Increased Abdominal Perimeter Differently Affects Respiratory Function in Men and Women.

Obesity is a worldwide epidemic being the main cause of cardiovascular, metabolic disturbances and chronic pulmonary diseases. The increase in body weight may affect the respiratory system due to fat deposition and systemic inflammation. Herein, we evaluated the sex differences in the impact of obesity and high abdominal circumference on basal ventilation. Thirty-five subjects, 23 women and 12 men with a median age of 61 and 67, respectively, were studied and classified as overweight and obese according to body mass index (BMI) and were also divided by the abdominal circumference. Basal ventilation, namely, respiratory frequency, tidal volume, and minute ventilation, was evaluated. In normal and overweight women, basal ventilation did not change, but obese women exhibited a decrease in tidal volume. In men, overweight and obese subjects did not exhibit altered basal ventilation. In contrast, when subjects were subdivided based on the abdominal perimeter, a higher circumference did not change the respiratory frequency but induced a decrease in tidal volume and minute ventilation in women, while in men these two parameters increased. In conclusion, higher abdominal circumference rather than BMI is associated with alterations in basal ventilation in women and men.

Associations between perfusion defects, tissue changes and myocardial deformation in hypertrophic cardiomyopathy, uncovered by a cardiac magnetic resonance segmental analysis.

BACKGROUND: Microvascular dysfunction is an often overlooked feature of hypertrophic cardiomyopathy (HCM). Our aim was to assess the association between microvascular dysfunction, wall thickness, tissue characteristics and myocardial deformation in HCM patients, by analyzing individual myocardial segments. METHODS: Prospective assessment including cardiac magnetic resonance to assess wall thickness, T1 and T2 mapping, extracellular volume, late gadolinium enhancement (LGE) and stress perfusion. Results were stratified according to the 16 American Heart Association segments. RESULTS: Seventy-five patients were recruited (1200 segments), 63% male, mean age 54.6±14.8 years, maximal wall thickness of 20.22±4.6 mm. Among the 424 segments (35%) with perfusion defects, 24% had defects only in the endocardial layer and 12% in both endocardial and epicardial layers. Perfusion defects were more often detected in hypertrophied segments (64%). Among the 660 segments with normal wall thickness, 19% presented perfusion defects. Independently of wall thickness, segments with perfusion defects had a higher T1 (ß-estimate 30.28, p<0.001), extracelluar volume (ß-estimate 1.50, p<0.001) and T2 (ß-estimate 0.73, p<0.001) and had late gadolinium enhancement more frequently (odds ratio 4.16, p<0.001). Higher values of circumferential strain (lower deformation) and lower values of radial strain were found in segments with perfusion defects (ß-estimate 2.76, p<0.001; and ß-estimate -10.39, p<0.001, circumferential and radial strain, respectively). CONCLUSION: While microvascular dysfunction was more prevalent in more hypertrophied segments, it also had a major presence in segments without hypertrophy. In this segmental analysis, we found an association between the presence of ischemia and tissue abnormalities, replacement fibrosis as well as impaired strain, independently of the segmental wall thickness.

Index of microcirculatory resistance in the assessment of coronary microvascular dysfunction in hypertrophic cardiomyopathy.

INTRODUCTION AND OBJECTIVES: Coronary microvascular dysfunction (CMD) is one of the most important pathophysiological features in hypertrophic cardiomyopathy (HCM). The index of microcirculatory resistance (IMR) is an invasive method to assess the coronary microcirculation. The aim was to assess CMD in patients with HCM by IMR. METHODS: Adult patients with HCM without epicardial coronary artery disease underwent cardiac catheterization for the assessment of CMD by IMR (normal cut-off value ≤22.0) and coronary flow reserve (CFR) (normal cut-off value ≥2). Cardiovascular magnetic resonance (CMR) was performed to assess the ischemic burden by perfusion imaging during regadenoson-induced hyperemia, and the extent of myocardial fibrosis was assessed by late gadolinium enhancement (LGE), native T1 mapping and extracellular volume (ECV). RESULTS: Fourteen patients were enrolled with a mean age of 62.8±6.2years, 8 (57.1%) males, of whom 9 (64.3%) had obstructive HCM. Using IMR, CMD was detected in 4 (29%) patients. Among four patients with an IMR>22.0, all had non-obstructive HCM and two had angina. CFR<2 was reported in eight patients (57%). Concordance between IMR and CFR (both normal or both abnormal) was verified in 6 patients (43%). Among four patients with IMR>22.0, perfusion defects were found in two of the three patients who underwent stress CMR. Increased ECV (>28%) was documented in two of the patients with IMR>22 and in three of the patients with IMR≤22.0. LGE was >15% in 2 of the patients with IMR>22 and in 4 with IMR≤22.0. CONCLUSIONS: IMR assessment in HCM is feasible and safe. Patients with abnormal IMR seemed to have more significant tissue abnormalities on CMR.

Association between miR-146a and Tumor Necrosis Factor Alpha (TNF-α) in Stable Coronary Artery Disease.

Background and Objectives: Tumor necrosis factor alpha (TNF-α) is proatherogenic and associated with the risk of acute ischemic events, although the mechanisms that regulate TNF-α expression in stable coronary artery disease (SCAD) are not fully understood. We investigated whether metabolic, inflammatory, and epigenetic (microRNA (miRNA)) markers are associated with TNF-α expression in SCAD. Materials and Methods: Patients with SCAD were prospectively recruited and their metabolic and inflammatory profiles were assessed. TNF-α levels were assessed using an enzyme-linked immunosorbent assay. The relative expression of six circulating miRNAs associated with the regulation of inflammation and/or atherosclerosis was determined. Results: Of the 24 included patients with the mean age of 65 (9) years, 88% were male, and 54% were diabetic. The TNF-α levels were (median (interquartile range)) 1.0 (0.7-1.1) pg/mL. The percentage of glycosylated hemoglobin (r = 0.418, p = 0.042), serum triglyceride levels (r = 0.429, p = 0.037), and C-reactive protein levels (r = 0.407, p = 0.048) were positively correlated with TNF-α levels. Of the candidate miRNAs, miR-146a expression levels were negatively correlated with TNF-α levels (as indicated by r = 0.500, p = 0.035 for correlation between delta cycle threshold (ΔCt) miR-146a and TNF-α levels). In multivariate analysis, serum triglyceride levels and miR-146a expression levels were independently associated with TNF-α levels. miR-146 expression levels were not associated with metabolic or other inflammatory parameters and were negatively correlated with the number of coronary vessels with obstructive disease (as indicated by r = 0.556, p = 0.017 for correlation between ΔCt miR-146a and number of diseased vessels). Conclusions: miR-146a expression levels were negatively correlated with TNF-α levels in patients with SCAD, irrespective of other metabolic or inflammatory markers, and with the severity of coronary artery disease. The results add to the knowledge on the role of miR-146a in TNF-α-based inflammation in SCAD and support future research on the potential therapeutic use of miR-146a in such a clinical scenario.

Coronary microvascular dysfunction in hypertrophic cardiomyopathy: Pathophysiology, assessment, and clinical impact.

Myocardial ischemia constitutes one of the most important pathophysiological features in hypertrophic cardiomyopathy. Chronic and recurrent myocardial ischemia leads to fibrosis, which may culminate in myocardial dysfunction. Since the direct visualization of coronary microcirculation in vivo is not possible, its function must be studied indirectly. Invasive and noninvasive techniques allow microcirculatory dysfunction to be evaluated, including echocardiography, magnetic resonance, positron emission tomography, and cardiac catheterization. Blunted myocardial blood flow and coronary flow reserve have been suggested to associate with unfavorable prognosis. Microcirculatory dysfunction may be one additional important parameter to take into account for risk stratification beyond the conventional risk factors.

The Impact of Ischemia Assessed by Magnetic Resonance on Functional, Arrhythmic, and Imaging Features of Hypertrophic Cardiomyopathy.

Aims: The aim of the study is to investigate the association between the degree of ischemia due to coronary microvascular dysfunction (CMD) and the left ventricular (LV) tissue characteristics, systolic performance, and clinical manifestations in hypertrophic cardiomyopathy (HCM). Methods and Results: This prospective study enrolled 75 patients with HCM without obstructive epicardial coronary artery disease. Each patient underwent cardiovascular magnetic resonance (CMR) including parametric mapping, perfusion imaging during regadenoson-induced hyperemia, late gadolinium enhancement (LGE) and three-dimensional longitudinal, circumferential, and radial strains analysis. Electrocardiogram, 24-h Holter recording, and cardiopulmonary exercise testing (CPET) were performed to assess arrhythmias and functional capacity. In total, 47 (63%) patients were men with the mean age of 54.6 (14.8) years, 51 (68%) patients had non-obstructive HCM, maximum wall thickness (MWT) was 20.2 (4.6) mm, LV ejection fraction (LVEF) was 71.6 (8.3%), and ischemic burden was 22.5 (16.9%) of LV. Greater MWT was associated with the severity of ischemia (ß-estimate:1.353, 95% CI:0.182; 2.523, p = 0.024). Ischemic burden was strongly associated with higher values of native T1 (ß-estimate:9.018, 95% CI:4.721; 13.315, p < 0.001). The association between ischemia and LGE was significant in following subgroup analyses: MWT 15-20 mm (ß-estimate:1.941, 95% CI:0.738; 3.143, p = 0.002), non-obstructive HCM (ß-estimate:1.471, 95% CI:0.258; 2.683, p = 0.019), women (ß-estimate:1.957, 95% CI:0.423; 3.492, p = 0.015) and age <40 years (ß-estimate:4.874, 95% CI:1.155; 8.594, p = 0.016). Ischemia in ≥21% of LV was associated with LGE >15% (AUC 0.766, sensitivity 0.724, specificity 0.659). Ischemia was also associated with atrial fibrillation or flutter (AF/AFL) (OR-estimate:1.481, 95% CI:1.020; 2.152, p = 0.039), but no association was seen for non-sustained ventricular tachycardia. Ischemia was associated with shorter time to anaerobic threshold (ß-estimate: -0.442, 95% CI: -0.860; -0.023, p = 0.039). Conclusion: In HCM, ischemia associates with morphological markers of severity of disease, fibrosis, arrhythmia, and functional capacity.

Carotid body chemosensitivity: early biomarker of dysmetabolism in humans.

OBJECTIVE: The carotid bodies (CBs) are peripheral chemoreceptor organs classically described as being O2 sensors, which are increasingly emerging as core players in metabolic control. Herein we evaluated CB activity in prediabetes patients and determined its correlation with dysmetabolism clinical features. DESIGN AND METHODS: Prediabetes patients were recruited at the Cardiology Service, Hospital Santa Marta, Centro Hospitalar Lisboa Central, EPE (CHLC-EPE). The study was approved by CHLC-EPE and NOVA Medical School Ethics Committee. Thirty-three prediabetic and 14 age-matched, non-prediabetic, volunteers had their peripheral chemosensitivity evaluated by the Dejours test. Serum biomarkers of metabolic disease, insulin sensitivity (HOMA-IR), blood pressure, carotid intima-media thickness (cIMT) and glucose tolerance were assessed. RESULTS: CB chemosensitivity was significantly increased in prediabetic group (P < 0.01). Fasting blood, glucose intolerance, fasting insulin and HOMA-IR were significantly higher in prediabetes patients. Insulin resistance correlated both with peripheral chemosensitivity, assessed by the Dejours test (P < 0.05) and with abdominal circumference (P < 0.01). HbA1c correlated with HOMA-IR (P < 0.05) and left cIMT (P < 0.05) in prediabetes patients. CONCLUSIONS: We conclude that CB is overactive in prediabetes subjects and that peripheral chemosensitivity correlates with fasting insulin and insulin resistance representing a novel non-invasive functional biomarker to forecast early metabolic disease.

Is metabolic syndrome a prognostic marker in patients at high cardiovascular risk? A long-term cohort study. / Será a síndrome metabólica um marcador de prognóstico em doentes com elevado risco cardiovascular? Um estudo de coorte a longo­prazo.

INTRODUCTION: Obese patients are at higher cardiovascular risk in primary prevention. In secondary prevention, an obesity paradox has been reported. We analyzed a cohort of individuals from a previous cross-sectional study on the impact of metabolic syndrome (MS) on coronary artery disease (CAD), aiming to assess the occurrence of cardiovascular events in a long follow-up. METHODS: We analyzed 296 individuals in a mean follow-up of 6.9±2.2 years. Subjects were divided into four groups according to the presence of MS or CAD (defined as ≥70% coronary stenosis). RESULTS: The study population had a mean age of 65±9 years at the beginning of the study; 59.5% were male, 55.7% had MS and 41.6% had CAD. During follow-up 10.1% of the population suffered all-cause death, 3.7% cardiovascular death, 14.2% cardiovascular readmission and 22.0% the composite outcome (mortality, acute coronary syndrome, coronary revascularization, stroke/transient ischemic attack or heart failure admission). There were no significant differences in any type of mortality. Patients with CAD had more readmissions and composite outcomes (log-rank p<0.001 and p=0.001, respectively), but there was no difference according to the presence of MS. Only CAD was an independent predictor of cardiovascular admission (HR 3.21, 95% CI 1.66-6.21) and composite outcomes (HR 2.41, 95% CI 1.44-4.02). CONCLUSIONS: In patients with high cardiovascular risk or established CAD, the presence of MS is not associated with cerebral or cardiac events in long-term follow-up.

Has carotid intima-media thickness prognostic impact in patients with high cardiovascular risk? A long-term cohort study.

BACKGROUND: Carotid intima-media thickness (CIMT) is an established surrogate marker for cardiovascular events in patients with intermediate risk. In patients with high cardiovascular risk or established cardiovascular disease, the impact of CMIT measurement on risk stratification for future events is less clear. Our objective was to evaluate the impact of CIMT on the occurrence of cardiovascular events in a cohort of individuals with high cardiovascular risk, in long-term follow-up. METHODS: We analyzed 296 individuals, mean follow-up of 6.9 ± 2.2 years. Individuals were divided into tertiles according to CIMT. Tertiles were compared in terms of baseline characteristics and outcomes during follow-up-all-cause mortality and composite outcome (mortality, acute coronary syndromes, coronary revascularization, stroke/transient ischemic attack, heart failure, or cardiovascular admission). RESULTS: Our population had a mean age of 65 ± 9 years at the beginning of the study, 55% males. Patients with higher CIMT showed a trend for higher cardiovascular mortality (P = 0.084) and for the composite outcome (P = 0.049). A CIMT ≥ 0.85 mm was also associated with higher rate of events; however, CIMT was not an independent predictor of outcome after adjustment for age and gender. CIMT assessment was useful in patients with hypertension, hyperlipidemia, and metabolic syndrome and in nondiabetic patients. For the composite outcome, it was also useful in females, smokers, and in patients without coronary artery disease. CONCLUSIONS: Patients with higher CIMT have worst outcome, but this was mainly driven by age and gender. CIMT is useful as a prognostic marker in specific subsets of patients.

Circulating microRNA profiles in different arterial territories of stable atherosclerotic disease: a systematic review.

AIMS: Atherosclerosis is associated with altered circulating microRNA profiles. It is yet unclear whether the expression of these potential biomarkers differs according to the location of atherosclerosis. We assessed whether atherosclerosis of different arterial territories, except the coronary, is associated with specific circulating microRNA profiles. METHODS: A systematic search in PubMed, Web of Science, Embase, and Cochrane Library was carried out using a retrieval strategy including MESH and non-MSH terms. Eligible studies have compared circulating microRNA profiles between individuals with and without stable atherosclerotic disease of large or medium size arteries. The review protocol was registered in PROSPERO database (reference CRD42017073846). RESULTS: Eighteen studies were selected for qualitative synthesis: ten focused on carotid, six on lower limbs, and two on renal arteries atherosclerosis, none reporting on other locations. A common microRNA profile to different atherosclerotic disease locations was identified, including deregulation of miR-21, miR-30, miR-126, and miR-221-3p. Specific microRNA profiles for each territory were also identified, with consistency across studies, such as deregulation of miR-21 and miR-29 in carotid atherosclerosis, and let 7e, miR-27b, miR-130a, and miR-210 in lower limbs atherosclerosis. The robustness of the results was very high for let 7e, miR-29, miR-30, considering both the adjustment of microRNA expression for baseline variables and the replication of results in different studies (miR-29 in carotid, let 7e in lower limbs, and miR-30 in carotid and lower limbs atherosclerosis). Globally, the deregulated microRNAs are associated with control of angiogenesis, endothelial cell function, inflammation, cholesterol metabolism, oxidative stress and extracellular matrix composition. CONCLUSIONS: A common microRNA profile to different atherosclerotic disease locations and specific microRNA profiles for each territory were identified. These findings may provide insights into pathophysiology and be useful for selecting potential biomarkers for clinical practice. To the best of our knowledge, no systematic data on this subject has been reported.

Impact of cardiac resynchronization therapy on inflammatory biomarkers and cardiac remodeling: The paradox of functional and echocardiographic response.

INTRODUCTION: Response to cardiac resynchronization therapy (CRT) can currently be assessed by clinical or echocardiographic criteria, and there is no strong evidence supporting the use of one rather than the other. Reductions in B-type natriuretic peptide (BNP) and C-reactive protein (CRP) have been shown to be associated with CRT response. This study aims to assess variation in BNP and CRP six months after CRT and to correlate this variation with criteria of functional and echocardiographic response. METHODS: Patients undergoing CRT were prospectively enrolled between 2011 and 2014. CRT response was defined by echocardiography (15% reduction in left ventricular end-systolic volume) and by cardiopulmonary exercise testing (10% increase in peak oxygen consumption) from baseline to six months after device implantation. RESULTS: A total of 115 patients were enrolled (68.7% male, mean age 68.6±10.5 years). Echocardiographic response was seen in 51.4% and 59.2% were functional responders. There was no statistical correlation between the two. Functional response was associated with a significantly greater reduction in BNP (-167.6±264.1 vs. -24.9±269.4 pg/ml; p=0.044) and CRP levels (-1.6±4.4 vs. 2.4±9.9 mg/l; p=0.04). Nonetheless, a non-significant reduction in BNP and CRP was observed in echocardiographic responders (BNP -144.7±260.2 vs. -66.1±538.2 pg/ml and CRP -7.1±24.3 vs. 0.8±10.3 mg/l; p>0.05). CONCLUSION: An increase in exercise capacity after CRT implantation is associated with improvement in myocardial remodeling and inflammatory biomarkers. This finding highlights the importance of improvement in functional capacity after CRT implantation, not commonly considered a criterion of CRT response.

Does permanent atrial fibrillation modify response to cardiac resynchronization therapy in heart failure patients?

INTRODUCTION: The benefits of cardiac resynchronization therapy (CRT) documented in heart failure (HF) may be influenced by atrial fibrillation (AF). We aimed to compare CRT response in patients in AF and in sinus rhythm (SR). METHODS: We prospectively studied 101 HF patients treated by CRT. Rates of clinical, echocardiographic and functional response, baseline NYHA class and variation, left ventricular ejection fraction, volumes and mass, atrial volumes, cardiopulmonary exercise test (CPET) duration (CPET dur), peak oxygen consumption (VO2max) and ventilatory efficiency (VE/VCO2 slope) were compared between AF and SR patients, before and at three and six months after implantation of a CRT device. RESULTS: All patients achieved ≥95% biventricular pacing, and 5.7% underwent atrioventricular junction ablation. Patients were divided into AF (n=35) and SR (n=66) groups; AF patients were older, with larger atrial volumes and lower CPET dur and VO2max before CRT. The percentages of clinical and echocardiographic responders were similar in the two groups, but there were more functional responders in the AF group (71% vs. 39% in SR patients; p=0.012). In SR patients, left atrial volume and left ventricular mass were significantly reduced (p=0.015 and p=0.021, respectively), whereas in AF patients, CPET dur (p=0.003) and VO2max (p=0.001; 0.083 age-adjusted) showed larger increases. CONCLUSION: Clinical and echocardiographic response rates were similar in SR and AF patients, with a better functional response in AF. Improvement in left ventricular function and volumes occurred in both groups, but left ventricular mass reduction and left atrial reverse remodeling were seen exclusively in SR patients (ClinicalTrials.gov identifier: NCT02413151; FCT code: PTDC/DES/120249/2010).

Predictors of response to cardiac resynchronization therapy: A prospective cohort study.

INTRODUCTION: Cardiac resynchronization therapy (CRT) has modified the prognosis of chronic heart failure (HF) with left ventricular systolic dysfunction. However, 30% of patients do not have a favorable response. The big question is how to determine predictors of response. AIMS: To identify baseline characteristics that might influence echocardiographic response to CRT. METHODS AND RESULTS: We performed a prospective single-center hospital-based cohort study of consecutive HF patients selected to CRT (NYHA class II-IV, left ventricular ejection fraction (LVEF) <35% and QRS complex ≥120 ms). Responders were defined as those with a ≥5% absolute increase in LVEF at six months. Clinical, electrocardiographic, laboratory, echocardiographic, autonomic, endothelial and cardiopulmonary function parameters were assessed before CRT device implantation. Logistic regression models were used. Seventy-nine patients were included, 54 male (68.4%), age 68.1 years (standard deviation 10.2), 19 with ischemic etiology (24%). At six months, 51 patients (64.6%) were considered responders. Although by univariate analysis baseline tricuspid annular plane systolic excursion (TAPSE) and serum creatinine were significantly different in responders, on multivariate analysis only TAPSE was independently associated with response, with higher values predicting a positive response to CRT (OR=1.13; 95% CI: 1.02-1.26; p=0.020). TAPSE ≥15 mm was strongly associated with response, and TAPSE <15 mm with non-response (p=0.005). Responders had no TAPSE values below 10 mm. CONCLUSION: From a range of clinical and technical baseline characteristics, multivariate analysis only identified TAPSE as an independent predictor of CRT response, with TAPSE <15 mm associated with non-response. This study highlights the importance of right ventricular dysfunction in CRT response. ClinicalTrials.gov identifier: NCT02413151.

Global and regional patterns of longitudinal strain in screening for chemotherapy-induced cardiotoxicity. / Importância da deformação longitudinal na deteção da cardiotoxicidade induzida por quimioterapia e na identificação de padrões específicos de afetação segmentar.

INTRODUCTION: Serial echocardiographic assessment of left ventricular ejection fraction (LVEF) is the gold standard in screening for chemotherapy-induced cardiotoxicity (CIC). Measurement of myocardial deformation using speckle tracking enables more detailed assessment of myocardial contractility. The aim of this study was to determine the relationship between global and regional longitudinal strain and CIC. METHODS: This was a prospective study of 158 breast cancer patients undergoing chemotherapy with anthracyclines with or without adjuvant trastuzumab who underwent serial monitoring by transthoracic echocardiography with assessment of myocardial deformation. CIC was defined as a decrease in LVEF to <53%. Global longitudinal strain (GLS) was estimated using EchoPAC BT12 software on a GE Vivid E9 cardiac ultrasound system. Patients were classified according to the 2015 ASE/EACVI criteria as having impaired myocardial deformation when GLS was reduced (less negative), with a cutoff of -18%. RESULTS: During a mean follow-up of 5.4 months (1-48 months), the incidence of CIC was 18.9%. A decrease in GLS was observed during follow-up for the entire cohort (baseline GLS -20.1±3.5% vs. -18.7±3.4% at last follow-up assessment, p=0.001). A total of 97 patients (61.4%) were observed to have impaired myocardial deformation (GLS ≥18%) at some point during follow-up. This decrease was more significant in patients who eventually developed CIC (GLS -17.2±2.5%, p=0.02). On analysis of regional strain, impaired contractility was observed in the septal (6 out of 6) and anterior (2 out of 3) segments. Multivariate logistic regression showed that patients who developed impaired longitudinal strain had a 4.9-fold increased risk of developing CIC (odds ratio 4.88, confidence interval 1.32-18.0, p=0.017). CONCLUSIONS: Worsening of myocardial deformation as assessed by speckle tracking is common in breast cancer patients undergoing chemotherapy, with predominantly septal and anterior wall involvement. Impaired myocardial deformation was independently associated with increased incidence of CIC.

Twelve Years of Kawasaki Disease in Portugal: Epidemiology in Hospitalized Children.

BACKGROUND: Kawasaki disease (KD) is the leading cause of acquired heart disease in developed countries. Reported incidences vary worldwide but incidence of KD has not been established in Portugal. AIM: The aims of the study were to describe the epidemiologic characteristics and estimate incidence rates of KD among hospitalized children in Portugal. METHODS: This study was a descriptive, population-based study, which used hospital discharge records of patients <20 years of age diagnosed with KD from the Hospital Register database for 2000-2011. Incidence rates were calculated using the number of KD patients and corresponding National census data. RESULTS: There were 533 hospitalizations of 470 patients with KD as the primary diagnosis in Portugal, 63 hospitalizations were transfers of patients between hospitals and there were no relapses. The mean age at admission was 2.8 years, with male predominance (male-to-female ratio: 1.6:1). Children <5 years and infants <1 year represented 83% and 23% of all the patients admitted, respectively. Mean annual incidence was 6.5 per 100,000 children <5 years, 4.5 per 100,000 infants <1 year and 7.8 per 100,000 infants 1-4 years. We found considerable differences between national territorial regions, with majority of cases in most dense regions. The mean length of hospital stay was 9 days, and the incidence peaked in spring (35%) and spring/winter (63%). Coronary aneurysms were reported in 8.5% of patients with a higher male-to-female ratio (3.4:1) and a lower mean age (1.93 years). Reported mortality was 0.4%. CONCLUSIONS: This is the first large-scale epidemiologic study of KD in Portugal. The highest incidences occurred among male children 1-4 years of age and in spring/winter.

Prognostic value of VEGF in patients submitted to percutaneous coronary intervention.

We examined the longitudinal changes of VEGF levels after percutaneous coronary intervention for predicting major adverse cardiac events (MACE) in coronary artery disease (CAD) patients. VEGF was measured in 94 CAD patients' serum before revascularization, 1-month and 1-year after. Independently of clinical presentation, patients had lower VEGF concentration than a cohort of healthy subjects (median, IQ: 15.9, 9.0-264 pg/mL versus 419, 212-758 pg/mL; P < 0.001) at baseline. VEGF increased to 1-month (median, IQ: 276, 167-498 pg/mL; P < 0.001) and remained steady to 1-year (median, IQ: 320, 173-497 pg/mL; P < 0.001) approaching control levels. Drug eluting stent apposition and previous medication intake produced a less steep VEGF evolution after intervention (P < 0.05). Baseline VEGF concentration <40.8 pg/mL conveyed increased risk for MACE in a 5-year follow-up. Results reflect a positive role of VEGF in recovery and support its importance in CAD prognosis.

[Non-obstructive coronary artery disease documented by cardiac computed tomography: Discrepancy between atherosclerotic burden and cardiovascular risk]. / Doença coronária não obstrutiva documentada por tomografia computorizada cardíaca: contraste entre a carga aterosclerótica e o risco cardiovascular.

Cardiac computed tomography (CT) documents the presence of coronary artery disease, regardless of the degree of stenosis. The prognostic value of non-obstructive coronary artery disease documented by cardiac CT has recently been validated. However, there are still no clear guidelines on the management of such patients, particularly concerning initiation of more aggressive pharmacological measures for primary prevention. The approach to these patients remains controversial, especially in cases in which there is a discrepancy between cardiovascular risk and the atherosclerotic burden as documented by cardiac CT. The authors describe the case of a patient with a discrepancy between the extent of documented coronary atherosclerosis and that estimated according to pretest probability and cardiovascular risk scores. As this individual had more severe coronary atherosclerosis than expected (calcium score above the 90th percentile and non-obstructive coronary artery disease on cardiac CT) but was a competitive athlete and otherwise asymptomatic and without risk factors or cardiovascular history, with a very low estimated cardiovascular risk, it was difficult to decide on the risks and benefits of pharmacological primary prevention.

Coronary computed tomography angiography-adapted Leaman score as a tool to noninvasively quantify total coronary atherosclerotic burden.

To describe a coronary computed tomography angiography (CCTA)-adapted Leaman score (CT-LeSc) as a tool to quantify total coronary atherosclerotic burden with information regarding localization, type of plaque and degree of stenosis and to identify clinical predictors of a high coronary atherosclerotic burden as assessed by the CT-LeSc. Single center prospective registry including a total of 772 consecutive patients undergoing CCTA (Dual-source CT) from April 2011 to March 2012. For the purpose of this study, 581 stable patients referred for suspected coronary artery disease (CAD) without previous myocardial infarction or revascularization procedures were included. Pre-test CAD probability was determined using both the Diamond-Forrester extended CAD consortium method (DF-CAD consortium model) and the Morise score. Cardiovascular risk was assessed with the HeartScore. The cut-off for the 3rd tercile (CT-LeSc ≥8.3) was used to define a population with a high coronary atherosclerotic burden. The median CT-LeSc in this population (n = 581, 8,136 coronary segments evaluated; mean age 57.6 ± 11.1; 55.8 % males; 14.6 % with diabetes) was 2.2 (IQR 0-6.8). In patients with CAD (n = 341), the median CT-LeSc was 5.8 (IQR 3.2-9.6). Among patients with nonobstructive CAD, most were classified in the lowest terciles (T1, 43.0 %; T2, 36.1 %), but 20.9 % were in the highest tercile (T3). The majority of the patients with obstructive CAD were classified in T3 (78.2 %), but 21.8 % had a CT-LeSc in lower terciles (T1 or T2). The independent predictors of a high CT-LeSc were: Male sex (OR 1.73; 95 % CI 1.04-2.90) diabetes (OR 2.91; 95 % CI 1.61-5.23), hypertension (OR 2.54; 95 % CI 1.40-4.63), Morise score ≥ 16 (OR 1.97; 95 % CI 1.06-3.67) and HeartScore ≥ 5 (OR 2.42; 95 % CI 1.41-4.14). We described a cardiac CT adapted Leaman score as a tool to quantify total (obstructive and nonobstructive) coronary atherosclerotic burden, reflecting the comprehensive information about localization, degree of stenosis and type of plaque provided by CCTA. Male sex, hypertension, diabetes, a HeartScore ≥5 % and a Morise score ≥ 16 were associated with a high coronary atherosclerotic burden, as assessed by the CT-LeSc. About one fifth of the patients with nonobstructive CAD had a CT-LeSc in the highest tercile, and this could potentially lead to a reclassification of the risk profile of this subset of patients identified by CCTA, once the prognostic value of the CT-LeSc is validated.

Marfan syndrome with ascending aortic aneurysm: value of cardiac computed tomography.

We report the case of a 40-year-old man with known Marfan syndrome who presented with severe aortic valve regurgitation secondary to significant aortic root dilatation. To rule out coronary artery disease and to evaluate the rest of the thoracic aorta before surgery, cardiac computed tomography (CT) was performed. A brief review of the literature shows how cardiac CT can, in selected cases, rule out coronary artery disease before non-coronary cardiothoracic surgery.

Long-term evaluation of endothelial function in Kawasaki disease patients.

BACKGROUND: Kawasaki disease is an acute systemic vasculitis. Cardiac complications are frequent and include endothelial dysfunction in patients with coronary anomalies. So far, the presence of endothelial dysfunction in patients with no coronary lesions has not been demonstrated. Peripheral arterial tonometry (Endo-PAT) measures the microvascular function in response to local ischaemia and has been validated in adult population, but its use in children is scarce. Aim To evaluate endothelial dysfunction in children as a long-term complication after Kawasaki disease using Endo-PAT. METHODS: We evaluated two groups of subjects: (1) Kawasaki disease patients over 11 years of age, diagnosed for >5 years, with no coronary lesions, or any other risk factors for cardiovascular disease; (2) control group of individuals without cardiovascular risk factors. Patients and controls were clinically accessed. Endo-PAT was performed to determine reactive hyperaemia index and augmentation index. RESULTS: A total of 35 individuals (21 males, age 21 ± 6 years) were evaluated (group 1: 19; controls: 16). Kawasaki disease patients presented significant lower reactive hyperaemia index (1.68 ± 0.49 versus 2.31 ± 0.53; p = 0.001). Augmentation index was similar in both groups (-10 ± 7 versus -11 ± 5; p > 0.005). Most patients with Kawasaki disease disclosed endothelial dysfunction (68%) compared with only 12% in controls. CONCLUSIONS: Endo-PAT is feasible and reproducible in the child population. Endothelial dysfunction is a frequent long-term complication in patients after Kawasaki disease with normal appearing coronary arteries. However, these results need validation in a larger population.