Utilidade do Doppler Tecidular na Predição de Eventos Arrítmicos em Adultos com Tetralogia de Fallot Corrigida

INTRODUCTION: Adults with repaired tetralogy of Fallot (TOF) may be at risk for progressive right ventricular (RV) dilatation and dysfunction, which is commonly associated with arrhythmic events. In frequently volume-overloaded patients with congenital heart disease, tissue Doppler imaging (TDI) is particularly useful for assessing RV function. However, it is not known whether RV TDI can predict outcome in this population. OBJECTIVE: To evaluate whether RV TDI parameters are associated with supraventricular arrhythmic events in adults with repaired TOF. METHODS: We studied 40 consecutive patients with repaired TOF (mean age 35 +/- 11 years, 62% male) referred for routine echocardiographic exam between 2007 and 2008. The following echocardiographic measurements were obtained: left ventricular (LV) ejection fraction, LV end-systolic volume, LV end-diastolic volume, RV fractional area change, RV end-systolic area, RV end-diastolic area, left and right atrial volumes, mitral E and A velocities, RV myocardial performance index (Tei index), tricuspid annular plane systolic excursion (TAPSE), myocardial isovolumic acceleration (IVA), pulmonary regurgitation color flow area, TDI basal lateral, septal and RV lateral peak diastolic and systolic annular velocities (E' 1, A' 1, S' 1, E' s, A' s, S' s, E' rv, A' rv, S' rv), strain, strain rate and tissue tracking of the same segments. QRS duration on resting ECG, total duration of Bruce treadmill exercise stress test and presence of exercise-induced arrhythmias were also analyzed. The patients were subsequently divided into two groups: Group 1--12 patients with previous documented supraventricular arrhythmias (atrial tachycardia, fibrillation or flutter) and Group 2 (control group)--28 patients with no previous arrhythmic events. Univariate and multivariate analysis was used to assess the statistical association between the studied parameters and arrhythmic events. RESULTS: Patients with previous events were older (41 +/- 14 vs. 31 +/- 6 years, p = 0.005), had wider QRS (173 +/- 20 vs. 140 +/- 32 ms, p = 0.01) and lower maximum heart rate on treadmill stress testing (69 +/- 35 vs. 92 +/- 9%, p = 0.03). All patients were in NYHA class I or II. Clinical characteristics including age at corrective surgery, previous palliative surgery and residual defects did not differ significantly between the two groups. Left and right cardiac chamber dimensions and ventricular and valvular function as evaluated by conventional Doppler parameters were also not significantly different. Right ventricular strain and strain rate were similar between the groups. However, right ventricular myocardial TDI systolic (Sa: 5.4+2 vs. 8.5 +/- 3, p = 0.004) and diastolic indices and velocities (Ea, Aa, septal E/Ea, and RV free wall tissue tracking) were significantly reduced in patients with arrhythmias compared to the control group. Multivariate linear regression analysis identified RV early diastolic velocity as the sole variable independently associated with arrhythmic history (RV Ea: 4.5 +/- 1 vs. 6.7 +/- 2 cm/s, p = 0.01). A cut-off for RV Ea of < 6.1 cm/s identified patients in the arrhythmic group with 86% sensitivity and 59% specificity (AUC = 0.8). CONCLUSIONS: Our results suggest that TDI may detect RV dysfunction in patients with apparently normal function as assessed by conventional echocardiographic parameters. Reduction in RV early diastolic velocity appears to be an early abnormality and is associated with occurrence of arrhythmic events. TDI may be useful in risk stratification of patients with repaired tetralogy of Fallot.

Tratamento Cirúrgico dos Defeitos do Septo Auriculo-Ventricular. Experiência Uni-Institucional

OBJECTIVES: Atrio-ventricular septal (AVSD) defects include a variable spectrum of congenital malformations with different forms of clinical presentation. We report the surgical results, from a single institution, with this type of congenital cardiac malformation. Patients with hypoplasia of one of the ventricles were excluded from this analysis. POPULATION: Between November of 1998 and June of 2005, 49 patients with AVSD were operated on by the same team and in the same department. The average age was 37.3 months (medium 6 months) and 31 patients were female. In 38 patients (78%) an inter-ventricular communication was present (AVSD-complete) and of these, 26 were of the type A of Rastelli, being 13 of type B or C. The age for defect correction of the complete form was of 5.5 months, palliative surgery was not carried out on any of the patients. Associated lesions included: Down's syndrome in 22 patients (45%), patent arterial duct in 17 patients (35%), severe AV regurgitation in 4 patients (8%), tetralogy of Fallot in two (4%) and sub-aortic stenosis in one patient (2%). Pre-operatively 10 patients presented severe congestive heart failure and two were mechanically ventilated. RESULTS: Complete biventricular correction was carried out in all patients. The average time on bypass (ECC) was 74.1+/-17.5 min. and time of aortic clamping was 52.0+/-12.9 min. The complete defects were corrected by the double patch technique, and in all patients the mitral cleft was closed, except in two with single papillary muscle. There was no intra-operative mortality, but hospital mortality was 8%(4 patients), due to pulmonary hypertension crises, in the first 15 post-operative days. The mean ventilation time was of 36.5+/-93 hours (medium 7 h) and the average ICU stay was of 4.3+/-4.8 days (medium 3 days). The minimum follow-up period is 1 month and the maximum is 84 months (medium 29.5 months), during which time 4 re-operations (8%) took place: two for residual VSD's and two for mitral regurgitation. There was no mortality at re-do surgery. At follow up there was residual mitral regurgitation, mild in 17 patients and moderate in two. Four other patients presented with minor residual defects. CONCLUSIONS: The complete correction of AVSD can be carried out with acceptable results, in a varied spectrum of anatomic forms and of clinical severity. Despite the age of correction, for the complete forms, predominantly below 12 months, pulmonary hypertension was the constant cause for post operative mortality. Earlier timing of surgery and stricter peri-operative control might still improve results.

Cirurgias de Derivação do Ventrículo Pulmonar

Cavopulmonary connections have been extensively used in the palliation of complex forms of congenital heart disease requiring some form of right heart bypass. We examine the mid term outcomes of pulmonary ventricle bypass operations in a single institution and performed by the same surgical team. POPULATION: Between March 1999 and April 2006, 62 patients underwent pulmonary ventricle bypass operations: bidirectional cavopulmonary anastomosis (Glenn procedure), total cavopulmonary connections (Fontan procedure) and one and a half ventricle correction in two cases. Age at operation averaged three years (range: 0.42-25 years) for the Glenn procedure and seven years (range: 3-14 years) for the Fontan procedure. There were 36 male patients (58%) and 26 female patients (42%). The most common indication for surgery was the single ventricle defect, present in 66% of patients. Associated lesions included: transposition of the great arteries in 16 patients (35.6%), bilateral superior vena cava in four patients (8.9%), situs ambigus in five patients (11%), situs inversus in another patient (2.2%), Ebstein disease in one patient (2.2) and coronary fistula in another patient (2.2%). Sub-aortic stenosis was present in one patient (2.2%). Palliative surgery was performed in all, but three patients (5%), before the Fontan procedure. RESULTS: Thirty two patients underwent bidirectional cavopulmonary anastomosis and thirty patients underwent cavopulmonary connections, total or 2nd stage. Mean cardiopulmonary bypass times were 50.6+/-21.9 minutes for the Glenn procedure and 88.5+/-26.3 minutes for the Fontan procedure. There was no intra-operative mortality, but two patients (3.2% (died in the first month after surgery; one due to failure of the Glenn circuit and sepsis and the other due to a low cardiac output syndrome and multi-organ dysfunction. Mean ventilation time was 5.2+/-1.7 hours for the Glenn operation and 6.2+/-3.2 hours for the Fontan operation. The mean length of stay in ICU was 3.4+/-2.8 days for patients undergoing the Glenn operation and 4.6+/-3.1 days for patients undergoing the Fontan operation and the mean length of hospital stay was 10.6+/-5.8 days for the Glenn operation and 19.1+/-12.6 days for the Fontan operation respectively. The mean follow up time was 4+/-2.1 years (minimum 0 years and maximum seven years), most patients being in NYHA class I. Epicardiac pacemakers were implanted in three patients due to arrhythmias. Two re-operations (6.7%) were needed, both in the same patient, after the Fontan procedure, this patient eventually died a few years after surgery. CONCLUSIONS: The immediate and mid term outcomes of pulmonary ventricle bypass operations can have excellent results. From our point of view there has been an improvement, namely in the use of the extracardiac conduit technique in the 2nd stage of the Fontan operation.

Toxoplasma-IgM and IgG-avidity in single samples from areas with a high infection rate can determine the risk of mother-to-child transmission

Anti-Toxoplasma IgG-avidity was determined in 168 serum samples from IgG- and IgM-positive pregnant women at various times during pregnancy, in order to evaluate the predictive value for risk of mother-to-child transmission in a single sample, taking the limitations of conventional serology into account. The neonatal IgM was considered the serologic marker of transmission. Fluorometric tests for IgG, IgM (immunocapture) and IgG-avidity were performed. Fifty-one of the 128 pregnant women tested gave birth in the hospital and neonatal IgM was obtained. The results showed 32 (62.75%) pregnant women having high avidity, IgM indexes between 0.6 and 2.4, and no infected newborn. Nineteen (37.25%) had low or inconclusive avidity, IgM indexes between 0.6 and 11.9, and five infected newborns and one stillbirth. In two infected newborns and the stillbirth maternal IgM indexes were low and in one infected newborn the only maternal parameter that suggested fetal risk was IgG-avidity. In the present study, IgG-avidity performed in single samples from positive IgM pregnant women helped to determine the risk of transmission at any time during pregnancy, especially when the indexes of the two tests were analysed with respect to gestational age. This model may be less expensive in developing countries where there is a high prevalence of infection than the follow-up of susceptible mothers until childbirth with monthly serology, and it creates a new perspective for the diagnosis of congenital toxoplasmosis.

Survival of Trypanosoma cruzi in sugar cane used to prepare juice

Chagas disease can be transmitted to man by many different means, including contact with infected triatomine feces, blood transfusion, laboratory accidents, organ transplants, and congenital or oral routes. The latter mode has received considerable attention recently. In this assay, we evaluate the survival of Trypanosoma cruzi contaminating sugar cane used to prepare juice, as well as the viability and capacity for infection by the parasite after recovery. Thirty triatomines were contaminated with T. cruzi Y strain and 45 days later pieces of sugar cane were contaminated with the intestinal contents of the insects. The pieces were ground at different intervals after contamination (time = 0, 1, 4, 6, 12 and 24 hours) and the juice extracted and analyzed. Different methods were used to show T. cruzi in the juice: direct analysis, hematocrit tube centrifugation and QBC, and experimental inoculation in 47 female BALB/c mice (five control mice and seven mice for each interval examined (five inoculated orally and two intraperitoneally). Positive results were found using the direct analysis and QBC methods for juice prepared up to 12 hours after initial contamination. However, by the centrifugation technique, positivity was found only up to four hours after contamination of the sugar cane. Inoculated animals showed parasitemia during a 14 day observation period, demonstrating the high survival rate of T. cruzi in sugar cane.

Vasodilatadores Pulmonares Selectivos na Hipertensão Pulmonar Grave: Utilização de 3 Diferentes Tipos de Endpoint

The therapeutic approach to severe pulmonary arterial hypertension (PAH), whether primary or secondary to connective tissue disorders, thromboembolic phenomena or congenital heart disease with Eisenmenger syndrome, has evolved in recent years following the introduction of selective pulmonary vasodilators, including prostacyclin analogs and endothelin receptor antagonists. AIM: To correlate three different endpoints (6-minute walk test, Tei index and peak tricuspid regurgitation velocity by Doppler echocardiographic study) during follow-up of PAH patients under selective vasodilator therapy. METHODS: Eleven patients (9 female, age 42 +/- 18 years) with severe PAH (> or = 65 mmHg), 64% with Eisenmenger syndrome, in NYHA class > or = II, were assessed during a follow-up of 11 +/- 8 months. Eight patients were already under therapy with iloprost or bosentan. RESULTS: There was no correlation between the three endpoints before and after therapy as assessed by Pearson's correlation coefficient. There was, however, an improvement in all of them after selective vasodilatory therapy. CONCLUSION: Therapeutic response can be accurately measured by the traditional endpoint (6-minute walk test) or by echocardiographic endpoints. However, the lack of correlation between them excludes their use as alternatives in patient follow-up.

Resultado do Obstétrico de Gravidezes Gemelares Resultantes de Fecundação in Vitro, Microinjeção e Estimulação da Ovulação em Comparação com Espontâneas

Introduction: Uncontrolled studies suggest that twins conceived by in vitro fertilization have increased rates of preterm birth and low birth weight and would warrant increased antenatal monitoring. The objective of this study was to compare the obstetric outcome of twin pregnancies conceived by in vitro fertilization or intracytoplasmic sperm injection (IVF/ICSI) and ovulation induction with those conceived spontaneously. Methods: All twin deliveries achieved by IVF/ICSI (n=235) and ovulation induction (n=68) from September 1994 through December 2010 were evaluated. Both groups and an additional control group who conceived spontaneously (n=997) and was delivered during the same time period were compared with each other. Results: In univariate analysis, patients who conceived with the assistance of IVF/ICSI had a significantly higher risk of being older (p=0.01), nulliparous (p=0.01), having hypertensive disorders (p=0.012), gestational diabetes mellitus (p=0.031), cesarean section (p=0.008) and lower gestational age at birth, compared with the control group. Newborns had similar birthweights in all groups (2229±544g; 2102±619g; 2251±553g). Spontaneous pregnancies had a higher risk of being monochorionic 38.4% versus 16.2% and 10.2% (p=0.01). Multivariate analysis however showed that patients who conceived with the assistance of IVF/ICSI only had a higher risk of gestational diabetes (OR=1.91,95%CI 1.168-3.120; p=0.01). Conclusions: Our study shows that twin pregnancies conceived with the assistance of IVF/ICSI had a higher risk of gestacional diabetes and a lower gestacional age at birth. Birthweights were similar, as was the incidence of perinatal death, low birth weight infants, and congenital malformations.

A Sífilis Congénita Ainda Existe! Análise Retrospectiva de 12 Anos de uma Grande Maternidade

Objectivos: Estudar a prevalência, factores de risco, evolução clínica e abordagem terapêutica da sífilis congénita em recém-nascidos (RN) de risco, nascidos numa maternidade de referência com apoio perinatal diferenciado. Método: Realizou-se um estudo transversal para cálculo de prevalência à nascença de sífilis congénita, entre Janeiro de 1993 e Dezembro de 2004, através de recolha de dados registados nos processos clínicos das mães e respectivos RN. De acordo com os critérios definidos pelo Centers for Disease Control and Prevention (CDC) em 1989, os RN filhos de mãe com VDRL e/ou TPHA positivo foram divididos em três grupos de risco. Resultados: Foram identificados 467 recém-nascidos, verificando-se que a prevalência de risco de sífilis congénita à nascença se tem mantido ao longo dos anos (5,6‰). A maioria dos recém-nascidos (65%) enquadra-se no grupo de maior risco. Dezanove RN (4%) apresentaram sífilis congénita sintomática ao nascimento, a maioria pertencente ao grupo de maior risco. Outros factores de risco encontrados foram a gravidez não-vigiada, em 30% das mães, toxicodependência em 9%, coinfecção por vírus da hepatite B em 5%, por vírus da hepatite C em 4,7% e por vírus de imunodeficiência humana em 3,4% dos casos. Em alguns casos existia mais do que um factor de risco associado. Conclusões: Verificou-se que a prevalência de risco de sífilis congénita não sofreu grandes variações ao longo dos doze anos, pelo que a sífilis continua a constituir um problema de Saúde Pública em Portugal, com custos económicos e sociais.

Dermoid Tumor of the Lateral Wall of the Cavernous Sinus

BACKGROUND: Congenital intracranial dermoid tumors are very rare. The location of these dermoid lesions in the cavernous sinus and the complexity of the operative procedure for these lesions have been noted by several authors. Dermoid tumors originating in the cavernous sinus are usually interdural, and thus blurred vision is an uncommon presentation. CASE DESCRIPTION: Herein we report the first incidental case of a cavernous sinus dermoid cyst in a 21-year-old woman. CONCLUSIONS: A literature review was done and the possible treatments and approaches for this lesion are discussed. We consider that surgical treatment is indicated in most incidental cavernous sinus dermoid lesions due to the possible symptoms related to compression or rupture leading to chemical meningitis.

Correlation between specific IgM levels and percentage IgG-class antibody avidity to Toxoplasma gondii

Toxoplasmosis is an usually asymptomatic worldwide disseminated infection. In its congenital presentation it may lead to abortion or fetal malformations. Antenatal evaluation is considered of paramount importance to identify seronegative women and allow for prophylaxis. Recent improvements in sensitivity of IgM tests has made IgM detection an extremely protracted acute phase marker, and IgG avidity evaluation test became necessary. Observation has shown that a correlation can be established between IgM levels and avidity percentages, suggesting that frequently the avidity test may not be necessary. In this study we analyzed Toxoplasma gondii IgM levels of 202 samples and their IgG avidity percentages, in order to define specific levels whose IgM quantification could by itself define serodiagnosis and therefore make the avidity evaluation unnecessary. We showed that for IgM levels bellow 2.0 and above 6.0 serodiagnosis of toxoplasmosis could be established without need of IgG avidity test. IgM levels between these two parameters are associated with varying avidity indexes highlighting the importance of its evaluation as a means to confirm toxoplasmosis. Following this demonstration it was possible to avoid the avidity test for 75% of the cases, to reduce the turnaround time and to reduce costs.

Seroepidemiology of toxoplasmosis in women of childbearing age from a marginal community of Maracaibo, Venezuela

The aim of this study was to determine the prevalence of anti-T. gondii total and IgM antibodies in women of childbearing age. One hundred serum samples of women were studied with age range from 11 to 45 years old. Samples were chosen by random. The determination of total antibodies was carried out through the indirect hemagglutination technique and IgM antibodies by ELISA's technique. The statistical analysis was carried out through the Chi square and the Spearman correlation tests. The theoretical estimated incidence of congenital toxoplasmosis was calculated, according to the annual increment of antibody prevalence among the age groups. The overall prevalence of toxoplasmosis was 33%, while only six individuals (18.2%) were positive to IgM. The highest prevalence was observed in the 11-35 year-old age group. The theoretical estimated incidence was 1.5 for 100 pregnancies in women of 21-25 year-old group; it decreased until 0.1% in the 41-45 year-old age group. The findings show a high prevalence of toxoplasmosis in this community with a high infection risk in women of the studied age group and the high cat population observed, suggesting that the transmission way by contaminated soils may play a main role in the spreading of toxoplasmosis in this community.

Pilosidade Púbica na Criança e Hiperplasia Congénita Supra-Renal Não Clássica

Antes dos 8 anos de idade no sexo feminino e dos 9 no masculino, o aparecimento de pelos púbicos, pode ser a primeira manifestação de doença das gónadas ou das supra-renais. Para reconhecimento da Hiperplasia congénita supra-renal de início tardio, em situações de Pubarca prematura, realizámos a Prova curta de estimulação da supra-renal com Synacthen, em trinta crianças. Cinco meninas e um rapaz revelaram um défice de 21-hidroxilase, demonstrado pelo valores da 17-OH Progesterona sérica aos 60 minutos;28,8 +_ 15,2ng/ml, sendo num grupo de controle 2,30 ng/ml (p<0,01). A incidência de 20% encontrada, justifica a realização desta prova em todas as Pubarcas prematuras.

Partial Rhombencephalosynapsis.

Clinical history - A 4-year-old boy, born prematurely at 29 weeks (twin pregnancy), with periventricular leukomalacia and epilepsy underwent brain MRI. Neurological examination showed severe developmental retardation with axial hypotonia, spastic tetraparesis and convergent strabismus. Imaging findings - Cranial MRI revealed typical aspects of partial rhombencephalosynapsis with vermian hypoplasia, midline fusion of the cerebellar hemispheres and transversely oriented folia and fissures. There was also mild dilatation and dysmorphism of the ventricular system, the septum pellucidum was absent, the hippocampi were malrotated and had vertical orientation and additional finding of associated periventricular cystic leukomalacia. Discussion - Rhombencephalosynapsis (RS) is a rare congenital defect of the cerebellum classically characterised by vermian agenesis or hypogenesis, fusion of the hemispheres, and closely apposed or fused dentate nuclei. It is now considered to result from an absence of division of the cerebellar hemispheres, following an insult between the 28th and 44th day of gestation (i.e., before the formation of the vermis). Other features have also been described such as fusion of the thalami and cerebral peduncles, malrotated hippocampi, corpus callosum agenesis, hypoplastic chiasm, absence of the septum pellucidum, ventriculomegaly, agenesis of the posterior lobe of the pituitary and cortical malformations. Musculoskeletal, cardiovascular, urinary tract, and respiratory abnormalities have been reported. Typical symptoms consist of swallowing difficulties, delayed motor acquisitions, muscular hypotonia, spastic quadriparesis, cerebellar signs including dysarthria, gait ataxia, abnormal eye movements, and seizures and hydrocephalus. The major MRI signs consist of fused cerebellar hemispheres, with absent or hypoplastic vermis, narrow diamond-shaped fourth ventricle and fused dentate nuclei. In a minority of cases, partial RS has been identified by MRI, demonstrating the presence of the nodulus and the anterior vermis and absence of part of the posterior vermis with only partial fusion of the hemispheres in the inferior part. Other cerebellar malformations involving vermian agenesis or hypoplasia include the Dandy–Walker continuum, Joubert syndrome, tectocerebellar dysraphy or pontocerebellar hypoplasias, and are now easily distinguished from RS by both brain MRI and morphology.

Perinatal Death: Epidemiology and Etiology

Perinatal mortality rate is an important mark to evaluate women and perinatal health care. It is of utmost importance to know causes and the evolution of its two components aiming to improve health care in different fields – sanitary conditions, diagnosis and treatment of infectious disease, immunisations, diagnosing and caring for medical diseases induced by pregnancy or directly related to it, providing skilled birth attendance, preventing birth asphyxia, preventing preterm birth complications and infections. In high-income countries the epidemiology varies mainly with social and economic conditions; in low-income countries, paired with poverty, undernutrition, superstition, lack of medical care, deficient basic sanitary conditions are also found. Also, in rich countries, responsible for 1% of deaths, data are published and improvements evaluated, while in low-income countries responsible for 99% of deaths numbers and causes are unknown, making difficult to implement cost effective interventions, a reason why “stillbirth rates in low-income countries are now where they were in high-income countries 50 to 100 years ago”. Knowledge on causes of death are very important as often what is needed are “simple” measures as improvement of sanitary conditions and immunisation programmes rather than high technologies. About four million babies dye each year in the first 28 days of life and another 3 million dye before birth in the third-trimester, with 98% occurring in low-income and middle income countries and more than 1 million occurring during labour and delivery. Classically stillbirths are the major component of perinatal mortality rate. Causes of death are even more difficult to know. In low-income countries a great proportion of women give birth at home. Worldwide the main causes of stillbirth are asphyxia due to obstructed labour, eclampsia, abruption placenta and umbilical cord complications - making valid the assumption that skilled birth attendance would decrease stillbirth; and infection - chorioamnioitis, syphilis and malaria. In high-income countries placental pathology and infection, congenital anomalies, complications of preterm birth and post term delivery, are the most common. If in low-income countries famine and lack of provisions and health care are common, in high-income countries, advanced maternal age and diabetes, obesity, hypertension, smoking, are frequent findings.