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Background: Mortality of pulmonary hypertension associated with congenital heart disease (PAH-CHD) in adults remains high., Objectives: To identify predictors of death and to assess the impact of treatment on outcome., Methods: Retrospective, multicenter cohort study of 103 adults with PAH-CHD followed-up for 8.6 ± 4.6 years. Patients were grouped according to underlying shunt type into pre-tricuspid, post-tricuspid and complex. Survival rates were analyzed and predictors of death were investigated with Cox regression models., Results: In the post-tricuspid and complex groups (38 and 37 patients, respectively), the most common clinical PAH-CHD subgroup was Eisenmenger syndrome (76.3% and 59.5%, respectively) whereas, in the pre-tricuspid group (28 patients), 46.5% of patients had small or corrected defects. Overall, 88 patients received vasodilators; 39% required combination-therapy. Overall survival at 10 years was 65%. Mortality was highest in the pre-tricuspid group, FC-III-IV and amongst patients receiving monotherapy (p < 0.050). On multivariate analysis, predictors of poor outcome were pericardial effusion (HR: 4,520 [1,470-13,890]; p = 0,008), oxygen saturation(HR: 0.940 [0,900 - 0,990]; p = 0,018) and genetic syndromes(HR: 3,280 [1,098-9,780]; p = 0,033)., Conclusions: Patients in advanced stages at initiation of treatment were at high risk of death and strong consideration should be given for more aggressive therapy., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Background: Prevalence and prognostic significance of pulmonary artery (PA) dilatation in congenital heart disease (CHD) have never been studied systematically., Methods: Chest X-rays of 1192 consecutive adults with CHD were reviewed. Major diameter of the PA was determined by imaging techniques in those with PA dilatation. A value >29 mm was considered abnormal. Data on anatomy, hemodynamics, residual lesions and outcomes were retrospectively collected., Results: Overall prevalence of PA dilatation was 18%. A minority of patients (5.5%) reached 40 mm (aneurysm; PAA) and 1.8% exceeded 50 mm. The most common PAA underlying malformations were pulmonary stenosis (21%), and shunts (55%). Significantly larger diameters were observed in hypertensive shunts (40 mm; IQR 36.7-45 mm vs. 34 mm; IQR 32-36 mm) (p < 0.0001). However, the largest diameters were found in cono-truncal anomalies. There was no significant correlation between PA dimensions and systolic pulmonary pressure (r = -0.196), trans-pulmonary gradient (r = -0.203), pulmonary regurgitation (PR) (r = 0.071) or magnitude of shunt (r = 0.137) (p > 0.05 for all). Over follow-up, 1 sudden death (SD) occurred in one Eisenmenger patient. Complications included coronary (3), recurrent laryngeal nerve (1) and airway (1) compressions, progressive PR (1), and PA thrombosis (1). Coronary compression and SD were strongly associated (univariate analysis) with pulmonary hypertension (120 vs. 55 mm Hg; p = 0.002) but not with extreme PA dilatation (range: 40-65 mm)., Conclusions: PA dilatation in CHD is common but only a small percentage of patients have PAA. Clinical impact on outcomes is low. Complications occurred almost exclusively in patients with pulmonary hypertension whereas PA diameter alone was not associated with adverse outcomes., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Aims: Limited data exist on the treatment and outcomes of patients with chronic total occlusions due to in-stent restenosis (CTO-ISR). We sought to evaluate results and clinical outcomes in percutaneous interventions over CTO-ISR., Methods and Results: We conducted a registry in 16 centres involving consecutive patients undergoing attempted percutaneous intervention over CTO-ISR. A total of 233 patients were included, 192 (82.4%) with a successful acute result. The success rate was significantly lower in patients with: lesion in the circumflex artery, ostial lesions, J score >3 and no femoral access. The presence of tandem lesions (OR 0.19, 95% CI: 0.06-0.68; p=0.01) was identified as an independent predictor for outcome. After a median follow-up of 20 months, cardiac death in the successful vs. failed groups was 3% and 8.3%, respectively (p=0.09). Independent predictors for mortality were previous CABG (HR 8, 95% CI: 1.3-50; p=0.02) and multivessel disease (HR 2.3, 95% CI: 1.2-4.3; p=0.01). In the successful group, TLR was 15%, re-occlusion 6% and definite/probable stent thrombosis 1.8%., Conclusions: Percutaneous treatment of CTO due to ISR in contemporary practice shows a high success rate, resulting in good long-term results. The angiographic and procedural predictors identified may contribute to improving procedural outcome through better patient selection.

Background: The aim of this study was to evaluate the strategy and efficacy of a hyperglycemia treatment program supervised by Endocrinology., Methods: All patients with type 2 diabetes hospitalized at the vascular surgery department over a 12 month period were retrospectively reviewed. Clinical characteristics and hyperglycemia treatment during hospitalization, at discharge and 2-6 month after discharge were collected. Glycemic control was assessed using capillary blood glucose profiles and HbA1c at admission and 2-6 months post-discharge., Results: A total of 140 hospitalizations of 123 patients were included. The protocol to choose the insulin regimen was applied in 96.4% of patients (22.8% correction dose, 23.6% basal-correction dose and 50% basal-bolus-correction dose [BBC]). Patients with BBC had higher HbA1c (7.7±1.5% vs. 6.7 ±0.8%; P<.001) and mean glycemia on the first day of hospitalization (184.4±59.2 vs. 140.5±31.4mg/dl; P<.001). Mean blood glucose was reduced to 162.1±41.8mg/dl in the middle and 160.8±43.3mg/dl in the last 24h of hospitalization in patients with BBC (P=.007), but did not change in the remaining patients. In 22.1% patients with treatment changes performed at discharge, HbA1c decreased from 8.2±1.6 to 6.8±1.6% at 2-6 months post-discharge (P=.019)., Conclusions: The hyperglycemia treatment protocol applied by an endocrinologist in the hospital, allows the identification of the appropriate therapy and the improvement of the glycemic control during hospitalization and discharge, supporting its efficacy in clinical practice., (Copyright © 2014 AEC. Publicado por Elsevier España, S.L.U. All rights reserved.)

Background: Healthy diet and regular physical activity are powerful tools in reducing diabetes and cardiometabolic risk. Various international scientific and health organizations have advocated the use of new technologies to solve these problems. The PREDIRCAM project explores the contribution that a technological system could offer for the continuous monitoring of lifestyle habits and individualized treatment of obesity as well as cardiometabolic risk prevention., Methods: PREDIRCAM is a technological platform for patients and professionals designed to improve the effectiveness of lifestyle behavior modifications through the intensive use of the latest information and communication technologies. The platform consists of a web-based application providing communication interface with monitoring devices of physiological variables, application for monitoring dietary intake, ad hoc electronic medical records, different communication channels, and an intelligent notification system. A 2-week feasibility study was conducted in 15 volunteers to assess the viability of the platform., Results: The website received 244 visits (average time/session: 17 min 45 s). A total of 435 dietary intakes were recorded (average time for each intake registration, 4 min 42 s ± 2 min 30 s), 59 exercises were recorded in 20 heart rate monitor downloads, 43 topics were discussed through a forum, and 11 of the 15 volunteers expressed a favorable opinion toward the platform. Food intake recording was reported as the most laborious task. Ten of the volunteers considered long-term use of the platform to be feasible., Conclusions: The PREDIRCAM platform is technically ready for clinical evaluation. Training is required to use the platform and, in particular, for registration of dietary food intake., (© 2013 Diabetes Technology Society.)

Background: The aim of this study was to compare the prevalence of metabolic syndrome in human immunodeficiency virus (HIV)-infected patients treated with highly active antiretroviral therapy (HAART), using the National Cholesterol Education Program Adult Treatment Panel III (NCEP ATP III), European Group for the Study of Insulin Resistance (EGIR), and International Diabetes Federation (IDF) definitions., Methods: A cross-sectional study was carried out with 159 consecutive adult HIV-infected subjects (120 males and 39 females) under HAART. Anthropometric and laboratory parameters were measured by standard methods. Hyperinsulinemia was defined by a fasting concentration >75th percentile of values obtained in healthy individuals (107.5 pmol/L)., Results: The prevalence of ATP III-defined metabolic syndrome was 10.1%; it was 28.3% according to EGIR criteria and 15.1% using the IDF definition. The concordance between the definitions was low (kappa coefficient ranging between 0.134 and 0.296). All subjects with EGIR-defined metabolic syndrome had hyperinsulinemia, but only 50% of those with ATP III-defined metabolic syndrome and 62.5% in the IDF metabolic syndrome population had hyperinsulinemia., Conclusions: The inclusion of hyperinsulinemia as a criterion in the EGIR metabolic syndrome definition made it more discriminative than the ATP III definition, both in men and women, and than the IDF definition in men to identify metabolic syndrome in HIV-infected subjects under HAART.

Background: Rotational atherectomy followed by drug-eluting stent (DES) implantation for complex, severely calcified lesions is a rational combination that has not been sufficiently evaluated., Methods: We investigated 102 consecutive patients with angiographic evidence of heavily calcified lesions that underwent DES implantation following rotational atherectomy at our institution between June 2005 and October 2009, and we examined the long-term clinical outcomes. The major adverse cardiac events monitored were death, myocardial infarction and target lesion revascularization., Results: Patients were 68.8 ± 7.4 years old, 52.9% were diabetic, and 12.7% had chronic kidney disease. Forty-seven patients (46.1%) had three-vessel disease, and 13 (12.7%) had left main coronary artery stenosis. The radial approach was used in 37.3% of cases. The procedure was successful in 97%. In-hospital death occurred in 1 patient (0.9%), and 3 patients (2.9%) developed stent thrombosis. At the mean follow-up period of 15 months (range 1- 54), the total cardiac death rate was 4.9%, target lesion revascularization was 8.8% and the incidence of myocardial infarction was 3.9%. The combined endpoint occurred in 12.7% of cases., Conclusion: DES following rotational atherectomy for heavily calcified coronary lesions is a safe and effective procedure that provides good long-term clinical outcomes.

We describe a 37-year-old man with a 4-month history of episodic muscular weakness, involving mainly lower-limbs. Hypokalemia was documented in one episode and managed with intravenous potassium chloride. Hyperthyroidism was diagnosed 4 months after onset of attacks because of mild symptoms. The patient was subsequently diagnosed as having thyrotoxic periodic paralysis associated with Graves' disease. Treatment with propranolol and methimazol was initiated and one year later he remains euthyroid and symptom free. Thyrotoxic periodic paralysis is a rare disorder, especially among Caucasians, but it should always be considered in patients with acute paralysis and hypokalemia, and thyroid function should be evaluated.

Aims: The prognosis of chronic heart failure (CHF) is extremely variable, although generally poor. The purpose of this study was to develop prognostic models for CHF patients., Methods and Results: A cohort of 992 consecutive ambulatory CHF patients was prospectively followed for a median of 44 months. Multivariable Cox models were developed to predict all-cause mortality (n = 267), cardiac mortality (primary end-point, n = 213), pump-failure death (n = 123), and sudden death (n = 90). The four final models included several combinations of the same 10 independent predictors: prior atherosclerotic vascular event, left atrial size >26 mm/m(2), ejection fraction < or =35%, atrial fibrillation, left bundle-branch block or intraventricular conduction delay, non-sustained ventricular tachycardia and frequent ventricular premature beats, estimated glomerular filtration rate <60 mL/min/1.73 m(2), hyponatremia < or =138 mEq/L, NT-proBNP >1.000 ng/L, and troponin-positive. On the basis of Cox models, the MUSIC Risk scores were calculated. A cardiac mortality score >20 points identified a high-risk subgroup with a four-fold cardiac mortality risk., Conclusion: A simple score with a limited number of non-invasive variables successfully predicted cardiac mortality in a real-life cohort of CHF patients. The use of this model in clinical practice identifies a subgroup of high-risk patients that should be closely managed.

Background: Transradial access is the approach of choice in many laboratories for diagnostic coronariography or percutaneous coronary interventions (PCI) because of its lower vascular complication rate than the femoral approach. However, this approach has a higher rate (5-10%) of asymptomatic radial artery (RA) occlusion., Methods: We conducted a prospective, controlled, single-centre trial study. We investigated whether the pneumatic compression (using "TR Band of Terumo") of the radial artery guided by the mean artery pressure (MAP) after the procedure (group A) is efficient in reducing radial occlusion in patients under a coronary angiography by transradial access for diagnostic coronariography or PCI compared to use of standard procedure (group B, 15 cm(3) of air in the device)., Results: The study was suspended after analyzing the results of 50% of the scheduled patients. An analysis of the results of 351 consecutive random patients in Group A showed significantly (P = 0.0001) lower rate (1.1%) of occlusion than Group B (12.0%) without significant differences in the rate of other complications. Other variables associated with RA occlusion in univariate analysis were previous or active smoker (P < 0.04), absence of anti-aggregant treatment (P < 0.04), and the presence of RA flow postprocedure (PFP, P < 0.001). Independent predictors with logistic regression analysis were PFP (HR = 0.06, 95% CI 0.01-0.2), presence of hematoma (HR = 3.7, 95% CI 1.2-11.0), and standard pneumatic compression in group B patients (HR = 18.8, 95% CI 3.8-92.2)., Conclusions: Use of pneumatic compression guided by MAP is safe and efficient, and it can significantly lower the incidence of radial artery occlusion., ((c) 2009 Wiley-Liss, Inc.)

Subclinical hypothyroidism (SH) is a frequent condition that may be associated with increased cardiovascular risk. There is current interest in determining the effect, if any, of substitutive therapy with l-thyroxine (L-T4) on cardiovascular risk factors in SH and, particularly, on those associated with emerging cardiovacular risk, such as apolipoprotein (apo) B, lipoprotein (Lp) (a), total homocysteine (t-Hcy), and C-reactive protein (CRP). Thus, the aim of this study was to assess the impact of euthyroidism restoration on these emerging risk factors in SH. Forty-two patients diagnosed with SH were consecutively recruited before treatment. These patients were treated with L-T4 for 3 to 6 months with the dose necessary to restore euthyroidism. Lp(a), fasting and postmethionine (n = 28) t-Hcy, and CRP did not change with substitutive therapy, regardless of the respective baseline values, and the decrease in apo B paralleled that of low-density lipoprotein (LDL) cholesterol. Similarly, no treatment effect was observed on homocysteine or CRP in patients with thyrotropin-stimulating hormone (TSH) >10 mIU/L. Monitoring of emerging risk factors did not offer additional arguments for treating patients with SH and, thus, is not justified in their clinical management.

Coronary angioplasty is known to mediate an inflammatory response. Recently, we have characterized the transient systemic inflammatory response after coronary stent implantation in patients with unstable angina by measuring different soluble protein markers. In the present study we have characterized the expression of various cellular activation markers in neutrophils, monocytes and lymphocytes from the same group of patients. Peripheral blood samples were taken before and 24 h, 48 h and 7 days after successful coronary stenting in 58 patients. Cell surface markers (CD11b/CD18 and CD38) were analyzed by flow cytometry to determine the activation of neutrophils, monocytes and T lymphocytes. We found that coronary angioplasty with stent implantation produces an increase in the cell surface expression of CD11b/CD18 in neutrophils and CD38 in monocytes, following a similar time-course with a peak after 24 h, returning to basal levels after 48 h and a second peak after 7 days. However, T lymphocytes were not found to be activated. These results suggest that coronary stent implantation induces a different pattern inducing soluble and cellular inflammation markers, and therefore, they should be taken into account in patients undergoing stent implantation to study clinical correlations.

Aims/hypothesis: This study analysed the relationship between congenital malformations (CM) and severity of gestational diabetes mellitus., Methods: A cohort of 2060 infants of mothers with gestational diabetes was studied. Universal screening and 3(rd) Workshop-Conference criteria were used to diagnose gestational diabetes. The severity of diabetes was assessed on the basis of previous hyperglycaemia, blood glucose values in diagnostic OGTT, area under the glucose curve, gestational age and HbA(1)c at diagnosis, insulin requirements during pregnancy, and OGTT after delivery. Potentially confounding variables (age, pre-pregnancy BMI, smoking) were considered. The relationship of potential predictors with CM was analysed with several multivariate logistic regression analyses., Results: The rate of CM was 6% for minor and 3.8% for major malformations (1.4% heart, 0.8% renal/urinary, 0.7% skeletal, 0.3% hypospadias, 0.2% central nervous system, 0.2% cleft lip/palate, 0.1% digestive tract, 0.3% other). In the final models, forward logistic regression analysis identified pre-pregnancy BMI as the predictor of CM (area under receiver operating characteristic curve 0.616); in the backward analysis additional predictors were 1-h blood glucose in diagnostic OGTT and gestational age at diagnosis (area under receiver operating characteristic curve 0.646). Both BMI and severity of gestational diabetes were predictors of heart and minor CM, whereas BMI predicted renal/urinary CM and severity of diabetes predicted skeletal CM., Conclusions/interpretation: In these infants of mothers with gestational diabetes, severity of diabetes and pre-pregnancy BMI were predictors of CM, in accordance with the well-documented pathogenic role of BMI (in the general population) and hyperglycaemia (in diabetic pregnancy). BMI was the main predictor of more prevalent CM.

Objective: To compare the cytologic features of histologically proven lymphocytic (Hashimoto's) thyroiditis (Hashimoto's thyroitidis) and primary thyroid lymphomas (TL)., Study Design: Clinical histories, smears (stained with Diff-Quik, Papanicolaou stain or hematoxylin and eosin [HE]) and surgical specimens (HE slides) were reviewed in 25 cases of lymphocytic thyroiditis and 12 of thyroid lymphomas., Results: Surgical specimens of thyroiditis were obtained for other medical reasons: goiter and compressive symptomatology in 21 cases and neoplasms in 4 (2 papillary carcinomas, 1 follicular carcinoma and 1 oncocytic adenoma). Seven cases were primary lymphomas, and 5 were secondary. Histologically there were 6 large B-cell lymphomas, 2 mantle cell lymphomas, 1 Burkitt lymphoma, 2 mucosal-associated lymphoid tissue lymphomas in blastic transformation and 1 of unknown type. Sensitivity for the diagnosis was 67.5% for HT and 92.3% for lymphoma., Conclusion: A heterogeneous population of small and large lymphocytes was the most frequent pattern in both diseases. The presence of a monotonous population of large lymphocytes or, more rarely, of small cells indicates a probable TL. Plasma cells favor HT. Other techniques are mandatory for the differentiation of cases with inconclusive diagnoses.

Antithyroglobulin antibodies can interfere with the measurement of thyroglobulin yielding spuriously high or low levels depending on the method used. Interference is unrelated to the antibody concentration and can occur at very low concentrations. We report a patient in whom antithyroglobulin antibodies below the cut-off for positivity nearly led to an incorrect diagnosis of thyrotoxicosis factitia.

A 47-year-old woman diagnosed with arrhythmogenic right ventricular cardiomyopathy, was studied at our institution with palpitations, progressive dyspnoea and cyanosis over a 3-year period. Her haemoglobin saturation was 76.1%. The transthoracic echocardiogram showed that both right atrium and ventricle were dilated, with diffuse akinetic and hypokinetic areas. Radionuclide scintigraphy, angiography and magnetic resonance imaging showed dilated and severe dysfunction of the right ventricle, with depressed ejection fraction (0.16), and bulging at the level of the right ventricular anterior wall areas. Doppler examination revealed a right-to-left interatrial shunt through a patent foramen ovale. Contrast transoesophageal echocardiography confirmed the right-to-left shunting. Considering the clinical symptoms were severe and refractory to medical treatment, she was referred for cardiac transplantation. Unfortunately the patient died suddenly a few weeks later.

Previous evidence has shown that coronary angioplasty leads to the release of inflammatory mediators. In this study, we sought to characterize the systemic inflammatory response after coronary stent implantation in patients with unstable angina by measuring different protein markers. Peripheral blood samples were taken before and 24 h, 48 h, and 7 days after successful coronary stenting in 58 patients. Several markers of acute-phase response were determined: C-reactive protein (CRP), alpha2-macroglobulin, haptoglobin, acid alpha1-glycoprotein, prealbumin and albumin. Besides, proinflammatory cytokines (tumor necrosis factor-alpha, IL-6, IL-8) and the anti-inflammatory cytokine IL-10 were also measured. We have found that coronary angioplasty with stent implantation produces a systemic inflammatory response with a rise in inflammation markers concentration. CRP plasma levels declined 1 week after the intervention, but the other marker levels were even higher after 7 days. IL-6 was the only cytokine whose plasma levels significantly increased in peripheral blood after stenting, with a rise after 24 h, maintained after 48 h, and decreased to near-basal levels after 1 week. There was a good correlation between CRP and IL-6 plasma levels (r=0.5, p<0.001). IL-10 levels were slightly decreased after 24 h. Although no significant differences in the means at different time points were found, there was a decrease in IL-10 in most patients 24 h after the intervention. These results indicate that coronary stent implantation induces a systemic inflammatory reaction, with a temporal increase in the concentration of the inflammation markers, especially CRP and IL-6. Since these markers had been previously used as prognostic markers, this needs to be taken into account in patients undergoing stent implantation.

Nitric oxide is an important factor in the regulation of vasodilator tone. In vascular cells, NO is synthesized by endothelial nitric oxide synthase, a key enzyme of the endogenous vasodilator system. Some studies have described the interaction between NO and the other factors that promote vasodilatation in vascular smooth muscular cells. Some of those factors are angiotensin-converting enzyme (ACE), transforming growth factor beta (TGF beta), and endothelial oxide nitric synthase (eNOS). Polymorphism that can alter the expression or the function of the eNOS protein has been identified in the eNOS gene in the promoter and codification zones. We studied the Glu298Asp variant of the eNOS gene in 52 hemodialysis (HD) patients, 22 peritoneal dialysis (PD) patients, and 93 healthy controls. Identification of the Glu298Asp variant in exon 7 was performed by enzymatic amplification and restriction fragment length polymorphism (RFLP) analysis. The frequencies of eNOS genotypes in the control group were GG, 39.8%; GT, 43%; and TT, 17.2%. In HD patients, the frequencies were GG, 40.3%; GT, 38.7%; and TT, 21.7%. In PD patients, they were GG, 41.6%; GT, 50%; and TT, 8.6%. No significant differences were seen between the control group and the dialysis patients, or between the HD and the PD patients.