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Introduction and Objective: This is the first study in Angola with the aim of characterizing ventricular septal defect (VSD) among children and adolescents., Methods: A cross-sectional study based on echocardiographic records of the largest pediatric cardiology center in Angola included all children and adolescents (0 to 18 years old) with VSD between April 2010 and March 2011. The diagnosis was made by transthoracic and Doppler echocardiography with a Medison SA 8000 system. The sample was divided into two groups: Group 1, isolated VSD; and Group 2, VSD associated with other congenital heart defects (CHDs). Age, gender, type of VSD, associated CHDs and genetic syndromes were assessed., Results: A total of 490 CHDs were diagnosed, of which 283 were VSDs. In Group 1 (140, 49%) the mean age was 29±36 months. The most frequent age (mode) at diagnosis was 24 months. There was no predominance of gender (ratio 1:1). The majority (127, 91%) had perimembranous VSD. In Group 2 (143, 51%) 113 patients (79%) had one, 27 patients (19%) had two and three patients (2%) had three other CHDs. Trisomy 21 was the most common genetic syndrome (23, 96%)., Conclusions: The study shows that VSD is the most common CHD in childhood, the diagnosis is made late and almost half of VSDs are associated with other CHDs., (Copyright © 2014 Sociedade Portuguesa de Cardiologia. Published by Elsevier España. All rights reserved.)

Background: Since Wilcox's description of the simplified single-patch technique for atrioventricular septal defect (AVSD) repair in 1997, several studies have compared that technique with the two-patch technique., Objective: To report the mid- and long-term results of the simplified single-patch technique for complete AVSD repair., Methods: Retrospective study of 16 consecutive cases between January 2001 and December 2011. The patients' mean age was 18.31 ± 34.19 months (2 months - 11 years), and their mean weight, 7.80 ± 6.12 kg (3.77 - 25.0 kg). Six patients were males and 14 had Down syndrome. Mean follow-up duration was 54.97 ± 47.79 months., Results: Mean cardiopulmonary bypass time was 74.63 ± 18.48 min (49 - 112 min), and mean aortic cross-clamp time, 46.44 ± 11.89 min (34 - 67 min). Two patients died during hospitalization (12.5%), both of cardiovascular causes. Three patients underwent reoperation due to left atrioventricular (AV) valve regurgitation, and two had third-degree VA block, requiring permanent pacemaker implantation. No patient had left ventricular outflow tract obstruction. The 14 surviving patients remain asymptomatic, ten of whom with mild left VA valve regurgitation (71.42%)., Conclusion: The simplified single-patch technique for complete AVSD repair proved to be feasible, providing adequate correction of the defects and favorable clinical and echocardiographic outcome in the mean 57.97-month follow-up.

Objectives: To access the frequency of abnormal echocardiographic findings in patients with diagnosis of innocent murmur., Methods: A transversal study of 166 consecutive patients was carried out with diagnosis of innocent murmur evaluated in the pediatric cardiology outpatient clinic of "Instituto de Cardiologia/Fundação Universitária de Cardiologia", a tertiary reference center, between December 3, 2001 and December 2, 2002. History, clinical examination and echocardiogram were performed in all patients. Post-test probabilities of disease with positive and negative clinical examination were estimated, considering the echocardiogram as gold standard., Results: Of the 166 patients evaluated, 11 showed some alteration in the echocardiogram: pulmonary stenosis (4), patent foramen ovale (2), atrial septal defect (1), bicuspid aortic valve (2), minimum aortic regurgitation (1) and ventricular septal defect (1). Negative post-test probability (probability of disease with diagnosis of innocent murmur) was of 6.6%., Conclusion: In a referral outpatient clinic the proportion of minor lesions is high in patients with diagnosis of innocent murmur. These injuries may not be related to the heart murmur, as in the patent foramen ovale and bicuspid aortic valve. However, they raise the question of the indication of echocardiogram taking into account the low risk of detected lesions and, on the other hand, the resolutive character of a daycare reference center.

Objective: To assess the initial experiment with percutaneous closure of interventricular septal defects (IVSD), especially perimembranous IVSD (PMIVSD) with the new Amplatzer prosthesis., Methods: Eleven patients were submitted to the procedure. Six of them had perimembranous IVSD (PMIVSD) and five with muscular IVSD (MIVSD). Two showed repeated respiratory tract infections and had low ponderal gain. One of them showed a previous history of infectious endocarditis. The others were asymptomatic and were selected through transthoracic echocardiography (TTE)., Results: In MIVSD group (n = 5), a patient had apical IVSD, two had medioseptal defects and two patients showed multiple defects. In this group, an interatrial septal defects (IASD) (Amplatzer Septal Occluder) and five prostheses for MIVSD (Amplatzer VSD-MUSC Occluder), and one patient received two devices. All implants were well-succeeded and went by without complications. In PMIVSD group (n = 6), two patients had membranous septal aneurysms. In five, we identified two orifices with selective angiography. We used arterial canal Amplatzer prostheses (ACP) (Amplatzer Duct Occluder) in 1 patient and specific prostheses for PMIVSD in the others (Amplatzer VSD-MEMB Occluder). A perimembranous occluder migrated immediately after released, being removed from the descending aorta. Another patient showed total atrioventricular block (TAVB), which reversed after corticotherapy., Conclusion: The procedure is technically complex and must be performed only in specialized centers. Despite of that, it showed safe and efficient in the selected cases, and it can be provided as an alternative to traditional surgical treatment.

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 < or =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 in patients with pulmonary atresia with intact ventricular septum, knowledge about all these data is required for selecting candidates for the different therapeutic procedures.

Double outlet right ventricle (DORV) is a heterogeneous group of abnormal ventriculoarterial connections where, by definition, both great arteries (pulmonary artery and aorta) arise primarily from the morphologically right ventricle. This condition affects 1-1.5% of the patients with congenital heart diseases, with a frequency of 1 in each 10,000 live births. We report the case of an 18-day-old infant with DORV and extremely rare anatomical features, such as anterior and left-sided aorta and subpulmonary ventricular septal defect (VSD). In addition to the anatomic features, the role of the echocardiogram for guiding the diagnosis and the surgical therapy of this congenital heart disease are discussed.

Interruption of the aortic arch is an uncommon cardiovascular anomaly. It is usually associated with a large ductus arteriosus (DA) and interventricular septal defects. Two cases of one and two month old girls with restrictive DA are reported. The clinical, anatomical and surgical findings are discussed.

Objective: To evaluate the medium term results of surgical closure of ventricular septal defect (VSD) performed during the first year of life, using echocardiography (echo)., Material and Methods: We studied prospectively 29 patients aged from 17 to 68 months (mean = 37) who underwent surgical closure of perimembranous VSD during the first year of life. The postoperative follow-up time ranged from 6 to 60 months (mean = 26). Fifteen age-matched normal children were used as a control group in evaluating the left ventricular (LV) systolic function. The echo study included: 1) the assessment of LV systolic function using the shortening fraction, ejection fraction, pre-ejection to ejection time ratio (PET/ET), aortic flow acceleration time, acceleration to ejection time ratio, mean aortic flow acceleration; 2) detection and quantification of residual VSD as well as tricuspid and or aortic regurgitation; 3) determination of right ventricular systolic pressure (RVSP). The RVSP was evaluated from the maximum flow velocity from a residual VSD or tricuspid regurgitation, using the simplified Bernoulli equation., Results: The LV systolic function parameters from patients versus (vs) normals showed a significantly different shortening fraction (34 +/- 5 vs 39 +/- 4; p = 0.005) and PET/ET ratio (0.34 +/- 0.04 vs 0.31 +/- 0.03; p = 0.02). None of the other studied parameters was significantly different from normal. Five (17%) patients had a small residual VSD. Two (7%) patients had mild aortic regurgitation. Tricuspid regurgitation was detected in 23 (79%) patients being trivial in 20 and mild to moderate in 3. The RVSP was quantified in 22 (76%) patients, ranging from 30 to 45 mmHg (mean +/- SD = 36 +/- 4). None of the remaining 7 patients showed changes in the end-systolic interventricular septal configuration., Conclusions: Our study shows that surgical closure of perimembranous VSD performed during the first year of life was possible without significant mobility. Furthermore, the echo allowed a complete and noninvasive cardiac evaluation of the anatomic, functional and hemodynamic status of this group of patients, thus limiting the need for postoperative control cardiac catheterization.