Arterial stiffness in children and adolescents with and without continuous insulin infusion.

Background Arterial stiffness is a consequence of aging, but there are several diseases that contribute to this process. The evaluation of pulse wave velocity (PWV) allows a dynamic evaluation of vascular distensibility and the detection of atherosclerosis at an early stage. It was intended to evaluate the PWV in children and adolescents with type 1 diabetes mellitus (T1DM) and to compare their outcome according to the type of treatment used. Methods Forty-eight patients were randomly selected. Inclusion criteria: T1DM, under intensive insulin therapy (multiple daily insulin administrations [MDI] or continuous insulin infusion system [CIIS]). Exclusion criteria: existence of another chronic pathology or microvascular complications. Echocardiography was performed and three measurements of PWV were done, with their mean calculated. Results Most of the children and adolescents presented a PWV ≥ the 75th centile. There was a statistically significant difference for hemoglobin A1c (HbA1c) (7.8 in CIIS vs. 9 in MDI, p < 0.05). There were not statistically significant differences in the PWV between the two groups. This can be attributed to the fact that children with CIIS are those who previously presented greater glycemic instability. There was a significant correlation between PWV and disease duration (Pearson's correlation coefficient [r] = 0.314, p = 0.036). Conclusions This study showed that in children and adolescents with T1DM, there is an important prevalence of arterial stiffness, translated by an increase in PWV. This increase in PWV appears to exist even in very young children with little disease evolution time.

Intraoperative transesophageal echocardiogram using an intracardiac ultrasound catheter in a congenital heart surgery: a case report.

Transesophageal echocardiography (TEE) during cardiac surgery is a routine procedure. The use of pediatric TEE probes is limited in small infants weighing less than 5 kg. Recent reports have shown the safety of monoplane intravascular ultrasound catheters in transesophageal echocardiograms. This report describes the case of a newborn with total anomalous pulmonary venous return who underwent cardiac surgery. A pre- and postbypass TEE examination was performed, with successful visualization of the cardiac anatomy and function and no complications.

First-degree relatives of patients with early-onset gastric carcinoma show even at young ages a high prevalence of advanced OLGA/OLGIM stages and dysplasia.

BACKGROUND: First-degree relatives (FDRs) of early-onset gastric carcinoma (EOGC) patients are at increased risk of cancer development. OLGA/OLGIM (Operative Link on Gastritis/Intestinal Metaplasia Assessment) classifications have been proposed for the identification of individuals at high risk of gastric cancer development. AIM: To estimate the prevalence and severity of premalignant conditions and lesions in FDRs of EOGC patients. METHODS: A case-control study was conducted encompassing 103 FDRs of EOGC patients (cases) and 101 age- and gender-matched controls, all submitted to upper GI endoscopy and OLGA and OLGIM used for staging as well as modified versions with exclusion of the biopsies from incisura angularis in the analysis. RESULTS: Helicobacter pylori infection was present in 82% of cases (P = 0.001). Atrophy was present in 70% of cases (OLGA stages I-IV). High-risk stages (III-IV) were identified only in cases (19%) (P < 0.001). Dysplasia was diagnosed only in cases (n = 7, P = 0.007). The application of OLGIM, modified OLGA and modified OLGIM classifications led to downgrade of stages in comparison with the original OLGA classification (27%, 15% and 30% respectively). In all classification systems, dysplastic lesions clustered (86%) in high-risk stages. CONCLUSIONS: FDRs of EOGC patients have, even at young ages, a high prevalence of H. pylori infection, high-risk OLGA and OLGIM stages and dysplasia. These patients should undergo accurate endoscopic observation with at least four biopsies in antrum and corpus to allow adequate staging and follow-up of premalignant conditions and lesions scored in high-risk stages, in accordance with international guidelines recently proposed.

[Sinister cor triatriatum in an asymptomatic child]. / Cor triatriatum sinister em criança assintomática.

Cor triatriatum is a very rare congenital abnormality, usually symptomatic during childhood. The authors report the case of an asymptomatic 4-years old boy with two orifices in the cor triatriatum membrane. Surgical correction, with total removal of the membrane, was successfully accomplished.

The importance of ultrasound findings in the study of anal pain.

OBJECTIVE: Endoanal ultrasonography can detect organic causes of anal pain without pathology on physical examination. The aim of this study is to evaluate the importance of endoanal ultrasonography in the diagnosis and therapeutic management of idiopathic and functional anal pain. MATERIAL AND METHODS: Retrospective study, between 15 March 2005 and 15 June 2008, of all patients with proctalgia and normal examination or with alterations not responsible for anal pain at proctologic exam that have undergone an endoanal ultrasonography. RESULTS: A total of 90 patients were analyzed, with a mean age of 50.5 years, 58% were female. Twenty-three patients had functional anal pain clinic criteria. Endoanal ultrasonography revealed alterations in 49% of patients. The primary findings were changes in sphincters in 14 patients, followed by anal sepsis in 12 patients, anal fissure in 10 patients, perirectal lesions in 6 patients and ulcer of the anal canal in 2 patients. Of the patients with sphincter defects, 5 patients had criteria of chronic anal pain. In this group of patients, no differences were found in manometric and defecographic results between the different ultrasound abnormalities. CONCLUSIONS: The endoanal ultrasonography detected occult organic lesions to proctologic examination, in half the patients with anal pain. Ultrasound abnormalities were found in 22% of patients with functional anal pain. However, there was no correlation between ultrasound findings and physiological studies, and therefore could not find etiological or pathogenic factors of functional anal pain.

Importancia de los hallazgos ecográficos en el dolor anal / No disponible

Objetivo: la ecografía endoanal puede detectar causas orgánicasen el dolor anal sin patología en la exploración física. El objetivode este estudio es evaluar la importancia de la ecografía endoanalen el diagnóstico y en el abordaje terapéutico del dolor analidiopática y funcional.Material y métodos: estudio retrospectivo realizado entre el15 de marzo de 2005 y el 15 de junio de 2008, en todos los pacientesreferenciados para hacerse la ecografía endoanal, debido adolor anal con exploración física normal o sin alteraciones que justifiquenel dolor anal.Resultados: analizamos 90 pacientes con edad media de50,5 años, 58% mujeres. Veintitrés pacientes presentaban criteriosde dolor anal funcional. La ecografía endoanal reveló alteracionesen el 49% de los casos. Las alteraciones más frecuentesfueron las alteraciones esfinterianas, en 14 pacientes,seguido de sepsis anal, en 12 pacientes, de fisura anal, en 10pacientes, de las lesiones perirrectales, en 6 pacientes y de úlceradel canal anal, en 2 pacientes. De los pacientes con alteracionesesfinterianas, 5 pacientes tenían criterios de dolor crónico.En este grupo de pacientes, no se encuentran diferenciasen los hallazgos manométricos y defecográficos entre las diferentesalteraciones ecográficas.Conclusiones: la ecografía endoanal detectó lesiones estructuralesno sospechadas en el examen físico en la mitad de los enfermoscon dolor anal. Se han encontrado alteraciones ecográficasen el 22% de los pacientes con dolor anal funcional. Sinembargo, no se encontró correlación entre los hallazgos ecográficosy los estudios fisiológicos, así que no fue posible encontrar factoresetiológicos o patogénicos del dolor anal funcional(AU)

Objective: endoanal ultrasonography can detect organic causesof anal pain without pathology on physical examination. Theaim of this study is to evaluate the importance of endoanal ultrasonographyin the diagnosis and therapeutic management of idiopathicand functional anal pain.Material and methods: retrospective study, between 15March 2005 and 15 June 2008, of all patients with proctalgiaand normal examination or with alterations not responsible foranal pain at proctologic exam that have undergone an endoanalultrasonography.Results: a total of 90 patients were analyzed, with a meanage of 50.5 years, 58% were female. Twenty-three patients hadfunctional anal pain clinic criteria. Endoanal ultrasonography revealedalterations in 49% of patients. The primary findings werechanges in sphincters in 14 patients, followed by anal sepsis in 12patients, anal fissure in 10 patients, perirectal lesions in 6 patientsand ulcer of the anal canal in 2 patients. Of the patients withsphincter defects, 5 patients had criteria of chronic anal pain. Inthis group of patients, no differences were found in manometricand defecographic results between the different ultrasound abnormalities.Conclusions: the endoanal ultrasonography detected occultorganic lesions to proctologic examination, in half the patientswith anal pain. Ultrasound abnormalities were found in 22% ofpatients with functional anal pain. However, there was no correlationbetween ultrasound findings and physiological studies, andtherefore could not find etiological or pathogenic factors of functionalanal pain(AU)

Nitric oxide and prostaglandins - important players in endothelin-1 induced myocardial distensibility.

This study investigated whether endothelin (ET)-1-induced increase in myocardial distensibility is preserved in heart failure (HF) and whether it is modulated by nitric oxide (NO) and prostaglandins. New Zealand white rabbits were treated with doxorubicin (1 mg/kg, intravenously twice a week for 8 weeks, DOX-HF group) or saline (control group). Effects of ET-1 (0.1, 1, 10 nM) were tested in papillary muscles from the DOX-HF group and a control group in the presence of: i) intact endocardial endothelium (EE); ii) damaged EE; iii) N(G)-nitro-L-arginine (L-NNA; NO synthase inhibitor), and iv) indomethacin (INDO; cyclooxygenase inhibitor). In the presence of an intact EE, ET-1 promoted concentration-dependent positive inotropic and lusitropic effects that were maintained after damaging the EE, in the presence of L-NNA or INDO and in the DOX-HF Group. ET-1 reduced resting tension at the end of the isometric twitch (increased diastolic distensibility) by 3.2+/-1.3 %, 6.0+/-1.6 % and 8.8+/-2.7 % (at 0.1, 1 and 10 nM, respectively), in muscles with intact EE, effect that was completely abolished after damaging EE, in the presence of L-NNA or INDO or in the DOX-HF Group. This study demonstrated that the increase in myocardial distensibility induced by ET-1 is absent in HF and is dependent of NO and prostaglandin release.

Focos hiperecogénicos intracardíacos: ¿cuál puede ser su relevancia? / Intracardiac hiperechogenic foci: how relevant can they be?

Objetivos. Evaluar la incidencia de focos hiperecogénicos intracardíacos en fetos y reflexionar sobre su significado clínico. Material y métodos. Estudio retrospectivo en dos centros de diagnóstico prenatal durante dos años. Se seleccionaron casos en los que había focos hiperecogénicos intracardíacos. Se analizó: edad materna, edad gestacional, motivo de referencia para la consulta, localización y número de focos hiperecogénicos encontrados, existencia de anomalías cromosómicas y defectos cardíacos. Fueron excluidos los casos de aneuploidía. Resultados. Se revisaron 753 fichas clínicas, seleccionándose 61 fetos (8 por ciento); la edad media materna fue de 29 años (mínimo 19, máximo 43). La edad gestacional media fue de 23,4 semanas (mínimo 19, maximo 31). En el 48 por ciento de los casos el motivo de referencia para la realización del examen ecocardiográfico fetal fue la existencia de focos hiperecogénicos intracardíacos detectado en exámenes obstétricos maternos de rutina. En el 13 por ciento de los casos, el motivo fue la translucencia nucal aumentada. Entre otros motivos de referencia se destacan: edad materna en el 10 por ciento, existencia de historia familiar de cardiopatía congénita en el 8 por ciento, embarazos múltiples en el 4 por ciento, sospecha de defecto cardíaco en el 4 por ciento, abortos de repetición en el 2 por ciento. La patología materna diversa, alteraciones del cribado bioquímico y anomalías del cordón umbilical, fueron responsables del 10 por ciento de las ecocardiografías fetales. En 53 casos se encontró un foco hiperecogénico aislado y en 44 casos el foco se encontraba en el ventrículo izquierdo. Cinco fetos presentaron cardiopatía congénita. Conclusiones. Los focos hiperecogénicos intracardíacos son hallazgos frecuentes en ecografias obstétricas de rutina. Probablemente representan una variable normal del desarrollo del corazón fetal, pero su presencia debe ser interpretada como riesgo aumentado de existencia de defectos congénitos cardíacos asociados (AU)

One year of Lamivudine therapy for portuguese patients with chronic hepatitis B.

OBJECTIVE: To assess the efficacy of lamivudine treatment on hepatitis B e antigen (HBeAg) and/or hepatitis B surface antigen (HBsAg) seroconversion, on other virological and serological markers of response including hepatitis B virus (HBV) DNA and serum aminotransferases, and the safety of lamivudine treatment in hepatitis B patients. PATIENTS: This phase III open-label study evaluated the virological and biochemical response to lamivudine in 70 Portuguese patients with HBeAg positive chronic hepatitis B. Patients were treated with lamivudine 100mg once daily for 12 months. METHODS: Antiviral activity was assessed by measuring alanine aminotransferase (ALT)/aspartate aminotransferase (AST) levels at all protocol visits, and hepatitis B serology and HBV DNA were performed at baseline and at month 12 visits. Evaluation of safety and tolerance was based on clinical adverse events and laboratory analyses. RESULTS: The primary endpoint was virological response at month 12, defined as loss of detectable HBeAg from serum with a reduction of HBV DNA to undetectable levels, and this was observed in 19/69 (27.5%) of patients. Almost half of the patients were HBV DNA negative by this time. Mean ALT values decreased steadily during treatment and by 12 months 61% of patients had values within the normal range. HBeAg seroconversion (HBeAg negative, HBeAb positive) was achieved in 27.9% of patients by 12 months, although all patients remained HBsAg positive. CONCLUSION: Lamivudine was well tolerated and the incidence of adverse events was similar to those reported in previous studies. Lamivudine treatment resulted in virological and biochemical improvements in HBeAg positive chronic hepatitis B patients, with HBeAg seroconversion in one-third of patients.

Clinical and genetic heterogeneity in hereditary haemochromatosis: association between lymphocyte counts and expression of iron overload.

To identify a new marker of expression of disease, independent of HFE genotype in patients with hereditary haemochromatosis (HHC), the total peripheral blood lymphocyte counts were analysed according to iron status in two groups of subjects with HFE mutations. The groups consisted of 38 homozygotes for C282Y, and 107 heterozygotes for the C282Y or compound heterozygotes for C282Y and H63D. For control purposes, total lymphocyte counts and iron status were also examined in 20 index patients with African dietary iron overload, a condition not associated with HFE mutations, and in 144 members of their families and communities. Mean lymphocyte numbers were lower in C282Y homozygous HHC index subjects with cirrhosis and higher iron stores than in those without cirrhosis and with lower iron burdens [(1.65 +/- 0.43) x 10(6)/mL vs. (2.27 +/- 0.49) x 10(6)/mL; p = 0.008]. Similarly, mean lymphocyte counts were significantly lower in C282Y heterozygotes and C282Y/H63D compound heterozygotes with iron overload and increased serum ferritin concentrations compared to those with normal serum ferritin concentrations (p < 0.05). Statistically significant negative correlations were found, in males, between lymphocyte counts and the total body iron stores, either in C282Y homozygous HHC patients (p = 0.031 in a multiple regression model dependent on age) and in C282Y heterozygotes or C282Y/H63D compound heterozygotes with iron overload (p = 0.029 in a simple linear model). In contrast, lymphocyte counts increased with increasing serum ferritin concentrations among the index subjects with African iron overload (r = 0.324, not statistically significant) and among the members of their families and communities (r = 0.170, p = 0.042). These results suggest that a lower peripheral blood lymphocyte count is associated with a greater degree of iron loading in HFE haemochromatosis but not in African iron overload, and they support the notion that the lymphocyte count may serve as a marker of a non-HFE gene that influences the clinical expression of HFE haemochromatosis.

[Prognostic indexes derived from ventricular morphology in congenital diaphragmatic hernia induced by nitrofen]. / Indices de prognóstico derivados da morfologia ventricular na hérnia diafragmática congénita induzida pelo nitrofeno.

BACKGROUND: Some indices derived from cardiac morphology have been proposed as prognostic factors in fetuses and newborns with congenital diaphragmatic hernia (CDH). These would be of interest in evaluating the risk of pulmonary hypoplasia and the need for surgery in the fetus. The aim of this study was to evaluate the heart-related indices in the nitrofen-induced CDH rat model. METHODS: At day 9 1/2 of gestation dated pregnant female Wistar rats received 100 mg of nitrofen. Fetuses were harvested at day 21 1/2 by laparotomy. The fetuses exposed to nitrofen without CDH were defined as the control group (n = 38) while the fetuses exposed to nitrofen with CDH were defined as the CDH group (n = 30). The hearts from both groups were weighed, processed for paraffin embedding and fragmented in serial 7 microns thick transverse sections. In the hearts without cardiac morphological defects the right and the left ventricular cavity volumes were estimated. These allowed calculation of cardioventricular and left-ventricular mass indices. RESULTS: In the fetuses without cardiac malformations, no significant difference was found regarding heart-to-body weight ratio, cardioventricular index or left ventricular mass between the study groups. CONCLUSION: In this model the presence of CDH was not associated with any alteration in cardiac dimension, in the absence of structural malformations. These results suggest that the indices derived from ventricular morphology should not be used to evaluate the prognosis in fetuses and newborns with CDH.

Ebstein's anomaly in neonates.

Ebstein's anomaly is a rare congenital heart disease abnormality in which the tricuspid valve leaflets do not attach normally to the tricuspid valve annulus. The effective tricuspid valve orifice is displaced apically into the right ventricle (RV), near the junction of the inlet and the trabecular parts of the RV. The authors present a retrospective study of the patients with Ebstein's anomaly admitted to a neonatal intensive care unit, in the period between January 1993 and March 2000. There were ten patients, representing 0.24% of total neonates and 1.99% of total congenital heart disease admitted to the institution in the same period. Fifty per cent were male and only one case had prenatal diagnosis. Holosystolic murmur (100%) from tricuspid regurgitation and cyanosis (80%) were the most frequent clinical findings. Chest X-ray was abnormal in 90% of the neonates, with a "balloon-shaped" enlarged heart. The main electrocardiographic findings were right atrial enlargement (70%) and arrhythmias (40%). Apical displacement of the septal leaflet of the tricuspid valve, to a maximum of 20 mm, and leaflets tethering to underlying RV myocardium were found in all patients. Tricuspid valve regurgitation was found in 90% (severe form in four cases). An atrial intracardiac shunt, mostly right-to-left, was also found in 50%. Digoxin was used (40%) to restore sinus rhythm. Fifty per cent of the neonates received intravenous prostaglandins. Two patients required a surgical procedure. Two patients died in the neonatal period. During the follow-up period (range 0.3-74.6 months), only one episode of supraventricular tachycardia was recorded. At present seven patients are clinically stable, three of them on medication.

Heart-related indices in experimental diaphragmatic hernia.

BACKGROUND: Heart-related indices have been suggested as useful tools to evaluate left ventricular (LV) hypoplasia, which might predict the outcome of fetuses and infants with congenital diaphragmatic hernia (CDH). The current study analyzed the behavior of such indices in the nitrofen-induced CDH rat model. METHODS: Dated pregnant Wistar rats received at day 9.5 of gestation either a dose of 100 mg of nitrofen or just the vehicle. Body, lung, and heart weights were measured in 12 newborn rats not exposed to nitrofen (Ctrl group) and 68 animals exposed to nitrofen: 30 without CDH (non-CDH group) and 38 with left CDH (CDH group). Each heart was fragmented in 7-microm thick sections. Only hearts with no evidence of cardiac morphologic defects (CMD) were studied further to estimate right and left ventricular cavity volumes, septal, right, and left ventricular free wall masses. These parameters allowed the calculation of the cardio-ventricular (CVindex) and LV mass indices. The aorta-to-pulmonary artery ratio also was calculated. RESULTS: Excluding fetuses with CMD, the heart-to-body weight ratio was reduced significantly in animals exposed to nitrofen, whereas no significant differences were observed between non-CDH versus CDH groups. Although the left and right ventricular cavity volumes were both reduced significantly in nitrofen-treated rats, they were not changed significantly by the existence of CDH, and the calculated CVindex was similar in the 3 groups. Estimated septal and LV masses were reduced markedly in the nitrofen-treated animals and further reduced by the presence of CDH. However, when LV mass was normalized (LV mass index) the difference became restricted to the animals exposed to nitrofen but was not influenced by the presence of CDH. Finally, the aorta-to-pulmonary artery ratio was similar in all studied groups. CONCLUSIONS: The results of the current study suggest that, although nitrofen had been responsible by global heart hypoplasia, the presence of CDH was not associated with significant underdevelopment of the heart or of the LV in rat fetuses without CMD. Based on these results, we think that the evidence for prenatal counseling based on heart-related indices should be critically reconsidered.

Anticipating twin-twin transfusion syndrome in monochorionic twin pregnancy. Is there a role for nuchal translucency and ductus venosus blood flow evaluation at 11-14 weeks?

Twin-twin transfusion syndrome is a major complication of monochorionic twin pregnancies. In foetuses from monochorionic twinning the presence of increased nuchal translucency thickness (NT) has been associated with an increased risk of developing this syndrome. One of the presumed mechanisms of increased NT is early cardiac failure, indirectly indicated by abnormal blood flow in the ductus venosus. We present eleven cases of monochorionic twin pregnancies in which nuchal translucency thickness and ductus venosus blood flow evaluation was performed at 11-14 weeks. In the two cases presenting with nuchal translucency discrepancy between the two foetuses along with anomalous ductus venosus blood flow in the foetus with increased nuchal translucency, twin-twin transfusion syndrome (TTTS) eventually developed. In none of the twins displaying no inter-twin difference in NT measurements and in those with discrepant NT but normal flow in both ductus venosus, was the progression to TTTS observed. In the two cases which developed TTTS, foetoscopic laser coagulation of the vascular anastomosis was successfully carried out at 18 weeks and normalisation of the venous return was registered. These findings suggest that the association of increased NT and abnormal flow in the ductus venosus in monochorionic twins may be an early manifestation of haemodynamic imbalance between the donor and the recipient eventually manifested as twin-twin transfusion syndrome. Further studies, however, are necessary to establish the potential role of the combination of NT and ductus venosus blood flow assessment as a screening method for TTTS.

Haemodynamic evaluation of the first trimester fetus with special emphasis on venous return.

Knowledge of the fetal circulation is a prerequisite for understanding the physiological behaviour of the developing fetus. In this overview dealing with Colour and Power Doppler ultrasound findings in the first trimester of pregnancy and its pathophysiological background, we aim to report on the methodological aspects, normal blood flow waveform patterns, normal reference values for haemodynamic parameters and potential clinical applications for both arterial and venous flow information (umbilical artery, descending aorta, middle cerebral artery, umbilical vein, inferior vena cava, ductus venosus) and atrioventricular valves. Particular emphasis is devoted to the venous return to the heart. Alterations in venous waveforms, particularly in the ductus venosus, are correlated with the pathophysiology of some fetal diseases and are suggested as a promising tool for the screening of cardiac impairment and as an alternative method for fetal biophysical surveillance.

Prenatal diagnosis of congenital heart disease. Critical evaluation of a twelve-month experience.

INTRODUCTION: A retrospective study of fetal echocardiographies performed in the Ultrasound Unit of the Department of Obstetrics and Gynecology between January and December 1998 was carried out. MATERIAL AND METHODS: We performed 271 fetal echocardiographies (236 fetuses; 230 pregnant women). A triplex ultrasound machine was used with a 5 or 3.5 MHz transabdominal probe. RESULTS: The median maternal age was 31 years (17-44) and the median gestational age at the time of the first examination was 27 weeks (15-38). A hundred and thirty-two pregnant women (57.4%) were internal referrals and (98) 42.6% were referred by other hospitals. The most common indications for referral were maternal age > or = 35 years old (n = 48), gestational diabetes (n = 38), family history of congenital heart disease (n = 24) and insulin-dependent diabetes (n = 23). Accuracy of 32 echocardiographies was limited by technical difficulties. Out of 236 fetuses, 205 (86.9%) presented a normal scan, whereas 31 (13.1%) showed some abnormality: 22 structural cardiac defects; 5 rhythm anomalies and one with a mixed abnormality. In three cases a pericardial effusion was detected in the absence of any structural or rhythm anomaly and regressed spontaneously after birth. Three fetuses were referred prenatally for treatment to another center. Eight out of fifteen patients followed in the Pediatric Cardiology outpatient clinic were submitted to surgical treatment. In four cases the parents opted for termination of pregnancy and two neonatal deaths occurred. Four patients were lost for follow-up. DISCUSSION: Our study suggests that strict criteria for a referral of pregnant women to specialized fetal echocardiography are still missing. The median gestational age of 27 weeks at the time of the first examination was clearly out of the generally accepted recommended period (18-24 weeks). It is time to improve the collaboration between the different health professionals involved in the care of pregnant women in order to increase the sensitivity of screening for congenital heart disease.

[Aortic valvular stenosis: from infant to adult--Non-invasive evaluation and decision making through life]. / Estenose valvular aórtica: da criança ao adulto avaliação não-invasiva e estratégia de decisão ao longo da vida.

Non-invasive diagnosis and management of congenital aortic valvular stenosis has been possible with more accuracy by using echocardiography and Doppler. Adequate diagnosis, even in fetal life, has been performed more easily. Moreover, the choice of surgical procedure or balloon valvuloplasty has been made by using Doppler echocardiography. Echo Doppler examination is also an excellent method for following the natural course of the disease. Other non-invasive procedures, such as electrocardiogram, exercise testing, X-ray and magnetic resonance, are useful, but also have some limitations.