Marco Aurélio Santos

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.

Percutaneous closure of ductus arteriosus with the amplatzer prosthesis. The Brazilian experience

OBJECTIVE - To report the results of percutaneous occlusion of persistent ductus arteriosus with the Amplatzer prosthesis in 2 Brazilian cardiological centers. METHODS - From May 1998 to July 2000, 33 patients with clinical and laboratory diagnosis of persistent ductus arteriosus underwent attempts at percutaneous implantation of the Amplatzer prosthesis. The median age was 36 months (from 6 months to 38 years), and the median weight was 14kg (from 6 to 92kg). Sixteen patients (48.5%) were under 2 years of age at the time of the procedure. All patients were followed up with periodical clinical and echocardiographic evaluations to assess the presence and degree of residual shunt and possible complications, such as pseudocoarctation of the aorta and left pulmonary artery stenosis. RESULTS - The minimum diameter of the arterial ducts ranged from 2.5 to 7.0mm (mean of 4.0+/-1.0, median of 3.9). The rate of success for implantation of the prosthesis was 100%. Femoral pulse was lost in 1 patient. The echocardiogram revealed total closure prior to hospital discharge in 30 patients, and in the follow-up visit 3 months later in the 3 remaining patients. The mean follow-up duration was 6.4+/-3.4 months. All patients were clinically well, asymptomatic, and did not need medication. No patient had narrowing of the left pulmonary artery or of the aorta. No early or late embolic events occurred, nor did infectious endarteritis. A new hospital admission was not required for any patient. CONCLUSION - The Amplatzer prosthesis for persistent ductus arteriosus is safe and highly effective for occlusion of ductus arteriosus of varied diameters, including large ones in small symptomatic infants.

Is Myocardial Performance Index an Independent Echocardiographic Marker of Death 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 impact of malnutrition on 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.

Prognostic value of chest roentgenograms in children with idiopathic dilated cardiomyopathy

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.

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

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.

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

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.

Neonate Aortic Stenosis: Importance of Myocardial Perfusion in Prognosis

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.

Características morfológicas angiográficas na atresia pulmonar com septo interventricular íntegro

Objective 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. Methods 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 alpha ≤ 0.05. Results 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 (P<0.0001). 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). Conclusion Because of the heterogeneity of the morphology of the RV