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Background In tetralogy of Fallot, correction surgery is a priority choice, seeing that it is desirable to minimize pulmonary hypoflow and severe hypoxemia, which result in hypoxemic crises, with sudden worsening of cyanosis, tachypnea, and, in some cases, loss of consciousness, seizures, and even death. Objective To evaluate the predicting factors of surgical mortality in children and adolescents undergoing correction of tetralogy of Fallot. Methods Retrospective cross-sectional study carried out by consulting all surgical records of the Child and Adolescent Cardiology Service of the Brazilian National Institute of Cardiology, during the period from 2007 to 2010. Results with p values < 0.05 were considered significant. Results The study evaluated 93 medical records. In relation to the characteristics of the population at the time of surgery, the median age was 3.69 (2.13 to 5.79) years, and 58.06% (n = 54) were male. White was the most common skin color, accounting for 55.43% (n = 51) of cases. In relation to assessment of nutritional status, median weight was 13.25 (10.10 to 17.60) kg, and body mass index was 14.49 (13.44 to 16.28) kg/m2. Down syndrome was present in 11.83% (n = 11) of the patients. All patients underwent correction surgery (n = 93, 100%). Prior cyanotic crisis was found in 53.85% (n = 49) with p = 0.013; surgical procedure duration was 218.83 ± 60.63 minutes, with p = 0.003, and lactate was 1.88 ± 1.33 mg/dL during the immediate postoperative period, with p = 0.009. Regarding the outcome of surgical death, it was found in 15.05% (n = 14) of patients. Mean follow-up lasted 5.68 ± 3.76 years. Conclusions According to the factors analyzed, the duration of the surgical procedure, prior cyanotic crisis, and blood lactate level may be relevant to surgical mortality.

Background: In multivessel disease patients with moderate stenosis, fractional flow reserve (FFR) allows the analysis of the lesions and guides treatment, and could contribute to the cost-effectiveness (CE) of non-pharmacological stents (NPS). Objectives: To evaluate CE and clinical impact of FFR-guided versus angiography-guided angioplasty (ANGIO) in multivessel patients using NPS. Methods: Multivessel disease patients were prospectively randomized to FFR or ANGIO groups during a 5 year-period and followed for < 12 months. Outcomes measures were major adverse cardiac events (MACE), restenosis and CE. Results: We studied 69 patients, 47 (68.1%) men, aged 62.0 ± 9.0 years, 34 (49.2%) in FFR group and 53 (50.7%) in ANGIO group, with stable angina or acute coronary syndrome. In FFR, there were 26 patients with biarterial disease (76.5%) and 8 (23.5%) with triarterial disease, and in ANGIO, 24 (68.6%) with biarterial and 11 (31.4%) with triarterial disease. Twelve MACEs were observed - 3 deaths: 2 (5.8%) in FFR and 1 (2.8%) in ANGIO, 9 (13.0%) angina: 4(11.7%) in FFR and 5(14.2%) in ANGIO, 6 restenosis: 2(5.8%) in FFR and 4 (11.4%) in ANGIO. Angiography detected 87(53.0%) lesions in FFR, 39(23.7%) with PCI and 48(29.3%) with medical treatment; and 77 (47.0%) lesions in ANGIO, all treated with angioplasty. Thirty-nine (33.3%) stents were registered in FFR (0.45 ± 0.50 stents/lesion) and 78 (1.05 ± 0.22 stents/lesion) in ANGIO (p = 0.0001), 51.4% greater in ANGIO than FFR. CE analysis revealed a cost of BRL 5,045.97 BRL 5,430.60 in ANGIO and FFR, respectively. The difference of effectiveness was of 1.82%. Conclusion: FFR reduced the number of lesions treated and stents, and the need for target-lesion revascularization, with a CE comparable with that of angiography. Resumo Fundamentos: Em pacientes multiarteriais e lesões moderadas, a reserva de fluxo fracionada (FFR) avalia cada lesão e direciona o tratamento, podendo ser útil no custo-efetividade (CE) de implante de stents não farmacológicos (SNF). Objetivos: Avaliar CE e impacto clínico da angioplastia + FFR versus angioplastia + angiografia (ANGIO), em multiarteriais, utilizando SNF. Métodos: pacientes com doença multiarteriais foram randomizados prospectivamente durante ±5 anos para FFR ou ANGIO, e acompanhados por até 12 meses. Foram avaliados eventos cardíacos maiores (ECAM), reestenose e CE. Resultados: foram incluídos 69 pacientes, 47(68,1%) homens, 34(49,2%) no FFR e 35(50,7%) no ANGIO, idade 62,0 ± 9,0 anos, com angina estável e Síndrome Coronariana Aguda estabilizada. No FFR, havia 26 com doença (76,5%) biarterial e 8 (23,5%) triarterial, e no grupo ANGIO, 24(68,6%) biarteriais e 11(31,4%) triarteriais. Ocorreram 12(17,3%) ECAM – 3(4,3%) óbitos: 2(5,8%) no FFR e 1(2,8%) no ANGIO, 9(13,0%) anginas, 4(11,7%) no FFR e 5(14,2%) no ANGIO, 6 reestenoses: 2(5,8%) no FFR e 4 (11,4%) no ANGIO. Angiografia detectou 87(53,0%) lesões no FFR, 39(23,7%) com ICP e 48(29,3%) com tratamento clínico; e 77(47,0%) lesões no ANGIO, todas submetidas à angioplastia. Quanto aos stents, registrou-se 39(33,3%) (0,45 ± 0,50 stents/lesão) no FFR e 78(66,6%) (1,05 ± 0,22 stents/lesão) no ANGIO (p = 0,0001); ANGIO utilizou 51,4% a mais que o FFR. Análise de CE revelou um custo de R$5045,97 e R$5.430,60 nos grupos ANGIO e FFR, respectivamente. A diferença de efetividade foi 1,82%. Conclusões: FFR diminuiu o número de lesões tratadas e de stents e necessidade de revascularização do vaso-alvo, com CE comparável ao da angiografia.

Background: Coronary artery disease is the leading cause of death worldwide, with age being an independent risk factor for mortality in patients submitted to surgical revascularization. Objective: To evaluate the mortality risk predictors in patients older than 70 years submitted to myocardial revascularization. Methods: This is a retrospective cohort study of a cardiac surgery database. Logistic regression was used to assess independent death predictors. Results: A total of 372 patients submitted to surgical revascularization from 2004 to 2012 were assessed. The main cardiovascular risk factor was hypertension, followed by diabetes mellitus. Mortality at 30 days was 19.35%. The presence of peripheral vascular disease (OR: 2,47), emergency surgery (OR: 4,86) and combined valve procedure (OR: 3,86) were independent predictors of death. Conclusion: The surgical procedure in elderly patients showed a higher mortality than in the general population. Peripheral vascular disease, emergency surgery and combined valve procedures increased the risk of death in these patients.

Background Heart valve surgery outcomes are unknown in middle-income countries and thus cannot be used in health system decision making processes. This study estimated in-hospital mortality and medium and long-term survival. Methods This was a retrospective study of 78,806 patients who underwent heart valve surgery between 2001 and 2007 in Brazil. Two national databases were used, the Hospital Information System and the Mortality Information System. Kaplan-Meier survival analysis and log-rank tests were performed. Maximum and median follow-up was 7.7 and 2.8 years, respectively (0.002–7.707). Results Valve replacement accounted for 69.1% of procedures performed. Mitral stenosis, the most common valve injury, represented 38.9% of the total. In 94.7% of mitral stenosis patients, aetiology was rheumatic heart disease. In-hospital mortality was 7.6% and was higher for women, for patients who had undergone concomitant coronary artery bypass grafting (CABG) and for the elderly. Overall survival was 69.9% at the end of follow-up. Survival was worst among elderly, male and concomitant CABG patients (P

Cristiane da Cruz Lamas. Fundacao Oswaldo Cruz. Instituto Nacional de Infectologia Evandro Chagas. Documento produzido em parceria ou por autor vinculado a Fiocruz, mas nao consta a informacao no documento.

BACKGROUNDStudies on the cost-effectiveness ratio of drug-eluting stents (DES) are rare. Our objective was to evaluate the results and compare costs (incremental cost-effectiveness ratio – ICER) per restenosis avoided between DES and bare metal stents (BMS) using the propensity score.METHODSTwo hundred and twenty consecutive patients were included in the study, of which 111 were treated with DES and 109 with BMS. The propensity score was used to adjust the effect of the intervention, by means of matching, stratification and weighing.RESULTSMost patients were male (67.7% vs. 66.9%; P = 0.53), with a mean age of 65.9 years. Patients treated with the DES had a higher rate of diabetes (54% vs. 17.4%; P < 0.001), three-vessel disease (18.9% vs. 10.1%; P = 0.029) and poor ventricular function (54.1% vs. 22%; P < 0.0001). The diameter of stents was 2.76 ± 0.35mm vs. 2.91 ± 0.47mm (P = 0.006) and the sum of the lengths of stents was 37.6 ± 23mm vs. 24.8 ± 15.8mm (P < 0.0001). Restenosis was observed on 6.3% vs. 12.8% of the patients (P = 0.099) and in 4.1% vs. 9.8% of the lesions (P = 0.048). There was an incremental cost of R$ 9,500.00 and the ICER was R$ 147,538.00 per restenosis avoided (above the World Health Organization threshold). However, when the propensity score was used, the variables that best classified patients for DES and had a maximum ICER of R$ 4,776.96 were age > 72 years, diabetes and lesions with diameter < 3.2mm and length > 18mm.CONCLUSIONSAlthough DES were not cost-effective in the overall population, the propensity score showed that in elderly patients, diabetics and patients with long lesions or small vessels, the use of DES was cost-effective.

Background Myocardial performance index (MPI) was reported as a parameter of ventricular systolic and diastolic function, as well as a useful tool to predict the outcome in patients with ventricular dysfunction. Hypothesis To compare MPI with classical echocardiographic parameters as an independent marker of death in children with idiopathic dilated cardiomyopathy (IDCM). Methods Fifty-five children (13 deaths) underwent 104 echocardiograms from January 1996 to May 2005. Right ventricle (RV) MPI and left ventricle (LV) MPI, and 9 classical echocardiographic parameters (left atrium [LA]/body surface area [BSA], distance between mitral E point and ventricular septum, LV mass/body surface area, RV shortening fraction, LV end-systolic and end-diastolic dimensions/body surface area, LV ejection fraction, fiber circumferential shortening velocity, and mitral deceleration time) were compared. Statistical analysis was performed by chi-square, Pearson's correlation and Student t-test, Kaplan-Meier method, Cox's method, and receiver operating curve (ROC). Statistical significance was considered with α< 0.05 and p = 0.80]. Results Univariate analysis showed that all studied parameters were markers of death. There was a high correlation between RVMPI and LVMPI (r = 0.847]–p = 0.0001]); therefore, to avoid bias, RVMPI was discharged from multivariate analysis. In the deceased group, moderate/severe mitral regurgitation was frequent (76.9%; confidence interval [CI[ 95% = 46.2%– 94.9%) and it was considered in multivariate analysis. In Cox's multivariate analysis, LVMPI was the only independent marker of death (p = 0.0213]). The ideal cut-off was 0.63 with 92.3% sensitivity, 66.7% specificity, and fitted ROC area = 0.918]. Conclusions In children with IDCM, LVMPI is an independent marker of death. Copyright © 2008 Wiley Periodicals, Inc.

Background:Idiopathic dilated cardiomyopathy in children has a high rate of mortality. Cardiac transplantation is the treatment of choice in those who fail to respond to therapeutics. Several studies have been carried out to determine unfavourable prognoses, and to provide an early indication for cardiac transplantation. Nevertheless, no consensus has been reached on the matter.Objective:To propose predictors of death in children with idiopathic dilated cardiomyopathy.Methods:We reviewed data extending over 22 years from 142 consecutive children with idiopathic dilated cardiomyopathy, of whom 36 died. The criterions for inclusion were the presence of congestive heart failure or cardiomegaly in a routine chest X-ray, confirmed by enlargement and hypo kinesis of the left ventricle in the echocardiogram. We included asymptomatic children in functional class I. Based on Cox's analysis of clinical and laboratory data, we sought any predictors of death.Results:In univariate analysis, the predictors were functional class IV at presentation (p equal to 0.0001), dyspnoea (p equal to 0.0096), and reduced pedal pulses (p equal to 0.0413). In chest X-ray, they were maximal cardiothoracic ratio (p equal to 0.0001) and pulmonary congestion (p equal to 0.0072). In the electrocardiogram, right atrium overload (p equal to 0.0118), ventricular arrhythmias (p equal to 0.0148) and heart rate (p equal to 0.027). In the echocardiogram, mitral regurgitation of grade 3 to 4 (p equal to 0.002), the left atrial to aortic ratio (p equal to 0.0001), and left ventricle ejection fraction (p equal to 0.0266). In multivariate analysis, the independent predictors were maximum cardiothoracic ratio (p equal to 0.0001), left ventricle ejection fraction (p equal to 0.0013), mitral regurgitation of grade 3 or 4 (p equal to 0.0017), functional class IV at presentation (p equal to 0.0028), and ventricular arrhythmias (p equal to 0.0253).Conclusion:Children, who have these predictors of death should be considered for early heart transplantation when no improvement is observed in clinical treatment.

Objectives: The Carpentier-Edwards pericardial (CEP) prostheses are the type of bioprostheses most used worldwide. Although they were designed to minimize the rate of valve deterioration and reoperation, their clinical superiority over other prostheses models still lacks confirmation. The objective of this study was to evaluate its effectiveness.Methods: We performed a systematic review and meta-analysis in the PubMed, Embase, Cochrane, and Lilacs databases. Operative mortality, overall mortality and reoperation rates after heart valve surgery were compared between the use of CEP and other cardiac prostheses. Two independent reviewers screened studies for inclusion and extracted the data. Disagreements were resolved by consensus. The GRADE criterion was used to assess the evidence quality.Results: A total of twenty-eight studies were selected, including 19,615 individuals. The studies presented a high heterogeneity and low quality of evidence what limited the reliability of the results. The pooled data from the selected studies did not demonstrate significant differences between CEP and porcine, pericardial or stentless prostheses regarding operative mortality, overall mortality and reoperation rates. However, the pooled data from 3 observational trials pointed out a higher risk for reoperation after valve replacement using CEP prostheses against mechanical prostheses (OR 4.92 [95 percent confidence interval 2.43–9.96]).Conclusions: The current data present in the literature still does not support a clinical advantage for the use of CEP prostheses over other bioprostheses. The quality of the studies in the literature is limited and further studies are needed to address if CEP prostheses will have a clinical advantage over other prostheses.

Background The efficacy of implantable cardioverter-defibrillator (ICD) and cardiac resynchronization therapy-defibrillator (CRT-D) therapy has already been established in clinical trials but their effectiveness in several clinical settings remains undetermined. This study aimed to assess the effectiveness of ICD and CRT-D therapies within the Brazilian National Health System (SUS). Methods All patients who underwent ICD or CRT-D implantation within the SUS from 2001 to 2007 were included in the study. We compared estimated Kaplan-Meier survival curves using the Peto’s test. Prognostic factors were selected using Cox’s models. Results There were included 3,295 patients in the ICD group and 681 patients in the CRT-D group. Cardiac causes accounted for 79% of all deaths in both groups and Chagas’ heart disease accounted for 31% of these deaths. In the CRT-D group, survival significantly decreased around the fourth year of follow-up, with a decrease from 59.5% to 38.3% in 5.5 months. Transvenous implantation technique was used in 62% of CRT-D patients. In-hospital case-fatality rates were higher in those undergoing surgical implantation (5.3%) than those undergoing transvenous implantation (1.6%) (p = 0.02). Conclusions The results show that short-term, medium-term and long-term effectiveness of ICD therapy appears to be similar to that evidenced in clinical trials. In the CRT-D group, in-hospital case-fatality and 30-day case-fatality were higher than those reported in other studies. Surgical epicardial implantation technique was performed in this group at a higher frequency than that reported in the literature and was associated with poorer short-term prognosis.

Background: Heart failure (HF) is the final common event of all cardiac diseases. Technological advances have allowed for significant improvement to survival rates in cardiac patients. Correspondingly, an increase in the HF incidence has been observed. Few data are available on technological advances and their actual impact on the mortality rate of these patients. Objective: To assess the progress of hospital admission rates of adult patients with heart failure, average length of stay and mortality rate in the Brazilian SUS (Unified Health System). Methods: DATASUS data for the 2001-2012 period were obtained. An assessment was performed of data such as all-cause hospital admissions and heart failure-related admissions, average length of stay in hospital, mortality rate and hospital costs, after being stratified by sex, age and place of hospitalization. Results: Over the study period, there have been 91,272,037 hospital admissions, of which 3.96% were due to HF. Male patients accounted for 50.76%. The absolute number of HF-related hospital admissions decreased from 379,463, in 2001, to 240,280, in 2012. The average overall stay in hospital was 5.8 days, in 2001, and 6.6 days, in 2012. The mortality rate was on the rise, from 6.58%, in 2001, to 9.5%, in 2012 (a 46.1% increase). The average cost of AH increased from R$ 519.54, in 2001, to R$ 1,209.56, in 2012 (a 132.8% increase). Conclusion: Despite the decline in hospital admissions, HF is a highly costly syndrome for the Brazilian Unified Health System, with high mortality rates, which paradoxically increased over time, despite technological advances.

Background: Cardiovascular risk stratification is essential for an adequate approach to primary prevention. However, the current risk scores have no satisfactory accuracy in predicting cardiovascular events. Objective: To evaluate whether the two risk scores recommended by the major cardiology societies have predictive value in identifying patients with an established high cardiovascular risk. Methods: The study included 72 patients classified as high risk based on independent factors as recommended by the Brazilian Society of Cardiology. At the first appointment, the Framingham score and the SCORE risk were calculated in order to evaluate the ability to identify high-risk individuals. Results: Of 72 patients, 39 (53.4%) patients were male with a mean age of 59.0±9.5 years, mean 129.0±22 mmHg and 78.0±14 mmHg systolic and diastolic blood pressure, respectively. In the sample, 91.8% were hypertensive, 46.5% had diabetes, 36.9% were current smokers and 72.6% were patients with coronary artery disease, of which 56.1% had previous myocardial infarction. Among these patients, 32 (44.4%) were considered at low cardiovascular risk, 27 (37.5%) intermediate-risk and 13 (18.1%) high-risk by the Framingham score; and 26 (36.1%) patients were considered at high risk by the SCORE risk. Conclusion: Based on the high-risk population, neither the Framingham score nor the SCORE risk were considered good identifiers of patients at high cardiovascular risk.

OBJETIVO: A desnutrição é marcadora independente de óbito na cardiomiopatia dilatada idiopática. Foi analisada a repercussão da introdução da L-carnitina nos parâmetros nutricionais e ecocardiográficos em crianças com cardiomiopatia dilatada idiopática. MÉTODOS: Estudo prospectivo aberto de 11 crianças, comparadas com 40 controles, pareados para sexo e idade. Foi administrada L-carnitina oral (100 mg/kg/dia), além do tratamento padrão. Foram realizadas 118 pesagens no grupo L-carnitina e 264 nos controles, além de 65 ecocardiogramas no grupo L-carnitina e 144 nos controles. Análise estatística: qui-quadrado, teste t de Student, ANOVA e correlação de Pearson. Foi utilizado alfa = 0,05. RESULTADOS: Grupo L-carnitina: idade = 3,82 anos, 72,7% (p = 0,033) menores de 2 anos e do sexo feminino, e 90,9% (p = 0,001) em classe funcional III e IV. Não ocorreram óbitos no período. Não houve diferença no percentil de peso inicial (31,2±8,74 vs. 19,6±21,2) (p = 0,29) nem no índice z (-0,68±1,05 vs. -1,16±0,89) (p = 0,24). Ocorreu aumento do percentil (p = 0,026) e do índice z (p = 0,033) após a L-carnitina. Não houve diferença na fração de ejeção na apresentação (54,9%±3,8 vs. 49,3%±6,6) (p = 0,19), porém a massa VE/SC foi superior no grupo L-carnitina (169,12 g/m²±26,24 vs. 110,67 g/m²±15,62) (p = 0,0005). Após a L-carnitina, a ANOVA demonstrou aumento da fração de ejeção (48,3±7 para 67,2±7) (p = 0,044), e a massa do VE/SC foi reduzida (164,29g/m²±28,14 para 110,88g/m²±28,88), porém sem significância estatística (p = 0,089). CONCLUSÃO: Na cardiomiopatia dilatada idiopática na infância, a suplementação com L-carnitina pode auxiliar na recuperação nutricional e na melhora da fração de ejeção, facilitando a reversão do quadro de caquexia e da insuficiência cardíaca. OBJECTIVES: Malnutrition is an independent predictor of death in idiopathic dilated cardiomyopathy. An analysis was performed of the impact of L-carnitine supplementation on the nutritional status and echocardiogram parameters of children with idiopathic dilated cardiomyopathy. METHODS: This was an open label cohort of 11 patients who received L-carnitine (100 mg/kg/day) plus the conventional medical treatment, compared with 40 controls, matched for gender and age. The L-carnitine group was weighed 118 times and the controls 264 times. Additionally, the L-carnitine group underwent 65 two-dimensional echocardiograms and the controls 144. Chi-square, Student's t test, Pearson correlation and ANOVA were calculated with alpha = 0.05. RESULTS: For the L-carnitine group: age at presentation = 3.82 years old, 72.7% (p = 0.033) were females younger than 2 years and 90.9% (p = 0.0001) were in functional classes III and IV. There were no deaths during this period. At presentation, no differences were observed in weight percentile (31.2±8.74 vs. 19.6±21.2) (p = 0.29) or z score (-0.68±1.05 vs. -1.16±0.89) (p = 0.24). Increases were observed in both the percentile (p = 0.026) and z score (p = 0.033) after the introduction of L-carnitine. At presentation, there were no differences in ejection fraction (54.9%±3.8 vs. 49.3%±6.6) (p = 0.19), but LV mass/BSA were greater in the L-carnitine group (169.12 g/m²±26.24 vs. 110.67 g/m²±15.62) (p = 0.0005). After the introduction of L-carnitine an increase in ejection fraction (48.3±7 to 67.2±7) (p = 0.044) was observed. LV mass/BSA decreased (164.29g/m²±28.14 to 110.88g/m²±28.88), but without significance (p = 0.089) CONCLUSION: In children with idiopathic dilated cardiomyopathy, supplementation of L-carnitine may be helpful for nutritional and echocardiographic improvement.

OBJECTIVES: Malnutrition is an independent predictor of death in idiopathic dilated cardiomyopathy. An analysis was performed of the impact of L-carnitine supplementation on the nutritional status and echocardiogram parameters of children with idiopathic dilated cardiomyopathy. METHODS: This was an open label cohort of 11 patients who received L-carnitine (100 mg/kg/day) plus the conventional medical treatment, compared with 40 controls, matched for gender and age. The L-carnitine group was weighed 118 times and the controls 264 times. Additionally, the L-carnitine group underwent 65 two-dimensional echocardiograms and the controls 144. Chi-square, Student's t test, Pearson correlation and ANOVA were calculated with alpha = 0.05. RESULTS: For the L-carnitine group: age at presentation = 3.82 years old, 72.7% (p = 0.033) were females younger than 2 years and 90.9% (p = 0.0001) were in functional classes III and IV. There were no deaths during this period. At presentation, no differences were observed in weight percentile (31.2±8.74 vs. 19.6±21.2) (p = 0.29) or z score (-0.68±1.05 vs. -1.16±0.89) (p = 0.24). Increases were observed in both the percentile (p = 0.026) and z score (p = 0.033) after the introduction of L-carnitine. At presentation, there were no differences in ejection fraction (54.9%±3.8 vs. 49.3%±6.6) (p = 0.19), but LV mass/BSA were greater in the L-carnitine group (169.12 g/m2±26.24 vs. 110.67 g/m2±15.62) (p = 0.0005). After the introduction of L-carnitine an increase in ejection fraction (48.3±7 to 67.2±7) (p = 0.044) was observed. LV mass/BSA decreased (164.29g/m2±28.14 to 110.88g/m2±28.88), but without significance (p = 0.089) CONCLUSION: In children with idiopathic dilated cardiomyopathy, supplementation of L-carnitine may be helpful for nutritional and echocardiographic improvement.

OBJETIVO: Determinar a importância da desnutrição como marcadora e preditora do óbito na cardiomiopatia dilatada idiopática na infância. MÉTODOS: Este é um estudo retrospectivo envolvendo 165 pacientes (setembro de 1979 a março de 2003). As variáveis analisadas foram sexo, idade e história de infecção viral nos últimos 3 meses, classe funcional da New York Heart Association (NYHA), percentil e desvio padrão do peso (índice z) e avaliação do estado nutricional. Foram realizadas 744 pesagens nos primeiros 72 meses e 93 no primeiro mês de evolução. Análise estatística: qui-quadrado, teste t de Student e análise de variância. Foram utilizados o valor alfa de 0,05 e o valor beta de 0,80. RESULTADOS: A idade no diagnóstico foi de 2,1±3,2 anos, com maior incidência nos menores de 2 anos (75,8%; IC95 = 68,5-82,1%) (p < 0,0001). A classe funcional III e IV foi observada em 81,2% (IC95 = 74,4-86,9%) (p < 0,0001), tendo todos os 40 óbitos ocorrido neste grupo (p = 0,0008). Na apresentação, a miocardite ocorreu em 39,4% (IC95 = 31,9-47,3%) (p = 0,0001). Houve forte associação entre miocardite e doença viral prévia (p = 0,0005) (RC = 3,15; IC95 = 1,55-6,44). A desnutrição na apresentação não influenciou o óbito (p = 0,10), porém a desnutrição evolutiva foi marcadora de óbito (p = 0,02) (RC = 3,21; IC95 = 1,04-9,95). Não houve diferença significativa no percentil de peso (p = 0,15) ou no índice z (p = 0,14) na apresentação. A média do percentil de peso e do índice z foram superiores nos sobreviventes (34,9+32,6 versus 8,6+16,0 e -0,62+1,43 versus -2,02+1,12) (p < 0,0001). A análise de variância demonstrou diferença significativa na evolução para o percentil de peso (p = 0,0417) e para o índice z (p = 0,0005) desde o primeiro mês de evolução. CONCLUSÃO: A avaliação do estado nutricional é de fácil execução, não implica ônus adicional e deve tornar-se rotina no seguimento do paciente com insuficiência cardíaca crônica.

OBJETIVO: Determinar a importância da desnutrição como marcadora e preditora do óbito na cardiomiopatia dilatada idiopática na infância. MÉTODOS: Este é um estudo retrospectivo envolvendo 165 pacientes (setembro de 1979 a março de 2003). As variáveis analisadas foram sexo, idade e história de infecção viral nos últimos 3 meses, classe funcional da New York Heart Association (NYHA), percentil e desvio padrão do peso (índice z) e avaliação do estado nutricional. Foram realizadas 744 pesagens nos primeiros 72 meses e 93 no primeiro mês de evolução. Análise estatística: qui-quadrado, teste t de Student e análise de variância. Foram utilizados o valor alfa de 0,05 e o valor beta de 0,80. RESULTADOS: A idade no diagnóstico foi de 2,1±3,2 anos, com maior incidência nos menores de 2 anos (75,8%; IC95 = 68,5-82,1%) (p < 0,0001). A classe funcional III e IV foi observada em 81,2% (IC95 = 74,4-86,9%) (p < 0,0001), tendo todos os 40 óbitos ocorrido neste grupo (p = 0,0008). Na apresentação, a miocardite ocorreu em 39,4% (IC95 = 31,9-47,3%) (p = 0,0001). Houve forte associação entre miocardite e doença viral prévia (p = 0,0005) (RC = 3,15; IC95 = 1,55-6,44). A desnutrição na apresentação não influenciou o óbito (p = 0,10), porém a desnutrição evolutiva foi marcadora de óbito (p = 0,02) (RC = 3,21; IC95 = 1,04-9,95). Não houve diferença significativa no percentil de peso (p = 0,15) ou no índice z (p = 0,14) na apresentação. A média do percentil de peso e do índice z foram superiores nos sobreviventes (34,9+32,6 versus 8,6+16,0 e -0,62+1,43 versus -2,02+1,12) (p < 0,0001). A análise de variância demonstrou diferença significativa na evolução para o percentil de peso (p = 0,0417) e para o índice z (p = 0,0005) desde o primeiro mês de evolução. CONCLUSÃO: A avaliação do estado nutricional é de fácil execução, não implica ônus adicional e deve tornar-se rotina no seguimento do paciente com insuficiência cardíaca crônica. OBJECTIVE: To analyze the prognostic value of malnutrition in children with idiopathic dilated cardiomyopathy. METHODS: This is a retrospective study of 165 patients with idiopathic dilated cardiomyopathy, diagnosed from September 1979 to March 2003. It analyzed the following variables: gender, age, previous viral illness in the preceding 3 months, functional class according to the New York Heart Association (NYHA), evaluation of nutritional status (normal vs malnutrition), percentile and standard deviation (z index) of weight. Weight was measured 744 times during the first 72 months, 93 during the first month. Statistical analysis was performed by chi-squared, Student t test and analysis of variance for repeated measures (ANOVA). Ninety-five percent confidence intervals (CI95) and odds ratios (OR) were calculated. An alpha value of 0.05 and beta of 0.80 were used. RESULTS: Mean age at presentation was 2.2±3.2 years with higher incidence in those younger than 2 years (75.8%-CI95 = 68.5% to 82.1%) (p < 0.0001). NYHA classes III and IV were observed in 81.2% (CI95 = 74.4% to 86.9%) (p < 0.0001) and all 40 deaths were this group (p = 0.0008). At presentation, myocarditis occurred in 39.4% (CI95 = 31.9% to 47.3%) (p = 0.0001) and a high level of association between myocarditis and previous viral illness was observed (p = 0.0005) (OR = 3.15-CI95 = 1.55 to 6.44). Malnutrition at presentation did not influence death (p= 0.10), however progressive malnutrition was a marker for death (p = 0.02) (OR = 3.21-CI95 = 1.04 to 9.95). No significant differences weight percentiles (p = 0.15) or in z scores (p = 0.14) were observed. Observed mean weight percentiles (34.9+32.6 vs 8.6+16.0) (p < 0.0001) and z scores (-0.62+1.43 vs -2.02+1.12) (p < 0.0001) during the study period were greater among survivors. ANOVA demonstrated significant differences in weight percentile progression (p = 0.0417) and z scores (p = 0.0005) from the first month onwards. CONCLUSION: The evaluation of nutritional status. is easy to performer, it does not imply additional costs and should become routine for children with chronic heart failure.

OBJECTIVE: To analyze our experience with percutaneous aortic balloon valvuloplasty in newborn infants with aortic stenosis, emphasizing the extraordinary importance of myocardial perfusion.METHODS: Over a 10-year-period, 21 neonates underwent percutaneous aortic balloon valvuloplasty. Age ranged from 2 to 27 days, weight ranged from 2.2 to 4.1 kg and 19 were males. All patients presented with congestive heart failure that could not be treated clinically. The onset of symptoms in the first week of life occurred in 9 patients considered as having critical aortic stenosis. Severe aortic stenosis occurred in 12 patients with the onset of symptoms in the second week of life.RESULTS: Mortality reached 100% in the patients with critical aortic stenosis. The procedure was considered effective in the 12 patients with severe aortic stenosis. Vascular complications included the loss of pulse in 12 patients and rupture of the femoral artery in 2 patients. Cardiac complications included acute aortic regurgitation in 2 patients and myocardial perforation in one. In an 8.2±1.3-year follow-up, 5 of the 12 patients died (2 patients due to septicemia and 3 patients due to congestive heart failure). Five of the other 7 patients underwent a new procedure and 2 required surgery.CONCLUSION: Percutaneous aortic valvuloplasty in neonates is not an effective procedure in the 1st week of life, because at this age the common presentation is cardiogenic shock. It is possible that, in those patients with critical aortic stenosis, dilation of the aortic valve during fetal life may change the prognosis of its clinical outcome.

Oral care is frequently suboptimal in children from develop-ing countries, especially those suffering from severe systemic diseases. The aim of the present study was to analyze the oral epidemiological profile of 3-to-5-year-old children with congenital heart disease. Dental and medical records of children evaluated at the Dental Service of the National Institute of Cardiology, Rio de Janeiro, Brazil, were reviewed. Caries experience was reported using the dmft index. Negative behav-ior towards dental management was recorded. The sample consisted of 144 children aged 4.41 ± 0.95 years. The mean dmft value was 5.4 ± 4.9, and 80.5% had at least one caries lesion. Dmft index was greater in the presence of cyanotic cardiac disease and in children with negative behav-ior. An increase in the “missing” component of the dmft index was also found in children using medicine on a daily basis. A higher caries experi-ence was associated with children whose fathers had only an elementary education. In conclusion, children with congenital heart disease had high levels of caries experience at a young age. Cyanosis, negative behavior, daily use of medicine, one-parent family and the educational level of fa-thers seem to influence caries experience in children with congenital car-diac disease.Descriptors: Child; Heart Defects, Congenital; Dental Caries; Dentistry.

INTRODUÇÃO: Os estudos sobre a razão de custo-efetividade dos stents farmacológicos (SFs) são escassos. Nosso objetivo foi avaliar os resultados e comparar os custos (razão custo-efetividade incremental - RCEI) por reestenose evitada entre SFs e stents não-farmacológicos (SNFs) utilizando o escore de propensão. MÉTODOS: Incluímos na análise 220 pacientes tratados consecutivamente, dos quais 111 com SFs e 109 com SNFs. O escore de propensão foi usado para ajustar o efeito da intervenção por meio de pareamento, estratificação e ponderação. RESULTADOS: Predominaram pacientes do sexo masculino (67,7% vs. 66,9%; P = 0,53), com média de idade de 65,9 anos. Pacientes tratados com SFs apresentaram maior frequência de diabetes (54% vs. 17,4%; P < 0,001) e doença triarterial (18,9% vs. 10,1%; P = 0,029) e pior função ventricular (54,1% vs. 22%; P < 0,0001). O diâmetro dos stents foi de 2,76 ± 0,35 mm vs. 2,91 ± 0,47 mm (P = 0,006) e a soma do comprimento dos stents foi de 37,6 ± 23 mm vs. 24,8 ± 15,8 mm (P < 0,0001). Reestenose ocorreu em 6,3% vs. 12,8% dos pacientes (P = 0,099) e em 4,1% vs. 9,8% das lesões (P = 0,048). Houve incremento de custo de R$ 9.590,00 e a RCEI foi de R$ 147.538,00 por reestenose evitada (acima do limiar da Organização Mundial da Saúde). Entretanto, utilizando o escore de propensão, as variáveis que melhor classificaram os pacientes para SFs e apresentam RCEI máxima de R$ 4.776,96 foram idade > 72 anos, diabetes e lesões com diâmetro < 3,2 mm e comprimento > 18 mm. CONCLUSÕES: Apesar de os SFs não terem sido custo-efetivos na população em geral, o escore de propensão demonstrou que em idosos, diabéticos e pacientes com lesões longas ou vasos de fino calibre o uso de SFs foi custo-efetivo. BACKGROUND: Studies on the cost-effectiveness ratio of drug-eluting stents (DES) are rare. Our objective was to evaluate the results and compare costs (incremental cost-effectiveness ratio - ICER) per restenosis avoided between DES and bare metal stents (BMS) using the propensity score. METHODS: Two hundred and twenty consecutive patients were included in the study, of which 111 were treated with DES and 109 with BMS. The propensity score was used to adjust the effect of the intervention, by means of matching, stratification and weighing. RESULTS: Most patients were male (67.7% vs. 66.9%; P = 0.53), with a mean age of 65.9 years. Patients treated with the DES had a higher rate of diabetes (54% vs. 17.4%; P < 0.001), three-vessel disease (18.9% vs. 10.1%; P = 0.029) and poor ventricular function (54.1% vs. 22%; P < 0.0001). The diameter of stents was 2.76 ± 0.35 mm vs. 2.91 ± 0.47 mm (P = 0.006) and the sum of the lengths of stents was 37.6 ± 23 mm vs. 24.8 ± 15.8 mm (P < 0.0001). Restenosis was observed on 6.3% vs. 12.8% of the patients (P = 0.099) and in 4.1% vs. 9.8% of the lesions (P = 0.048). There was an incremental cost of R$ 9,500.00 and the ICER was R$ 147,538.00 per restenosis avoided (above the World Health Organization threshold). However, when the propensity score was used, the variables that best classified patients for DES and had a maximum ICER of R$ 4,776.96 were age > 72 years, diabetes and lesions with diameter < 3.2 mm and length > 18 mm. CONCLUSIONS: Although DES were not cost-effective in the overall population, the propensity score showed that in elderly patients, diabetics and patients with long lesions or small vessels, the use of DES was cost-effective.

OBJETIVO: Avaliar a viabilidade de estratégia de relacionamento probabilístico de bases de dados na identificação de óbitos de pacientes submetidos a procedimentos de alta complexidade em cardiologia. MÉTODOS: O custo de processamento foi estimado com base em 1.672 registros de pacientes submetidos à cirurgia de revascularização do miocárdio, relacionados com todos os registros de óbito no Brasil em 2005. A acurácia do relacionamento baseou-se em linkage probabilístico entre 99 registros de autorização de internação hospitalar de pacientes submetidos a cirurgias cardíacas em instituto de referência em cardiologia, com status vital conhecido, e todos os registros de óbito do estado do Rio de Janeiro em 2005. O linkage foi realizado em quatro etapas: padronização das bases, blocagem, pareamento e classificação dos pares. Utilizou-se a blocagem em cinco passos, com chaves de blocagem com combinação de variáveis como soundex do primeiro e último nome, sexo e ano de nascimento. As variáveis utilizadas no pareamento foram "nome completo", com a utilização da distância de Levenshtein, e "data de nascimento". RESULTADOS: O segundo e o quinto passos de blocagem tiveram os maiores números de pares formados e os maiores tempos de processamento para o pareamento. O quarto passo demandou menor custo de processamento. No estudo de acurácia, após os cinco passos de blocagem, a sensibilidade do linkage foi de 90,6% e a especificidade foi de 100%. CONCLUSÕES: A estratégia de relacionamento probabilístico utilizada apresenta boa acurácia e poderá ser utilizada em estudos sobre a efetividade dos procedimentos de alta complexidade e alto custo em cardiologia. OBJECTIVE: To evaluate the viability of a probabilistic record linkage strategy to identify patients who underwent complex cardiology procedures among the total deceased population. METHODS: The processing cost was estimated based on 1,672 records of patients undergoing coronary artery bypass grafting that were compared with all death records in Brazil in 2005. The accuracy of the linkage strategy was based on the probabilistic linkage of 99 hospital admissions records of patients, with known vital status, who underwent cardiac surgery at a single cardiology institute, with the death records of the state of Rio de Janeiro, Southeastern Brazil, in 2005. Linkage was conducted in four stages: standardizing the databases, blocking, matching, and rating peers. Blocking in five steps was used, with blocking keys formed by a combination of variables such as soundex codes for the first and last names, sex, and year of birth. The variables used for matching were "full name" with the use of Levenshtein distance and "birth date". RESULTS: The second and fifth blocking steps resulted in the largest number of formed pairs and the largest processing times for the matching. The fourth step required a lower processing cost. In the accuracy study, after five blocking steps, the sensitivity of the linkage was 90.6%, and the specificity was 100%. CONCLUSIONS: The probabilistic strategy used has high accuracy and can be used in studies of the effectiveness of high-complexity, high-cost cardiology procedures. OBJETIVO: Evaluar la viabilidad de estrategia de relación probabilística en la identificación de pacientes sometidos a procedimientos de alta complejidad en cardiología. MÉTODOS: El costo de procesamiento fue calculado con base en 1.672 registros de pacientes sometidos a cirugía de revascularización del miocardio, relacionados con todos los registros de óbito en Brasil en 2005. La precisión de la relación se basó en linkage probabilística de 99 registros de autorización de internación hospitalaria de pacientes sometidos a cirugías cardíacas en instituto de referencia en cardiología, con status vital conocido, con todos los registros de óbito del estado de Rio de Janeiro en 2005. El linkage fue realizado en cuatro etapas: estandarización de las bases, blocaje, pareamiento y clasificación de los pares. Se utilizó blocaje en cinco pasos, con claves de blocaje con combinación de variables como soundex del primero y último nombre, sexo y año de nacimiento. Las variables utilizadas en el pareamiento fueron "nombre completo", con la utilización de la distancia de Levenshtein y "fecha de nacimiento". RESULTADOS: El segundo y el quinto pasos de blocaje tuvieron los mayores números de pares formados y los mayores tiempos de procesamiento para el pareamiento. El cuarto paso demandó menor costo de procesamiento. En el estudio de precisión, posterior a cinco pasos de blocaje, la sensibilidad del linkage fue de 90,6% y la especificidad fue de 100%. CONCLUSIONES: La estrategia de relación probabilística utilizada presenta buena precisión y podrá ser utilizada en estudios sobre la efectividad de los procedimientos de alta complejidad y alto costo en cardiología.

FUNDAMENTO: Na literatura nacional há escassez de análises de custo-efetividade, comparando stents farmacológicos (SF) versus não farmacológicos (SNF), no seguimento tardio. OBJETIVO: Estimar a Razão de Custo-Efetividade Incremental (RCEI) entre SF e SNF na coronariopatia uniarterial. MÉTODOS: 217 pacientes (130 SF e 87 SNF), com 48 meses de seguimento (média=26). Desfecho primário: custo por re-estenose evitada, sendo efetividade definida como redução de eventos maiores. O modelo analítico de decisão foi baseado no estudo de Polanczyk et al. Os custos diretos foram aqueles utilizados diretamente nas intervenções. RESULTADOS: A amostra foi homogênea para idade e sexo. O SF foi mais utilizado em diabéticos: 59 (45,4%) vs 16 (18,4%)(p

To evaluate the viability of a probabilistic record linkage strategy to identify patients who underwent complex cardiology procedures among the total deceased population.The processing cost was estimated based on 1,672 records of patients undergoing coronary artery bypass grafting that were compared with all death records in Brazil in 2005. The accuracy of the linkage strategy was based on the probabilistic linkage of 99 hospital admissions records of patients, with known vital status, who underwent cardiac surgery at a single cardiology institute, with the death records of the state of Rio de Janeiro, Southeastern Brazil, in 2005. Linkage was conducted in four stages: standardizing the databases, blocking, matching, and rating peers. Blocking in five steps was used, with blocking keys formed by a combination of variables such as Soundex codes for the first and last names, sex, and year of birth. The variables used for matching were "full name" with the use of Levenshtein distance and "birth date".The second and fifth blocking steps resulted in the largest number of formed pairs and the largest processing times for the matching. The fourth step required a lower processing cost. In the accuracy study, after five blocking steps, the sensitivity of the linkage was 90.6%, and the specificity was 100%.The probabilistic strategy used has high accuracy and can be used in studies of the effectiveness of high-complexity, high-cost cardiology procedures.

There is a scarcity of cost-effectiveness analyses in the national literature comparing drug-eluting stents (DES) with bare-metal stents (BMS), at late follow-up.To estimate the Incremental Cost-Effectiveness Ratio (ICER) between DES and BMS in uniarterial coronariopathy.217 patients (130 DES and 87 BMS), with 48 months of follow-up (mean = 26 months) were assessed.cost per prevented restenosis, with effectiveness being defined as the decrease in major events. The analytical model of decision was based on the study by Polanczyk et al. The direct costs were those used directly in the interventions.The sample was homogenous for age and sex. The DES was more used in diabetic patients: 59 (45.4%) vs 16 (18.4%)(p0.0001) and with a history of coronary artery disease (CAD): 53 (40.7%) vs 13 (14.9%)(p0.0001). The BMS was more used in simple lesions, but with worse ventricular function. The DES were implanted preferentially in proximal lesions: (p=0.0428) and the BMS in the mid-third (p=0.0001). Event-free survival: DES = 118 (90.8%) vs BMS=74 (85.0%) (p=0.19); Angina: DES=9 (6.9%) vs BMS=9 (10.3%) (NS): Clinical restenosis: DES=3 (2.3%) vs BMS=10 (10.3%) (p=0.0253). Cardiac deaths: 2 (1.5%) in DES and 3 (3.5%) in BMS (NS).the tree of decision was modeled based on restenosis. The net benefit for the DES needed an increment of R$7,238.16. The ICER was R$131,647.84 per prevented restenosis (above the WHO threshold).The DES was used in more complex lesions. The clinical results were similar. The restenosis rate was higher in the BMS group. The DES was a non-cost-effective strategy.

Analysis of critical end diastolic left ventricular volume (EDLVV), defined as the lowest acceptable volume to keep cardiac output, in the selection of patients with post-valvotomy aortic stenosis, candidates to univentricular correction.A retrospective study in 21 patients with aortic stenosis, during the first year of life, and 232 patients compiled from literature. Values of end diastolic volume (EDLVV), from 20 to 60 ml/m2 were determined as normal. The EDLVV of deceased patients was compared to that from survival patients. A correlation between the age and EDLVV was carried out at the time of valvotomy, between the groups. Finally, the critical EDLVV through the theoretical relationship between the EDLVV and heart rate for different ejection fractions and designated cardiac indexes (CI): 2,000 and 2,500 20-60 ml/min/m2 was determined.For EDLVV values20 ml/m2 and60 ml/m2, there was statistical significance between deceased and survival patients (p0.0001). However, in the range between 20 and 60 ml/m2, that significance was lower (p = 0.0309). A greater incidence of death took place among those who showed it in the first week of life. For a CI of 2,500 ml/min/m2 and a heart rate (HR) of 140 bpm, critical EDLVV will be 26 ml/m2 if left ventricular ejection fraction (LVEF) is 0.70, and 44.5 ml/m2, if LVFE is 0.40.The analysis of critical end diastolic left ventricular volume (EDLVV) can add another parameter in the indication of univentricular correction in patients with post-valvotomy aortic stenosis, during the first year of life.

OBJETIVO: Análise do volume diastólico final ventricular esquerdo crítico, definido como o menor volume aceitável para a manutenção do débito cardíaco, na seleção de pacientes com estenose aórtica pós-valvotomia, candidatos à correção univentricular. MÉTODOS: Estudo retrospectivo em 21 pacientes com estenose aórtica, durante o primeiro ano de vida, e 232 compilados da literatura. Arbitraram-se como normais os valores do volume diastólico final (VDF) de 20 a 60 ml/m2. Foi comparado o VDF dos grupos que foram ao óbito com aqueles que sobreviveram. Procurou-se, também, estabelecer uma correlação da idade e volume ventricular, na época da valvotomia, entre os grupos. Por fim, determinou-se o VDF esquerdo crítico, obtido através de uma relação teórica entre o VDF esquerdo e freqüência cardíaca para diferentes frações de ejeção e determinados índices cardíacos (IC): 2.000 e 2.500 ml/min/m2. RESULTADOS: Para valores do VDF 60 ml/m2, houve significância estatística entre os mortos e os sobreviventes (p

To identify the types of pulmonary vascular blood supply in tetralogy of Fallot with pulmonary atresia by use of hemodynamic study.Fifty-six patients with tetralogy of Fallot and pulmonary atresia, and ages ranging from 20 days to 4 years, underwent cineangiocardiographic study with contrast medium injections in the following vascular structures: 1) wedged pulmonary vein; 2) aortopulmonary collaterals; 3) thoracic aorta; and 4) ductus arteriosus or systemic-pulmonary shunt.In the 56 patients studied, pulmonary blood was supplied as follows: in 15, by aortopulmonary collaterals; in 36, only by the ductus arteriosus; and in 5, by the ductus arteriosus and aortopulmonary collaterals. The patients were classified into 6 types depending on the type of pulmonary vascular perfusion and the presence or absence of vascular structures that compose the pulmonary circulation in tetralogy of Fallot with pulmonary atresia.This type of approach enables the obtainment of information necessary for the correct clinicosurgical management of patients, due to the great complexity and extreme variability of the pulmonary blood supply in tetralogy of Fallot with pulmonary atresia.

To emphasize the diagnostic possibility of the anomalous origin of one pulmonary artery from the ascending aorta in infants with clinically refractory heart failure and no intracardiac structural defect.Retrospective study of 4 infants with refractory heart failure undergoing 2-dimensional echocardiographic study with subcostal, suprasternal, and parasternal views, and hemodynamic and angiocardiographic study in the anteroposterior projection.Three of the 4 infants had their right pulmonary artery originating from the ascending aorta as their major diagnosis. In the fourth patient, the left pulmonary artery originated from the ascending aorta in association with a large interventricular septal defect. The pressure level in both pulmonary arteries in all infants was that of the systemic level. All patients underwent surgery, which consisted of translocation of the anomalous pulmonary artery from the aorta. Neither immediate nor late cardiac deaths occurred.Once the diagnosis of anomalous origin of the pulmonary artery from the ascending aorta in the isolated form is established, the surgical correction should be immediately performed, not only because of the risk of developing pulmonary vascular disease, but also because of the excellent surgical results currently obtained.

OBJETIVO: Enfatizar a possibilidade diagnóstica da origem anômala de uma artéria pulmonar da aorta ascendente, em lactentes com quadro de insuficiência cardíaca clinicamente intratável e sem defeito estrutural intracardíaco. MÉTODOS: Estudo retrospectivo em quatro lactentes com insuficiência cardíaca intratável, submetidos a estudo ecocardiográfico bidimensional com cortes subcostal, supraesternal e paraesternal, e a estudo hemodinâmico e angiocardiográfico na projeção ântero-posterior. RESULTADOS: Três dos quatro lactentes tinham a artéria pulmonar direita originando-se da aorta ascendente, como diagnóstico principal. No quarto paciente a artéria pulmonar esquerda originava-se da aorta ascendente associada à ampla comunicação interventricular. Os lactentes tinham pressão em ambas artérias pulmonares em nível sistêmico. Todos foram submetidos a tratamento cirúrgico que consistiu na translocação da artéria pulmonar anômala da aorta. Não houve óbitos cardíacos imediatos ou tardios. CONCLUSÃO: Uma vez estabelecido o diagnóstico de origem anômala de artéria pulmonar da aorta ascendente, na forma isolada, a correção cirúrgica deverá ser prontamente realizada, não só pelo desenvolvimento de doença vascular pulmonar, como pelos excelentes resultados cirúrgicos atualmente obtidos.

To determine the morphological and functional changes in the right ventricle in pulmonary atresia with intact ventricular septum (PAIVS) for assessing the candidates for the different therapeutic procedures currently available.Thirty-one patients underwent cineangiocardiographic study with axial projections. Their ages ranged from 1 to 50 days (x = 9.6), and 28 of them were studied during the first month of life. The statistical analysis comprised the following: chi-square test, Kruskal-Wallis test for the mean and standard deviation, multiple regression, and the 95% confidence interval (95%CI) was calculated. The significance level adopted was alphaor =0.05.The patients were divided into 3 groups according to the angiographic morphology of the right ventricle (RV): group A -- tripartite RV (n=16); group B -- bipartite RV (n=9); and group C -- unipartite RV (n=6). The diameter of the tricuspid valve was 10.28 +/- 2.67 mm (A); 7.82 +/- 3.41 (B); and 5.27 +/- 0.57 (C) (P=0.0005). Pulmonary atresia was of the valvular type in all group A patients and of the infundibular type in all group C patients (P0.0001). Coronary-cavitary connections were rare (2/16) in group A patients, but occurred in all group C patients (P=0.0006), with retrograde opacity of the aorta (flow from the RV to the aorta) in 2 group A patients and in all group C patients (P=0.0003). Three patients (2 in group C and 1 in group A) had right-ventricular-dependent coronary circulation. Isolated moderate/severe tricuspid regurgitation showed a tendency towards being more frequent in group A (P=0.0525). The angle of the ductus arteriosus with the descending aorta was as follows: 104.06 +/- 8.98 in group A; 79.17 +/- 33.08 in group B; and 39.0 +/- 6.52 in group C (P=0.0016). The correlation between the diameter of the tricuspid valve and the angle of the ductus arteriosus with the descending aorta was 0.6568 (P=0.0002).Because of the heterogeneity of the morphology of the RV in patients with pulmonary atresia with intact ventricular septum, knowledge about all these data is required for selecting candidates for the different therapeutic procedures.

To determine the echocardiographic predicting factors of death in children with idiopathic dilated cardiomyopathy.A retrospective study of 148 children with idiopathic dilated cardiomyopathy diagnosed between September 1979 and March 2003 was carried out. The inclusion criteria were as follows: heart failure and a reduction in contractility on the echocardiogram in the absence of congenital or secondary heart disease. Four hundred and seventy examinations during a period of 244.8 months of evolution were analyzed. The following parameters were assessed: left atrial dimension (LAD); left atrium/aorta ratio (LAD/Ao); left ventricular systolic (LVSD) and diastolic (LVDD) dimensions; left ventricular mass (LVmass); right ventricular dimension (RVD); left ventricular ejection fraction (LVEF); left ventricular shortening fraction (% SH); severity of the insufficiency of the atrioventricular and pulmonary valves; and right ventricular systolic (RVSP) and diastolic (RVDP) pressures. The significance level adopted was alpha0.05.The mean age was 2.37 years, and 35 patients died (23.7%--95 CI = 17.1% to 31.2%). The analysis of variance showed the following: LAD (p0.0001); LAD/Ao (p0.0001); LVSD (p=0.0061); LVDD (p=0.0086); LVmass (p0.0001); LVEF (p=0.0074); %SH (p=0.0072); and RVD (p0.0001). Worsening of mitral (MI) (p=0.0113) and tricuspid (TI) insufficiencies (p=0.0044) were markers of death, and the presence of MI, TI, and moderate/severe pulmonary insufficiency were deleterious to survival. The Cox proportional hazards regression model showed the following independent predictors of death: LAD/Ao (p=0.0487); LVEF (0.0001); and the presence of moderate/severe MI (p=0.0419).Patients with a progressive increase in LAD/Ao, a reduction in LVEF, and progressive worsening of MI, regardless of the clinical treatment, should be considered for early heart transplantation.

OBJETIVO: Determinar os fatores preditores ecocardiográficos de morte em crianças com cardiomiopatia dilatada idiopática. MÉTODOS: Estudo retrospectivo de 148 crianças com cardiomiopatia dilatada idiopática diagnosticadas entre setembro/1979 a março/2003. Critérios para inclusão: insuficiência cardíaca e redução da contratilidade no ecocardiograma, na ausência de cardiopatia congênita ou secundária. Foram analisados 470 exames, durante 244,8 meses de evolução. Parâmetros analisados: dimensão do átrio esquerdo (DAE), relação átrio esquerdo/aorta (DAE/Ao), dimensão sistólica (DSVE) e diastólica (DDVE) do VE, massa do VE (massa), dimensão do ventrículo direito (DVD), fração de ejeção do VE (FE), percentagem de encurtamento do VE (%EC), gravidade da insuficiência das valvas atrioventriculares e da valva pulmonar e medida da pressão sistólica (PsVD) e diastólica (PdVD) do VD. Foi considerado significativo erro alfa

OBJECTIVE To analyze the prognostic value of cardiomegaly, pulmonary congestion and cardiothoracic ratio as indicators of death and survival in children with idiopathic dilated cardiomyopathy. METHODS We carried out a retrospective review of 152 patients with idiopathic dilated cardiomyopathy diagnosed between September 1979 and March 2003. In the first 72 months, 722 exams were performed (100 in the first 15 days). STATISTICAL ANALYSIS chi-square, Student's t test, ANOVA and Kaplan-Meier curves. Alpha = 0.05; beta = 0.80. RESULTS The mean age at presentation was 2.2+/-3.2 years. Idiopathic dilated cardiomyopathy incidence was higher in children younger than 2 years (76.3% - 95% CI = 68.7% to 82.8%) (p < 0.0001). Sex (p = 0.07) and color (p = 0.11) were not significant and mortality was not influenced by age (p = 0.73), sex (p = 0.78) or color (p = 0.20). Most patients were severely ill (84.2% - 95% CI = 77.4% to 89.6%; functional class III and IV; p < 0.0001). All 43 deaths occurred in this group (p = 0.0008). Cardiomegaly at presentation was observed in 94.1% (95% CI = 89.1% to 97.2%) (p < 0.0001), and pulmonary congestion in 75.6% (95% CI = 68.0% to 82.2%) (p < 0.0001). Pulmonary congestion and cardiomegaly were more frequent in functional class III/IV patients (RC = 8.03 - 95% CI = 2.85% to 23.1%) (p < 0.0001). Pulmonary congestion was a marker of death (RC = 3.16 - 95% CI = 1.06% to 10.07) (p = 0.0222), but not cardiomegaly (p = 0.1185). Survival was influenced by both cardiomegaly (p = 0.0189) and pulmonary congestion (p = 0.0050). Mean and maximum cardiothoracic ratio were higher in the death group (0.749+/-0.053 vs. 0.662+/-0.080) (p < 0.0001) and (0.716+/-0.059 vs. 0.620+/-0.085) (p < 0.0001). ANOVA revealed a progressive decrease in cardiothoracic ratio in the survival group (p < 0.0001). CONCLUSIONS In children with idiopathic dilated cardiomyopathy, the presence of pulmonary congestion at presentation and increased cardiothoracic ratio are associated with poor survival.