Supravalvular Congenital Mitral Stenosis

Congenital supravalvular mitral stenosis is a rare malformation characterized by the presence of a shelf-like fibrous membrane, with 1 or 2 small orifices, covering and obstructing the mitral valve. The membrane is positioned closely to the mitral valve (and sometimes it is attached to it); therefore, a preoperative diagnosis is inevitably difficult, even with the use of biplane echocardiography. Two patients with supravalvular mitral stenosis aged 3 years and 3 months are described. In 1 patient, a preoperative diagnosis was made, and both successfully underwent correction.

Trends of the Risk of Death due to Circulatory, Cerebrovascular, and Ischemic Heart Diseases in 11 Brazilian Capitals from 1980 to 1998

OBJECTIVE: To assess the trends of the risk of death due to circulatory (CD), cerebrovascular (CVD), and ischemic heart diseases (IHD) in 11 Brazilian capitals from 1980 to 1998. METHODS: Data on mortality due to CD, CVD and IHD were obtained from the Brazilian Health Ministry, and the population estimates were calculated by interpolation with the Lagrange method based on census data from 1980 and 1991 and the population count of 1996. The trends were analyzed with the multiple linear regression method. RESULTS: CD showed a trend towards a decrease in most capitals, except for Brasília, where a mild increase was observed. The cities of Porto Alegre, Curitiba, Rio de Janeiro, Cuiabá, Goiânia, Belém, and Manaus showed a decrease in the risk of death due to CVD and IHD, while the city of Brasília showed an increase in CVD and IHD. The city of São Paulo showed a mild increase in IHD for individuals of both sexes aged 30 to 39 years and for females aged 40 to 59 years. In the cities of Recife and Salvador, a reduction in CD was observed for all ages and both sexes. In the city of Recife, however, an increase in IHD was observed at younger ages (30 to 49 years), and this trend decreased until a mild reduction (-4%) was observed in males ³ 70 years. CONCLUSION: In general, a reduction in the risk of death due to CD and an increase in IHD were observed, mainly in the cities of Recife and Brasília.

Surgical treatment of partial atrioventricular septal defect: functional analysis of the mitral valve in the postoperative period

OBJECTIVE: To study mitral valve function in the postoperative period after correction of the partial form of atrioventricular septal defect. METHODS: Fifty patients underwent surgical correction of the partial form of atrioventricular septal defect. Their mean age was 11.8 years and 62% of the patients were males. Preoperative echocardiography showed moderate and severe mitral insufficiency in 44% of the patients. The mitral valve cleft was sutured in 45 (90%) patients (group II - GII). Echocardiographies were performed in the early postoperative period, and 6 and 12 months after hospital discharge. RESULTS: The patients who had some type of arrhythmia in the postoperative period had ostium primum atrial septal defect of a larger size (2.74 x 2.08 cm). All 5 patients in group I (GI), who did not undergo closure of the cleft, had a competent mitral valve or mild mitral insufficiency in the preoperative period. One of these patients began to have moderate mitral insufficiency in the postoperative period. On the other hand, in GII, 88.8% and 82.2% of the patients had competent mitral valve or mild mitral insufficiency in the early and late postoperative periods, respectively. CONCLUSION: The mitral valve cleft was repaired in 90% of cases. Echocardiography revealed competent mitral valve or mild mitral insufficiency in 88.8% and 82.2% of GII patients in the early and late postoperative periods, respectively.

Tetralogy of Fallot and hypertrophic cardiomyopathy: a rare association

Tetralogy of Fallot is known as the most common cyanotic congenital heart disease and has a prevalence of 10% of all congenital heart diseases. Although many other heart anomalies may coexist, the association of tetralogy of Fallot and hypertrophic cardiomyopathy is extremely rare. We report this association in a 15-month-old female, cyanotic since birth, in her first hospital admission for diagnosis and treatment of recurring cyanotic crises. In addition, a review of the literature and of the problems related to the treatment is provided.

Electrocardiographic abnormalities in neurological diseases

OBJECTIVE: To evaluate electrocardiographic abnormalities in patients with neurologic diseases. METHODS: We studied 161 patients with neurologic disorders by analyzing the 12-lead electrocardiogram during the pathological process. An expert who did not know anything about the patients evaluated the traces. RESULTS: Neurological process included brain tumor (41%), stroke (27.3%), cerebral aneurysm (15.5%), subarachnoid hemorrhage (6.8%), subdural hemorrhage (5%), and head injury (4.4%). Electrocardiograms were normal in 61% of cases, and the most frequent abnormality was ventricular repolarization (23.7%). The presence of T waves (4.6%) and prolonged QT intervals (8.8%) was the most characteristic of brain injuries. CONCLUSION: We observed a lower incidence of electrocardiographic abnormalities than that described in the literature.

Short- and midterm follow-up results of valvuloplasty with balloon catheter for congenital aortic stenosis

OBJECTIVE: To report short and midtem follow-up results of balloon aortic valvuloplasty to treat congenital aortic stenosis. METHODS: Seventy-five patients (median age: 8 years) underwent the procedure through the retrograde femoral or carotid route. RESULTS: The procedure was completed in 74 patients (98.6%). The peak-to-peak systolic gradient dropped from 79.6±27.7 to 22.3±17.8 mmHg (P<0.001), the left ventricular systolic pressure dropped from 164±39.1 to 110±24.8 mmHg (P<0.001), and the left ventricular end diastolic pressure dropped from 13.3±5.5 to 8.5±8.3 mmHg (P< 0.01). Four patients (5.3%) died due to the procedure. Aortic regurgitation (AoR) appeared or worsened in 27/71 (38%) patients, and no immediate surgical intervention was required. A mean follow-up of 50±38 months was obtained in 37 patients. Restenosis and significant AoR were observed in 16.6% of the patients. The estimates for being restenosis-free and for having significant AoR in 90 months were 60% and 50%, respectively. CONCLUSION: Aortic valvuloplasty was considered the initial palliative method of choice in managing congenital aortic stenosis, with satisfactory short- and midterm results.

Ligation of the thoracic duct for the treatment of chylothorax in heart diseases

In children, chylothorax occurs mainly after cardiac and thoracic surgeries. One of the recommended postsurgery treatments is ligation of the thoracic tract, when all other conservative treatments have failed. We report 4 cases of chylothorax in patients who were successfully treated with this approach, which resulted in a decrease in pleural drainage without recurrent chylothorax.

Prevalencia das Doencas Cardiacas Ilustrada em 60 Anos dos Arquivos Brasileiros de Cardiologia

Considerando a relevância histórica e acadêmica dos Arquivos Brasileiros de Cardiologia, posto que sua indexação no MEDLINE iniciou-se em 1950, assumiu-se como hipótese que a análise das publicações de 60 anos poderia refletir as tendências evolutivas das doenças cardíacas no Brasil. Os dados do trabalho foram coletados com um programa desenvolvido para a finalidade, tornando possível a extração automática das informações do banco de dados MEDLINE. As informações do trabalho foram coletadas pesquisando-se "Arquivos Brasileiros de Cardiologia AND parâmetro selecionado em inglês". Foram arbitrados quatro grupos observacionais: (1) principais grupos de doenças do coração (doença arterial coronariana, valvulopatia cardíaca, cardiopatias congênitas e cardiomiopatias); (2) doenças relevantes na prática clínica (arritmias cardíacas, Cor Pulmonale, infarto do miocárdio e insuficiência cardíaca congestiva); (3) fatores de risco cardiovascular (hipertensão arterial, diabete, dislipidemia e aterosclerose), e; (4) grupo arbitrado em função da evolução crescente das publicações sobre insuficiência cardíaca congestiva constadas nos grupos anteriores (insuficiência cardíaca congestiva, infarto do miocárdio, cardiopatia reumática e cardiopatia chagásica). Foram descritas todas as publicações dentro dos grupos estabelecidos, ressaltando-se o crescente aumento da insuficiência cardíaca e do diabete como fatores de risco. Foi possível um levantamento relativamente fácil, com auxílio do programa de computação desenvolvido para a pesquisa bibliográfica de seis décadas. Ressaltando-se as limitações do estudo, sugere-se a existência de um elo epidemiológico entre as doenças cardiológicas prevalentes no Brasil e as publicações dos Arquivos Brasileiros de Cardiologia.

Association of Peripheral Arterial and Cardiovascular Diseases in Familial Hypercholesterolemia

Background: Familial hypercholesterolemia (FH) is an autosomal dominant genetic disease characterized by an elevation in the serum levels of total cholesterol and of low-density lipoproteins (LDL- c). Known to be closely related to the atherosclerotic process, FH can determine the development of early obstructive lesions in different arterial beds. In this context, FH has also been proposed to be a risk factor for peripheral arterial disease (PAD). Objective: This observational cross-sectional study assessed the association of PAD with other manifestations of cardiovascular disease (CVD), such as coronary artery and cerebrovascular disease, in patients with heterozygous FH. Methods: The diagnosis of PAD was established by ankle-brachial index (ABI) values ≤ 0.90. This study assessed 202 patients (35% of men) with heterozygous FH (90.6% with LDL receptor mutations), mean age of 51 ± 14 years and total cholesterol levels of 342 ± 86 mg /dL. Results: The prevalences of PAD and previous CVD were 17% and 28.2 %, respectively. On multivariate analysis, an independent association between CVD and the diagnosis of PAD was observed (OR = 2.50; 95% CI: 1.004 - 6.230; p = 0.049). Conclusion: Systematic screening for PAD by use of ABI is feasible to assess patients with FH, and it might indicate an increased risk for CVD. However, further studies are required to determine the role of ABI as a tool to assess the cardiovascular risk of those patients.

Congenital Heart Disease as a Warning Sign for the Diagnosis of the 22q11.2 Deletion

Background: To alert for the diagnosis of the 22q11.2 deletion syndrome (22q11.2DS) in patients with congenital heart disease (CHD). Objective: To describe the main CHDs, as well as phenotypic, metabolic and immunological findings in a series of 60 patients diagnosed with 22q11.2DS. Methods: The study included 60 patients with 22q11.2DS evaluated between 2007 and 2013 (M:F=1.3, age range 14 days to 20 years and 3 months) at a pediatric reference center for primary immunodeficiencies. The diagnosis was established by detection of the 22q11.2 microdeletion using FISH (n = 18) and/or MLPA (n = 42), in association with clinical and laboratory information. Associated CHDs, progression of phenotypic facial features, hypocalcemia and immunological changes were analyzed. Results: CHDs were detected in 77% of the patients and the most frequent type was tetralogy of Fallot (38.3%). Surgical correction of CHD was performed in 34 patients. Craniofacial dysmorphisms were detected in 41 patients: elongated face (60%) and/or elongated nose (53.3%), narrow palpebral fissure (50%), dysplastic, overfolded ears (48.3%), thin lips (41.6%), elongated fingers (38.3%) and short stature (36.6%). Hypocalcemia was detected in 64.2% and decreased parathyroid hormone (PTH) level in 25.9%. Decrease in total lymphocytes, CD4 and CD8 counts were present in 40%, 53.3% and 33.3%, respectively. Hypogammaglobulinemia was detected in one patient and decreased concentrations of immunoglobulin M (IgM) in two other patients. Conclusion: Suspicion for 22q11.2DS should be raised in all patients with CHD associated with hypocalcemia and/or facial dysmorphisms, considering that many of these changes may evolve with age. The 22q11.2 microdeletion should be confirmed by molecular testing in all patients.

Electrophysiological Studies and Radiofrequency Ablations in Children and Adolescents with Arrhythmia

Background:Radiofrequency ablation is the standard non-pharmacological treatment for arrhythmias in pediatric patients. However, arrhythmias and their associated causes have particular features in this population.Objective:To analyze the epidemiological characteristics and findings of electrophysiological diagnostic studies and radiofrequency ablations in pediatric patients referred to the Electrophysiology Unit at Instituto de Cardiologia do Rio Grande do Sul, in order to characterize the particularities of this population.Methods:Cross-sectional study with 330 electrophysiological procedures performed in patients aged less than 20 years between June 1997 and August 2013.Results:In total, 330 procedures (9.6% of the overall procedures) were performed in patients aged less than 20 years (14.33 ± 3.25 years, age range 3 months to 19 years), 201 of which were males (60.9%). A total of 108 (32.7%) electrophysiological diagnostic studies were performed and of these, 48.1% showed abnormal findings. Overall, 219 radiofrequency ablations were performed (66.3%) with a success rate of 84.8%. The presence of an accessory pathway was the most prevalent finding, occurring in 158 cases (72.1%), followed by atrioventricular nodal reentrant tachycardia (16.8%), typical atrial flutter (3.1%) and extrasystoles originating from the right ventricular outflow tract (2.7%). Three patients developed complications during ablation (1.4%). Among congenital heart diseases, which occurred in 51 (15.4%) patients, atrial sept defect was the most frequent (27.4%), followed by ventricular sept defect (25.4%) and Ebstein's anomaly (17.6%).Conclusion:Electrophysiological study and radiofrequency ablation are effective tools for diagnosis and treatment of arrhythmias in the pediatric population.

Evolution of Cardiovascular Diseases Mortality in the Counties of the State of Rio de Janeiro from 1979 to 2010

Background: Cardiovascular Diseases (CVD) are the leading cause of death in Brazil. Objective: To estimate total CVD, cerebrovascular disease (CBVD), and ischemic heart disease (IHD) mortality rates in adults in the counties of the state of Rio de Janeiro (SRJ), from 1979 to 2010. Methods: The counties of the SRJ were analysed according to their denominations stablished by the geopolitical structure of 1950, Each new county that have since been created, splitting from their original county, was grouped according to their former origin. Population Data were obtained from the Brazilian Institute of Geography and Statistics (IBGE), and data on deaths were obtained from DataSus/MS. Mean CVD, CBVD, and IHD mortality rates were estimated, compensated for deaths from ill-defined causes, and adjusted for age and sex using the direct method for three periods: 1979–1989, 1990–1999, and 2000–2010, Such results were spatially represented in maps. Tables were also constructed showing the mortality rates for each disease and year period. Results: There was a significant reduction in mortality rates across the three disease groups over the the three defined periods in all the county clusters analysed, Despite an initial mortality rate variation among the counties, it was observed a homogenization of such rates at the final period (2000–2010). The drop in CBVD mortality was greater than that in IHD mortality. Conclusion: Mortality due to CVD has steadily decreased in the SRJ in the last three decades. This reduction cannot be explained by greater access to high technology procedures or better control of cardiovascular risk factors as these facts have not occurred or happened in low proportion of cases with the exception of smoking which has decreased significantly. Therefore, it is necessary to seek explanations for this decrease, which may be related to improvements in the socioeconomic conditions of the population.

Blood Pressure and Hemodynamic Adaptations after a Training Program in Young Individuals with Down Syndrome

Background:Cardiovascular diseases affect people worldwide. Individuals with Down Syndrome (DS) have an up to sixteen-time greater risk of mortality from cardiovascular diseases.Objective:To evaluate the effects of aerobic and resistance exercises on blood pressure and hemodynamic variables of young individuals with DS.Methods:A total of 29 young individuals with DS participated in the study. They were divided into two groups: aerobic training (AT) (n = 14), and resistance training (TR) (n = 15). Their mean age was 15.7 ± 2.82 years. The training program lasted 12 weeks, and had a frequency of three times a week for AT and twice a week for RT. AT was performed in treadmill/ bicycle ergometer, at an intensity between 50%-70% of the HR reserve. RT comprised nine exercises with three sets of 12 repetition-maximum. Systolic blood pressure (SBP), diastolic blood pressure (DBP), mean blood pressure (MBP) and hemodynamic variables were assessed beat-to-beat using the Finometer device before/after the training program. Descriptive analysis, the Shapiro-Wilk test to check the normality of data, and the two-way ANOVA for repeated measures were used to compare pre- and post-training variables. The Pearson’s correlation coefficient was calculated to correlate hemodynamic variables. The SPSS version 18.0 was used with the significance level set at p < 0.05.Results:After twelve weeks of aerobic and/or resistance training, significant reductions in variables SBP, DBP and MBP were observed.Conclusion:This study suggests a chronic hypotensive effect of moderate aerobic and resistance exercises on young individuals with DS.

Palliative Senning in the Treatment of Congenital Heart Disease with Severe Pulmonary Hypertension

Background:Transposition of the great arteries (TGA) is the most common cyanotic cardiopathy, with an incidence ranging between 0.2 and 0.4 per 1000 live births. Many patients not treated in the first few months of life may progress with severe pulmonary vascular disease. Treatment of these patients may include palliative surgery to redirect the flow at the atrial level.Objective:Report our institutional experience with the palliative Senning procedure in children diagnosed with TGA and double outlet right ventricle with severe pulmonary vascular disease, and to evaluate the early and late clinical progression of the palliative Senning procedure.Methods:Retrospective study based on the evaluation of medical records in the period of 1991 to 2014. Only patients without an indication for definitive surgical treatment of the cardiopathy due to elevated pulmonary pressure were included.Results:After one year of follow-up there was a mean increase in arterial oxygen saturation from 62.1% to 92.5% and a mean decrease in hematocrit from 49.4% to 36.3%. Lung histological analysis was feasible in 16 patients. In 8 patients, pulmonary biopsy grades 3 and 4 were evidenced.Conclusion:The palliative Senning procedure improved arterial oxygen saturation, reduced polycythemia, and provided a better quality of life for patients with TGA with ventricular septal defect, severe pulmonary hypertension, and poor prognosis.