Denault AY

Abstract
OBJECTIVE: The use of cerebral near-infrared spectroscopy (NIRS) has become widespread in cardiac surgery after research demonstrated an association between perioperative cerebral desaturations and postoperative complications. Somatic NIRS desaturation also is associated with an increased risk of postoperative complications and mortality. The objective of this study was to explore the trends of both somatic and cerebral NIRS during liver transplantation.
DESIGN: A prospective, single-site, observational case series.
SETTING: Tertiary care center.
PARTICIPANTS: The study comprised 10 patients undergoing liver transplantation.
INTERVENTIONS: NIRS sensors were placed on the forehead (cerebral regional oxygen saturation [rSO2]) and on the right arm and right leg (somatic rSO2) to measure tissue perfusion. Desaturation was defined as a 20% decrease of baseline values for 15 seconds.
MEASUREMENTS AND MAIN RESULTS: In all patients, parallel changes in both cerebral and somatic rSO2 values were observed during phlebotomy, bleeding, transfusion, portal vein clamping, and the use of vasoactive agents. Induction of anesthesia increased cerebral rSO2 more than it did somatic values. However, ascites removal, abdominal manipulation, and clamping of the inferior vena cava (IVC) were associated with nonparallel changes in cerebral and somatic rSO2. Ascites removal was associated with increased somatic leg rSO2, and IVC clamping and abdominal hypertension were associated with a significant reduction in somatic leg rSO2. Somatic leg desaturation instead of arm or cerebral desaturation was associated with more postoperative complications.
CONCLUSIONS: The use of combined NIRS monitoring allows for the identification of the source of somatic or cerebral desaturation. Compromised venous flow from the IVC from clamping or abdominal compartment syndrome typically is associated with the appearance of more pronounced leg than arm desaturation.

PMID: 29229261 [PubMed - as supplied by publisher]

Factors Related to the Severity of Early Postoperative Infection After Heart Transplantation in Patients Surviving Prolonged Mechanical Support Periods: Experience at a Single University.

Wed, 12/13/2017 - 13:45
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Factors Related to the Severity of Early Postoperative Infection After Heart Transplantation in Patients Surviving Prolonged Mechanical Support Periods: Experience at a Single University.

J Cardiothorac Vasc Anesth. 2017 Jul 08;:

Authors: Abe R, Shibata SC, Saito S, Tsukamoto Y, Toda K, Uchiyama A, Sakata Y, Sawa Y, Tomono K, Fujino Y

Abstract
OBJECTIVE: The authors examined the effect of prolonged support with continuous-flow ventricular assist devices (CF-VADs) and other related factors on the severity of infections within 30 days of heart transplantation (HTx).
DESIGN: A retrospective analysis of consecutive HTx procedures.
SETTING: University hospital, between 2010 and 2016.
PARTICIPANTS: A cohort of 53 heart transplantation recipients (median age, 38.5 yr; interquartile range [IQR], 30.3-49.2 yr; women, 34%).
INTERVENTIONS: Forty-nine patients required CF-VAD support (median duration, 946 d; IQR, 600-1,132 d).
MEASUREMENTS AND MAIN RESULTS: Severity of postoperative infections was categorized as follows: no infection, minor infection (resolved within 14 days), major infection (resolved after >14 days), and severe infection (septic shock). Results were expressed as number (frequency) and median with IQR. Potential risk factors for increased infection severity were expressed as odds ratio (OR) with 95% confidence interval (CI). Postoperatively, no infection, minor infection, major infection, and severe infection occurred in 32 (60.4%), 8 (15.1%), 8 (15.1%), and 5 patients (9.4%), respectively. Active ventricular assist device (VAD)-specific infections at the time of HTx occurred in 37.7% of patients. Moderate-to-severe primary graft dysfunction occurred in 26.4% of the patients. Multivariable analysis indicated that risk factors for increased infection severity included active VAD-specific infection (OR 4.8; 95% CI 2.3-11.2) and moderate-to-severe primary graft dysfunction (OR 8.8; 95% CI 2.1-42.5) but not duration of CF-VAD support (OR 1.0; 95% CI 1.0-1.0).
CONCLUSION: Active VAD-specific infection and poor graft function likely contribute to the severity of early postoperative infections after HTx.

PMID: 29229257 [PubMed - as supplied by publisher]

Pulmonary veno-occlusive disease: An important consideration in patients with pulmonary hypertension.

Wed, 12/13/2017 - 13:45
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Pulmonary veno-occlusive disease: An important consideration in patients with pulmonary hypertension.

Respir Med. 2017 Nov;132:203-209

Authors: Balko R, Edriss H, Nugent K, Test V

Abstract
Pulmonary veno-occlusive disease is a rare subcategory of pulmonary arterial hypertension (WHO Group 1). The disease is poorly understood and difficult to diagnose; it has no definitive cure to date. These patients present with nonspecific symptoms, including dyspnea, exercise intolerance, and weakness. Chest x-rays sometimes differ from idiopathic pulmonary arterial hypertension and may demonstrate alveolar infiltrates and pleural effusions. High resolution computed tomography scans reveal ground glass opacities, interlobular septal thickening, and lymphadenopathy. Echocardiography can estimate the level of pulmonary artery pressures; right heart catheterization is needed for complete hemodynamic characterization of these patients. Lung biopsies demonstrate remodeling of the venules and small veins with intimal and adventitial fibrosis. This can result in total venous occlusion and subsequent recanalization. Similar changes occur in the small arteries and arterioles but are less pronounced than the venous changes. There is no effective medical therapy for these patients, and treatment with the pulmonary arterial hypertension specific medications often causes acute deterioration with pulmonary edema. The recent discovery of the biallelic mutations of the EIF2AK4 gene as an etiology for heritable form of pulmonary veno-occlusive disease increases our understanding of the disease pathogenesis and potentially identifies a future approach to treatment. Without definitive treatment, the prognosis is very poor, and the life expectancy of these patients is much shorter than patients with pulmonary arterial hypertension. These patients need early referral to transplantation centers.

PMID: 29229098 [PubMed - in process]

Gremlin-1 is a key regulator of the invasive cell phenotype in mesothelioma.

Wed, 12/13/2017 - 13:45
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Gremlin-1 is a key regulator of the invasive cell phenotype in mesothelioma.

Oncotarget. 2017 Nov 17;8(58):98280-98297

Authors: Yin M, Tissari M, Tamminen J, Ylivinkka I, Rönty M, von Nandelstadh P, Lehti K, Hyytiäinen M, Myllärniemi M, Koli K

Abstract
Malignant mesothelioma originates from mesothelial cells and is a cancer type that aggressively invades into the surrounding tissue, has poor prognosis and no effective treatment. Gremlin-1 is a cysteine knot protein that functions by inhibiting BMP-pathway activity during development. BMP-independent functions have also been described for gremlin-1. We have previously shown high gremlin-1 expression in mesothelioma tumor tissue. Here, we investigated the functions of gremlin-1 in mesothelioma cell migration and invasive growth. Gremlin-1 promoted mesothelioma cell sprouting and invasion into three dimensional collagen and Matrigel matrices. The expression level of gremlin-1 was linked to changes in the expression of SNAI2, integrins, matrix metalloproteinases (MMP) and TGF-β family signaling - all previously associated with a mesenchymal invasive phenotype. Small molecule inhibitors of MMPs completely blocked mesothelioma cell invasive growth. In addition, inhibitors of TGF-β receptors significantly reduced invasive growth. This was associated with reduced expression of MMP2 but not SNAI2, indicating that gremlin-1 has both TGF-β pathway dependent and independent mechanisms of action. Results of in vivo mesothelioma xenograft experiments indicated that gremlin-1 overexpressing tumors were more vascular and had a tendency to send metastases. This suggests that by inducing a mesenchymal invasive cell phenotype together with enhanced tumor vascularization, gremlin-1 drives mesothelioma invasion and metastasis. These data identify gremlin-1 as a potential therapeutic target in mesothelioma.

PMID: 29228689 [PubMed]

Kleefstra Syndrome: The First Case Report From Iran.

Wed, 12/13/2017 - 13:45
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Kleefstra Syndrome: The First Case Report From Iran.

Acta Med Iran. 2017 Oct;55(10):650-654

Authors: Noruzinia M, Ahmadvand M, Bashti O, Salehi Chaleshtori AR

Abstract
Kleefstra Syndrome is characterized by severe mental retardation, brachycephaly, microcephaly, epileptic seizures, distinct facial features, and infantile weak muscle tone and heart defects. Deletion of EHMT1 is the main player in 75% of cases. Because of blurriness in genotype-phenotype correlation through clinical and molecular features of both 9q34.3 microdeletion patients and those with an intragenic EHMT1 mutation in Kleefstra Syndrome, genetic characterization of patients with clinical symptoms of such spectrum is desirable. We report the first Kleefstra Syndrome patient in Iran characterized through genetic approaches. Our report could improve KS diagnosis in Iran and prepare PND and PGs options for involved families.

PMID: 29228531 [PubMed - in process]

Popliteal Artery Pseudoaneurysm Caused by Osteochondroma.

Wed, 12/13/2017 - 13:45
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Popliteal Artery Pseudoaneurysm Caused by Osteochondroma.

Ann Vasc Surg. 2017 Aug;43:313.e5-313.e7

Authors: Sakata T, Mogi K, Sakurai M, Nomura A, Fujii M, Takahara Y

Abstract
A 16-year-old boy developed pulsating pain and dysesthesia in his right knee. Computed tomography showed a large aneurysm in the right upper popliteal artery and a spiked bone tumor arising from the right distal femoral shaft. Pseudoaneurysm due to osteochondroma was suspected, and the patient underwent emergency surgery. A 2-mm pinhole was detected in the arterial wall behind the tumor. After resection of the tumor, the damaged arterial wall was removed, and the defect was repaired using a saphenous vein patch. We suggest that patch repair is preferable to direct closure or end-to-end anastomosis to prevent recurrent pseudoaneurysm at a later time, even if the defect is small.

PMID: 28478169 [PubMed - indexed for MEDLINE]

Accuracy of left ventricular ejection fraction by contemporary multiple gated acquisition scanning in patients with cancer: comparison with cardiovascular magnetic resonance.

Wed, 12/13/2017 - 13:45
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Accuracy of left ventricular ejection fraction by contemporary multiple gated acquisition scanning in patients with cancer: comparison with cardiovascular magnetic resonance.

J Cardiovasc Magn Reson. 2017 Mar 24;19(1):34

Authors: Huang H, Nijjar PS, Misialek JR, Blaes A, Derrico NP, Kazmirczak F, Klem I, Farzaneh-Far A, Shenoy C

Abstract
BACKGROUND: Multiple gated acquisition scanning (MUGA) is a common imaging modality for baseline and serial assessment of left ventricular ejection fraction (LVEF) for cardiotoxicity risk assessment prior to, surveillance during, and surveillance after administration of potentially cardiotoxic cancer treatment. The objective of this study was to compare the accuracy of left ventricular ejection fractions (LVEF) obtained by contemporary clinical multiple gated acquisition scans (MUGA) with reference LVEFs from cardiovascular magnetic resonance (CMR) in consecutive patients with cancer.
METHODS: In a cross-sectional study, we compared MUGA clinical and CMR reference LVEFs in 75 patients with cancer who had both studies within 30 days. Misclassification was assessed using the two most common thresholds of LVEF used in cardiotoxicity clinical studies and practice: 50 and 55%.
RESULTS: Compared to CMR reference LVEFs, MUGA clinical LVEFs were only lower by a mean of 1.5% (48.5% vs. 50.0%, p = 0.17). However, the limits of agreement between MUGA clinical and CMR reference LVEFs were wide at -19.4 to 16.5%. At LVEF thresholds of 50 and 55%, there was misclassification of 35 and 20% of cancer patients, respectively.
CONCLUSIONS: MUGA clinical LVEFs are only modestly accurate when compared with CMR reference LVEFs. These data have significant implications on clinical research and patient care of a population with, or at risk for, cardiotoxicity.

PMID: 28335788 [PubMed - indexed for MEDLINE]

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