Catecholaminergic polymorphic ventricular tachycardia. An important diagnosis in children with syncope and normal heart

Syncope in children is primarily related to vagal hyperreactivity, but ventricular tachycardia (VT) way rarely be seen. Catecholaminergic polymorphic VT is a rare entity that can occur in children without heart disease and with a normal QT interval, which may cause syncope and sudden cardiac death. In this report, we describe the clinical features, treatment, and clinical follow-up of three children with syncope associated with physical effort or emotion and cathecolaminergic polymorphic VT. Symptoms were controlled with beta-blockers, but one patient died suddenly in the fourth year of follow-up. Despite the rare occurrence, catecholaminergic polymorphic VT is an important cause of syncope and sudden death in children with no identified heart disease and normal QT interval.

Consequences of the Prolonged Waiting Time for Patients Candidates for Heart Surgery

OBJECTIVE - To assess mortality and the psychological repercussions of the prolonged waiting time for candidates for heart surgery. METHODS - From July 1999 to May 2000, using a standardized questionnaire, we carried out standardized interviews and semi-structured psychological interviews with 484 patients with coronary heart disease, 121 patients with valvular heart diseases, and 100 patients with congenital heart diseases. RESULTS - The coefficients of mortality (deaths per 100 patients/year) were as follows: patients with coronary heart disease, 5.6; patients with valvular heart diseases, 12.8; and patients with congenital heart diseases, 3.1 (p<0.0001). The survival curve was lower in patients with valvular heart diseases than in patients with coronary heart disease and congenital heart diseases (p<0.001). The accumulated probability of not undergoing surgery was higher in patients with valvular heart diseases than in the other patients (p<0.001), and, among the patients with valvular heart diseases, this probability was higher in females than in males (p<0.01). Several patients experienced intense anxiety and attributed their adaptive problems in the scope of love, professional, and social lives, to not undergoing surgery. CONCLUSION - Mortality was high, and even higher among the patients with valvular heart diseases, with negative psychological and social repercussions.

Epidemiological study of congenital heart defects in children and adolescents: analysis of 4,538 cases

OBJECTIVE: To analyze the frequency and prevalence of congenital heart defects in a tertiary care center for children with heart diseases. METHODS: We carried out an epidemiological assessment of the first medical visit of 4,538 children in a pediatric hospital from January 1995 to December 1997. All patients with congenital heart defects had their diagnoses confirmed at least on echocardiography. The frequency and prevalence of the anomalies were computed according to the classification of sequential analysis. Age, weight, and sex were compared between the groups of healthy individuals and those with congenital heart defects after distribution according to the age group. RESULTS: Of all the children assessed, 2,017 (44.4%) were diagnosed with congenital heart disease, 201 (4.4%) with acquired heart disease, 52 (1.2%) with arrhythmias, and 2,268 (50%) were healthy children. Congenital heart diseases predominated in neonates and infants, corresponding to 71.5% of the cases. Weight and age were significantly lower in children with congenital heart defects. Ventricular septal defect was the most frequent acyanotic anomaly, and tetralogy of Fallot was the most frequent cyanotic anomaly. CONCLUSION: Children with congenital heart defects are mainly referred during the neonatal period and infancy with impairment in gaining weight. Ventricular septal defect is the most frequent heart defect.

Investigation Route of the Coronary Patient in the Public Health System in Curitiba, São Paulo and in Incor - IMPACT Study

Background: The investigation of stable coronary artery disease (CAD) and its treatment depend on risk stratification for decision-making on the need for cardiac catheterization and revascularization. Objective: To analyze the procedures used in the diagnosis and invasive treatment of patients with CAD, at the Brazilian Unified Health System (SUS) in the cities of Curitiba, São Paulo and at InCor-FMUSP. Methods: Retrospective, descriptive, observational study of the diagnostic and therapeutic itineraries of the Brazilian public health care system patient, between groups submitted or not to prior noninvasive tests to invasive cardiac catheterization. Stress testing, stress echocardiography, perfusion scintigraphy, catheterization and percutaneous or surgical revascularization treatment procedures were quantified and the economic impact of the used strategies. Results: There are significant differences in the assessment of patients with suspected or known CAD in the metropolitan region in the three scenarios. Although functional testing procedures are most often used the direct costs of these procedures differ significantly (6.1% in Curitiba, 20% in São Paulo and 27% in InCor-FMUSP). Costs related to the procedures and invasive treatments represent 59.7% of the direct costs of SUS in São Paulo and 87.2% in Curitiba. In InCor-FMUSP, only 24.3% of patients with stable CAD submitted to CABG underwent a noninvasive test before the procedure. Conclusion: Although noninvasive functional tests are the ones most often requested for the assessment of patients with suspected or known CAD most of the costs are related to invasive procedures/treatments. In most revascularized patients, the documentation of ischemic burden was not performed by SUS.

Value of the Qrs-T Angle in Predicting the Induction of Ventricular Tachyarrhythmias in Patients with Chagas Disease

Background:The QRS-T angle correlates with prognosis in patients with heart failure and coronary artery disease, reflected by an increase in mortality proportional to an increase in the difference between the axes of the QRS complex and T wave in the frontal plane. The value of this correlation in patients with Chagas heart disease is currently unknown.Objective:Determine the correlation of the QRS-T angle and the risk of induction of ventricular tachycardia / ventricular fibrillation (VT / VF) during electrophysiological study (EPS) in patients with Chagas disease.Methods:Case-control study at a tertiary center. Patients without induction of VT / VF on EPS were used as controls. The QRS-T angle was categorized as normal (0-105º), borderline (105-135º) or abnormal (135-180º). Differences between groups for continuous variables were analyzed with the t test or Mann-Whitney test, and for categorical variables with Fisher's exact test. P values < 0.05 were considered significant.Results:Of 116 patients undergoing EPS, 37.9% were excluded due to incomplete information / inactive records or due to the impossibility to correctly calculate the QRS-T angle (presence of left bundle branch block and atrial fibrillation). Of 72 patients included in the study, 31 induced VT / VF on EPS. Of these, the QRS-T angle was normal in 41.9%, borderline in 12.9% and abnormal in 45.2%. Among patients without induction of VT / VF on EPS, the QRS-T angle was normal in 63.4%, borderline in 14.6% and abnormal in 17.1% (p = 0.04). When compared with patients with normal QRS-T angle, those with abnormal angle had a fourfold higher risk of inducing ventricular tachycardia / ventricular fibrillation on EPS [odds ratio (OR) 4; confidence interval (CI) 1.298-12.325; p = 0.028]. After adjustment for other variables such as age, ejection fraction (EF) and QRS size, there was a trend for the abnormal QRS-T angle to identify patients with increased risk of inducing VT / VF during EPS (OR 3.95; CI 0.99-15.82; p = 0.052). The EF also emerged as a predictor of induction of VT / VF: for each point increase in EF, there was a 4% reduction in the rate of sustained ventricular arrhythmia on EPS.Conclusions:Changes in the QRS-T angle and decreases in EF were associated with an increased risk of induction of VT / VF on EPS.

Efficacy of Patient Selection for Diagnostic Coronary Angiography in Suspected Coronary Artery Disease

AbstractBackground:Guidelines recommend that in suspected stable coronary artery disease (CAD), a clinical (non-invasive) evaluation should be performed before coronary angiography.Objective:We assessed the efficacy of patient selection for coronary angiography in suspected stable CAD.Methods:We prospectively selected consecutive patients without known CAD, referred to a high-volume tertiary center. Demographic characteristics, risk factors, symptoms and non-invasive test results were correlated to the presence of obstructive CAD. We estimated the CAD probability based on available clinical data and the incremental diagnostic value of previous non-invasive tests.Results:A total of 830 patients were included; median age was 61 years, 49.3% were males, 81% had hypertension and 35.5% were diabetics. Non-invasive tests were performed in 64.8% of the patients. At coronary angiography, 23.8% of the patients had obstructive CAD. The independent predictors for obstructive CAD were: male gender (odds ratio [OR], 3.95; confidence interval [CI] 95%, 2.70 - 5.77), age (OR for 5 years increment, 1.15; CI 95%, 1.06 - 1.26), diabetes (OR, 2.01; CI 95%, 1.40 - 2.90), dyslipidemia (OR, 2.02; CI 95%, 1.32 - 3.07), typical angina (OR, 2.92; CI 95%, 1.77 - 4.83) and previous non-invasive test (OR 1.54; CI 95% 1.05 - 2.27).Conclusions:In this study, less than a quarter of the patients referred for coronary angiography with suspected CAD had the diagnosis confirmed. A better clinical and non-invasive assessment is necessary, to improve the efficacy of patient selection for coronary angiography.

Hemorheological and Glycemic Parameters and HDL Cholesterol for the Prediction of Cardiovascular Events

Abstract Background: Hemorheological and glycemic parameters and high density lipoprotein (HDL) cholesterol are used as biomarkers of atherosclerosis and thrombosis. Objective: To investigate the association and clinical relevance of erythrocyte sedimentation rate (ESR), fibrinogen, fasting glucose, glycated hemoglobin (HbA1c), and HDL cholesterol in the prediction of major adverse cardiovascular events (MACE) and coronary heart disease (CHD) in an outpatient population. Methods: 708 stable patients who visited the outpatient department were enrolled and followed for a mean period of 28.5 months. Patients were divided into two groups, patients without MACE and patients with MACE, which included cardiac death, acute myocardial infarction, newly diagnosed CHD, and cerebral vascular accident. We compared hemorheological and glycemic parameters and lipid profiles between the groups. Results: Patients with MACE had significantly higher ESR, fibrinogen, fasting glucose, and HbA1c, while lower HDL cholesterol compared with patients without MACE. High ESR and fibrinogen and low HDL cholesterol significantly increased the risk of MACE in multivariate regression analysis. In patients with MACE, high fibrinogen and HbA1c levels increased the risk of multivessel CHD. Furthermore, ESR and fibrinogen were significantly positively correlated with HbA1c and negatively correlated with HDL cholesterol, however not correlated with fasting glucose. Conclusion: Hemorheological abnormalities, poor glycemic control, and low HDL cholesterol are correlated with each other and could serve as simple and useful surrogate markers and predictors for MACE and CHD in outpatients.

Cardiopathies congénitales de l'âge adulte: nouvelle population de patients, prise en charge multidisciplinaire [Adults with congenital heart diseases: a growing population, a multidisciplinary approach].

Untill recently, congenital heart disease was considered as a childhood's disease. With improvement in pediatric survival, adults with a congenital heart disease (ACHD) represent an emerging group of patients who need specialized medical care. In 2010, the ESC published newguidelines on global and specific management of adults with congenital heart disease. ACHD centers organize appropriate medical care for these patients, promote specialist training and national scientific research in collaboration with other national ACHD centers.

No healthy heart for heavy drinkers: a population-based study in Switzerland

Background: Low to moderate alcohol consumption has been associated with lower coronary heart disease (CHD) risk, an effect mainly mediated by an increase in HDL-cholesterol levels. However, data on the CHD risk associated with high alcohol consumption are conflicting. Methods: In a population-based study of 5,769 men and women, aged 35-75 years, without cardiovascular disease in Switzerland, last week alcohol consumption was categorized into 0, 1-6, 7-13, 14-20, 21-27, 28-34, 035 drinks/week and into nondrinkers (0 drink/week), moderate (1-13), high (14-34) and very high drinkers (035). Blood pressure, lipids and high sensitivity C-reactive protein (hs-CRP) were measured, and the 10-year CHD risk was calculated according to the Framingham risk score. Results: 73% (n = 4,214) of the participants consumed alcohol; 16% (n = 909) were considered as high drinkers and 2% (n = 119) as very high drinkers. In multivariate analysis, increasing alcohol consumption was associated with higher HDL-cholesterol (from 1.57 ± 0.01 [adjusted mean ± SE] in nondrinkers to 1.88 ± 0.03 mmol/L in very high drinkers); triglycerides (1.17 ± 1.01 to 1.32 ± 1.05 mmol/L), and systolic and diastolic blood pressure rose significantly (127.4 ± 0.4 to 132.2 ± 1.4 and 78.7 ± 0.3 to 81.7 ± 0.9 mm Hg, respectively, all p for trend <0.001). Predicted 10-year CHD risk increased from 4.31 ± 0.10 to 4.90 ± 0.37 (p = 0.03) with increasing alcohol use, with a J-shaped relationship. Conclusion: As measured by the 10-year CHD risk, the protective effect of alcohol consumption disappears in very high drinkers, namely because the beneficial increase in HDL-cholesterol may be blunt by a rise in blood pressure levels.

Cell mediated immunity in Chagas' disease: Trypanosoma cruzi antigens induce suppression of the in vitro proliferative response of mononuclear cells

The partial suppression of the cell-mediated immune response by Trypanosoma cruzi antigens in patients with Chagas' disease is demonstrated in a costimulation assay with T. cruzi antigens and Mycobacterium tuberculosis purified protein derivative (PPD) or Tetanus toxoid (TT). ononuclear cells from 13 patients with chagasic infection without evidence of heart disease, 10 patients with chagasic cardiomyopathy and 7 healthy blood donors were stimulated with antigen A (autoclaved epimastigotes), PPD, TT, PPD + A, PPD + TT and TT + A. The average percentage of suppression induced by costimulation of mononuclear cells with PPD and antigen A was 47.1% in patients with chagasic infection without heart disease (INF), 38.8% in patients with chagasic cardiomyopathy (CDM) and 23.3% in healthy controls. Similar values were observed when living trypomastigotes were used. A costimulatory study with PPD and TT, PPD and A and TT and A was carried out in 8 patients with chagasic infection, in order to evaluate the possibility that this difference could be due to a nonspecific inhibitory effect. The mean suppression induced by TT + PPD was -8.9, with TT + A was 52.7 and with PPD + A was 50.1. The data reported show that T. cruzi antigens induce a specific suppression of the proliferative responseof mononuclear cells, that might be relevant to the persistence of the parasite in the host.

Stroke Aphasia: 1,500 Consecutive Cases.

Background: To study the characteristics of vascular aphasia in a cohort of patients with a first-ever stroke. Methods: All patients admitted to the Lausanne neurology department for a first-ever stroke between 1979 and 2004 were included. Neurological examination including language was performed on admission. Stroke risk factors, stroke origin and location, associated symptoms and Rankin scale scores were recorded for each patient. The influence of these factors on aphasia frequency and subtypes was analyzed using logistic regression models. Results: 1,541 (26%) of patients included in this study had aphasia. The more frequent clinical presentations were expressive-receptive aphasia (38%) and mainly expressive aphasia (37%), whereas mainly receptive aphasia was less frequently observed (25%). In ischemic stroke, the frequency of aphasia increased with age (55% of nonaphasic vs. 61% of aphasic patients were more than 65 years old), female sex (40% of women in the nonaphasia group vs. 44% in the aphasia group) and risk factors for cardioembolic origin (coronary heart disease 20 vs. 26% and atrial fibrillation 15 vs. 24%). Stroke aphasia was more likely associated with superficial middle cerebral artery (MCA) stroke and leads to relevant disability. Clinical subtypes depended on stroke location and associated symptoms. Exceptions to the classic clinical-topographic correlations were not rare (26%). Finally, significant differences were found for patients with crossed aphasia in terms of stroke origin and aphasia subtypes. Conclusions: Risk factors for stroke aphasia are age, cardioembolic origin and superficial MCA stroke. Exceptions to classic clinical-topographic correlations are not rare. Stroke aphasia is associated with relevant disability. Stroke location and associated symptoms strongly influence aphasia subtypes.

Evolution of the clinical and epidemiological knowledge about Chagas disease 90 years after its discovery

Three different periods may be considered in the evolution of knowledge about the clinical and epidemiological aspects of Chagas disease since its discovery: (a) early period concerning the studies carried out by Carlos Chagas in Lassance with the collaboration of other investigators of the Manguinhos School. At that time the disease was described and the parasite, transmitters and reservoirs were studied. The coexistence of endemic goiter in the same region generated some confusion about the clinical forms of the disease; (b) second period involving uncertainty and the description of isolated cases, which lasted until the 1940 decade. Many acute cases were described during this period and the disease was recognized in many Latin American countries. Particularly important were the studies of the Argentine Mission of Regional Pathology Studies, which culminated with the description of the Romaña sign in the 1930 decade, facilitating the diagnosis of the early phase of the disease. However, the chronic phase, which was the most important, continued to be difficult to recognize; (c) period of consolidation of knowledge and recognition of the importance of Chagas disease. Studies conducted by Laranja, Dias and Nóbrega in Bambuí updated the description of Chagas heart disease made by Carlos Chagas and Eurico Villela. From then on, the disease was more easily recognized, especially with the emphasis on the use of a serologic diagnosis; (d) period of enlargement of knowledges on the disease. The studies on denervation conducted in Ribeirão Preto by Fritz Köberle starting in the 1950 decade led to a better understanding of the relations between Chagas disease and megaesophagus and other visceral megas detected in endemic areas.

Subclinical hypothyroidism and the risk of heart failure, other cardiovascular events, and death.

BACKGROUND: Subclinical hypothyroidism has been associated with systolic and diastolic cardiac dysfunction and an elevated cholesterol level, but data on cardiovascular outcomes and death are limited. METHODS: We studied 2730 men and women, aged 70 to 79 years, with baseline thyrotropin (TSH) measurements and 4-year follow-up data to determine whether subclinical hypothyroidism was associated with congestive heart failure (CHF), coronary heart disease, stroke, peripheral arterial disease, and cardiovascular-related and total mortality. After the exclusion of participants with abnormal thyroxine levels, subclinical hypothyroidism was defined as a TSH level of 4.5 mIU/L or greater, and was further classified according to TSH levels (4.5-6.9, 7.0-9.9, and > or = 10.0 mIU/L). RESULTS: Subclinical hypothyroidism was present in 338 (12.4%) of the participants. Compared with euthyroid participants, CHF events occurred more frequently among those with a TSH level of 7.0 mIU/L or greater (35.0 vs 16.5 per 1000 person-years; P = .006), but not among those with TSH levels between 4.5 and 6.9 mIU/L. In multivariate analyses, the risk of CHF was higher among those with high TSH levels (TSH of 7.0-9.9 mIU/L: hazard ratio, 2.58 [95% confidence interval, 1.19-5.60]; and TSH of > or = 10.0 mIU/L: hazard ratio, 3.26 [95% confidence interval, 1.37-7.77]). Among the 2555 participants without CHF at baseline, the hazard ratio for incident CHF events was 2.33 (95% confidence interval, 1.10-4.96; P = .03) in those with a TSH of 7.0 mIU/L or greater. Subclinical hypothyroidism was not associated with increased risk for coronary heart disease, stroke, peripheral arterial disease, or cardiovascular-related or total mortality. CONCLUSIONS: Subclinical hypothyroidism is associated with an increased risk of CHF among older adults with a TSH level of 7.0 mIU/L or greater, but not with other cardiovascular events and mortality. Further investigation is warranted to assess whether subclinical hypothyroidism causes or worsens preexisting heart failure.

Integrate Study of a Bolivian Population Infected by Trypanosoma cruzi, the Agent of Chagas Disease

A cross section of a human population (501 individuals) selected at random, and living in a Bolivian community, highly endemic for Chagas disease, was investigated combining together clinical, parasitological and molecular approaches. Conventional serology and polymerase chain reaction (PCR) indicated an active transmission of the infection, a high seroprevalence (43.3%) ranging from around 12% in < 5 years to 94.7% in > 45 years, and a high sensitivity (83.8%) and specificity of PCR. Abnormal ECG tracing was predominant in chagasic patients and was already present among individuals younger than 13 years. SAPA (shed acute phase antigen) recombinant protein and the synthetic peptide R-13 were used as antigens in ELISA tests. The reactivity of SAPA was strongly associated to Trypanosoma cruzi infection and independent of the age of the patients but was not suitable neither for universal serodiagnosis nor for discrimination of specific phases of Chagas infection. Anti-R-13 response was observed in 27.5% only in chagasic patients. Moreover, anti-R13 reactivity was associated with early infection and not to cardiac pathology. This result questioned previous studies, which considered the anti-R-13 response as a marker of chronic Chagas heart disease. The major clonets 20 and 39 (belonging to Trypanosoma cruzi I and T. cruzi II respectively) which circulate in equal proportions in vectors of the studied area, were identified in patients' blood by PCR. Clonet 39 was selected over clonet 20 in the circulation whatever the age of the patient. The only factor related to strain detected in patients' blood, was the anti-R-13 reactivity: 37% of the patients infected by clonet 39 (94 cases) had anti-R13 antibodies contrasting with only 6% of the patients without clonet 39 (16 cases).