A frequência da Anomalia de Ebstein encontrada foi semelhante a de outros Centros. A técnica de Cone foi viável na maior parte dos pacientes. A mortalidade. Ebstein’s anomaly is a malformation of the tricuspid valve. In this defect, there is a downward displacement of the tricuspid valve (located between the upper and. Antecedentes: en la actualidad, con la ecocardiografía fetal, es pos ble confirmar el diagnóstico in utero de la anomalía de Ebstein y determinar, con certeza.
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Cavopulmonary shunt was reserved for patients less than six months old and for those with significant RV dysfunction. Antenatal diagnosis Antenatal diagnosis is now possible with fetal echocardiography that reveals hydrops fetalis. The mean follow-up time was 1.
Mean CTI was 0. Ebstein’s anomaly in a patient with Down’s syndrome. Medical treatment relies on inotropic agents in case of cardiac failure and antiarrhythmic drugs in case of tachyarrhythmia. Ebbstein frequency of Ebstein’s anomaly was similar to that in other centers. J Am Coll Cardiol, 3pp.
Ventricular septal defect in children and adolescents in Congenital stenosis and insufficiency of the tricuspid valve. Mean cardiothoracic index decreased to 0.
Only comments written in English can be processed. Although associations between EA and certain genetic syndromes have been described, mainly trisomy 21 and WPW syndrome, 9,14 these syndromes were not found in the present series. Emmanuilides A, Riemenschneider G.
Cavopulmonary shunt is reserved for patients with severe RV dysfunction. If the valve is too malformed, replacement is the only option. The authors have no conflicts of interest to declare.
What Is Ebstein’s Anomaly? Clinical description Clinical presentation is heterogeneous and depends on the severity of the lesion extent of tethering of the antero-superior leaflet across the normal valvar orificeand the degree of dysfunction of the right ventricle.
We analyzed all patients diagnosed with congenital heart defects. Services on Demand Journal. You can change the settings or obtain more information by clicking here. It is usually associated with an atrial septal defect. Asymptomatic patients, and patients with anomaliaa forms of the disease, have a normal life expectancy.
We aimed ebstien describe the frequency, clinical profile, and early and short-term post-operative results in patients under the age of 18 years operated for this anomaly in a tertiary center in Angola. This result is similar to that reported in a study conducted in Sudan, in which the authors found that half of the patients were symptomatic. Prognosis Asymptomatic patients, and patients with mild forms of the disease, have a normal life expectancy.
We analyzed all patients diagnosed with congenital heart defects. A descriptive statistical analysis was performed absolute and relative frequencies, mean and standard deviation. Our patients had access to medical care at a critical stage of the disease.
Three patients had mild anomqlia two had moderate tricuspid regurgitation. Artigo anterior Artigo seguinte. Follow-up was in the first half of Junewhen all patients underwent clinical, radiological and echocardiography assessment.
Mean hospital stay was The severest forms of Ebstein’s malformation can produce an imperforate tricuspid valve, which must be distinguished from tricuspid atresia see this term. Data on X-ray, echocardiography, indications for surgery, type of surgical techniques and complications are presented in Table 2. Management and treatment Medical treatment relies sbstein inotropic agents in case of cardiac failure and antiarrhythmic drugs in case of tachyarrhythmia.
Methods A retrospective cross-sectional study was conducted over a period of 37 months. J Am Coll Cardiol ; Access to health care services are still a serious problem in our continent, from the prenatal period to old age, which is why we have a small sample without neonates with severe forms of EA and older patients with good functional status. Br Heart J ; Secondary repair of Ebstein’s anomaly with atrioventricular septal defect using the cone technique.
Mosby-Year Book, ; Ebstein’s Anomaly congenital heart defect ebsteins-anomaly Adult Congenital Heart Program How to cite this article. Uber einen sehr seltenen fall von insufficienz der valvula tricuspidalis, bedingt durch eine angeborene hochgradige missbikdung derselben. Natural history of Ebstein’s anomaly of tricuspid valve in childhood and adolescence. The cone reconstruction of the tricuspid valve in Ebstein’s anomaly.
Embryology and pathologic features of Ebstein’s anomaly. Access your health information from any device with MyHealth. The authors have anoma,ia the written informed consent of the patients or subjects mentioned in the article. Patients with severe forms of the disease, particularly those presenting during the fetal period or as neonates, have an increased risk of death due to cardiac failure, including at birth or during physical exercise.
Several techniques have been described to avoid this complication.