Vitor Manuel Pereira Azevedo

Outcome factors of idiopathic dilated cardiomyopathy in children - a long-term follow-up review.

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 criteria 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 Coxs 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.

Como o ecocardiograma pode ser útil em predizer a morte em crianças com cardiomiopatia dilatada idiopática

OBJECTIVE: To determine the echocardiographic predicting factors of death in children with idiopathic dilated cardiomyopathy. METHODS: 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 alpha < 0.05. RESULTS: 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 (p<0.0001); LAD/Ao (p<0.0001); LVSD (p=0.0061); LVDD (p=0.0086); LVmass (p<0.0001); LVEF (p=0.0074); %SH (p=0.0072); and RVD (p<0.0001). 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). CONCLUSION: 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.

Análise de custo-efetividade dos stents farmacológicos e não farmacológicos na doença coronariana

BACKGROUND: 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. OBJECTIVE: To estimate the Incremental Cost-Effectiveness Ratio (ICER) between DES and BMS in uniarterial coronariopathy. METHODS: 217 patients (130 DES and 87 BMS), with 48 months of follow-up (mean = 26 months) were assessed. Primary outcome: 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. RESULTS: The sample was homogenous for age and sex. The DES was more used in diabetic patients: 59 (45.4%) vs 16 (18.4%)(p<0.0001) and with a history of coronary artery disease (CAD): 53 (40.7%) vs 13 (14.9%)(p<0.0001). 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). Costs: the tree of decision was modeled based on restenosis. The net benefit for the DES needed an increment of R

Is Myocardial Performance Index an Independent Echocardiographic Marker of Death in Children with Idiopathic Dilated Cardiomyopathy

7,238.16. The ICER was R

The impact of malnutrition on idiopathic dilated cardiomyopathy in children

131,647.84 per prevented restenosis (above the WHO threshold). CONCLUSIONS: 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.

Prognostic value of chest roentgenograms in children with idiopathic dilated cardiomyopathy

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.

The role of L-carnitine in nutritional status and echocardiographic parameters in idiopathic dilated cardiomyopathy in children

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 perform, it does not imply additional costs and should become routine for children with chronic heart failure.

Origem anômala de uma artéria pulmonar da aorta ascendente: resolução da hipertensão arterial pulmonar com a correção cirúrgica

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, Students 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.

Neonate Aortic Stenosis: Importance of Myocardial Perfusion in Prognosis

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, Students t test, Person 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 (164.29 g/m2+/-28.14 to 110.88 g/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.

Uso do escore de propensão na análise de custo-efetividade com utilização seletiva de stents farmacológicos e não-farmacológicos

OBJECTIVE: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.