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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Ebstein’s anomaly is a rare complex congenital heart defect of the tricuspid valve. We aimed to 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. A retrospective cross-sectional study was conducted over a period of 37 months. We analyzed all patients diagnosed with congenital heart defects. Of the patients studied, ano,alia 0.

Mean cardiothoracic index was 0. All patients were operated: The mean follow-up time was 1. Mean cardiothoracic index decreased to 0. Three patients had mild and two had moderate tricuspid regurgitation.

The patient who underwent cone reconstruction and a Glenn procedure was in functional class I. The frequency of Ebstein’s anomaly was similar to that in other centers.

Cone reconstruction was viable in the majority of patients, with good early and short-term results. Foram analisados ecocardiogramas sequenciais. Data on clinical presentation, chest X-ray, electrocardiography, echocardiographic studies and surgical reports were analyzed. Cavopulmonary shunt was reserved for patients less than six months old and for those with significant RV dysfunction.

Follow-up was in the first half of Junewhen all patients underwent clinical, radiological and echocardiography assessment. A descriptive statistical ce was performed absolute and relative frequencies, mean and standard deviation. During the study period, patients under the age of 19 years with a diagnosis of CHD were analyzed, eight anoma,ia.

Demographic data, clinical presentations, and NYHA functional class of the study population are shown in Table 1. Female gender was predominant, with a female: Regarding clinical presentation, five patients Data on X-ray, echocardiography, indications for surgery, type of surgical techniques and complications are presented in Table Distribution of demographic data, year of diagnosis, clinical presentation and NYHA functional class.

Anomalía de Ebstein en niños

New York Heart Association. Cardiothoracic index, echocardiographic findings, surgical indications, surgery performed and early post-operative evaluation. Mean CTI was 0. In one of these cases an extremely rare association was found ostium primum ASD, pulmonary atresia and patent ductus arteriosus [PDA].

No patients with a genetic syndrome or WPW syndrome were anomaoia. All patients underwent surgical intervention. The indications for surgery were: In these patients, the mean bypass time was Mean hospital stay was At discharge, in this group two patients had ebtsein TR and three had mild TR. The mean follow-up was 1. In this period, another patient who underwent an MBT shunt died CTI decreased in all patients, by a mean of 0.

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The third ebstien showed the greatest improvement Figure 3. The patient with a permanent pacemaker was readmitted five months after surgery for pericardial effusion and minimal right pleural effusion and was discharged in NYHA class II seven days after readmission. Evolution of functional class in the five patients undergoing cone reconstruction.

Evolution of cardiothoracic index in the five patients undergoing cone reconstruction. Chest X-rays showing an impressive reduction in cardiothoracic index after cone reconstruction. This study revealed that 0.

Anomalía de Ebstein en niños | HCA Healthcare

This result contrasts with those of Anderson 9 and Da Silva, 15 who report a slight predominance of males, and with other studies that described no predominance of either gender. 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. 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.

Transthoracic echocardiography is the most important diagnostic test for EA, to determine TV morphology and physiology and to assess the cardiac chambers and the presence of other CHD.

On the other hand, Arizmendi et al. 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.

Finally, it is noteworthy that one patient in our series had an extremely rare association ostium primum ASD, pulmonary atresia and PDA. The complexity of the anatomical and functional changes in EA and the possibility of association with other CHDs mean that the surgical approach should be individualized. Cavopulmonary shunt is reserved for patients with severe RV dysfunction. Various techniques for tricuspid valve reconstruction have been aomalia in the last 35 anpmalia, with different results.

Several techniques have been described to avoid this complication. The low in-hospital mortality and long-term clinical improvement reported by da Silva et al. However, the technique could not be applied in two patients for the reasons described above.

The prevalence of EA found in this series was similar to other centers. Our patients had access to medical care at a ed stage of the disease. CR was performed in the majority of patients with excellent immediate and short-term results. The authors declare that the procedures followed were in accordance with the regulations of the relevant clinical research ethics committee and with those of the Code of Ethics of the World Medical Association Declaration of Helsinki.

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The authors declare that they have followed the protocols of their work center on the publication of patient data. The authors have obtained the written informed consent of the patients or subjects mentioned in the article. The corresponding author is in possession of this document. The authors have no conflicts of interest to declare. Artigo anterior Artigo seguinte.

Ebstein’s anomaly in children: A single-center study in Angola. Valdano Manuel a. Under a Creative Commons aonmalia. Mostrar mais Mostrar menos. Introduction and Objective Ebstein’s anomaly is a rare complex congenital heart defect of the tricuspid valve.

We aimed to 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. Methods A retrospective cross-sectional study was conducted over a period of 37 months. We analyzed all patients diagnosed with congenital heart defects. Results Of the patients studied, eight 0.

The patient who underwent cone reconstruction and a Glenn procedure was in functional class I.

Ebstein’s Anomaly | Stanford Health Care

Conclusion The frequency of Ebstein’s anomaly was similar to that in other centers. Cone reconstruction was viable in the majority of patients, with good early and short-term results.

Resultados Foram analisados ecocardiogramas sequenciais. New York Heart Association. Follow-up was in the first half of Junewhen all patients underwent clinical, radiological and echocardiography assessment. A descriptive statistical analysis was performed absolute and relative frequencies, mean and standard deviation.

Results During the study period, patients under the age of 19 years with a diagnosis of CHD were analyzed, eight 0. Data on X-ray, echocardiography, indications for surgery, type of surgical techniques and complications are presented in Table 2. Distribution of demographic data, year of diagnosis, clinical presentation and NYHA functional class. Cardiothoracic index, echocardiographic findings, surgical indications, surgery performed and early post-operative evaluation. Evolution of cardiothoracic index in the five patients undergoing cone reconstruction.

Chest X-rays showing an impressive reduction in cardiothoracic index after cone reconstruction. On a very rare case of insufficiency of the tricuspid valve caused by a severe congenital malformation of the same. Ebstein’s malformation of the tricuspid valve: Teratology, 50pp.

The clinical profile of Ebstein’s malformation as seen from the fetus to the adult in 52 patients. Cardiol Young, 14pp. Embryology and pathologic features of Ebstein’s anomaly.

Prog Pediatr Cardiol, 2pp. Familial Ebstein anomaly, left ventricular hypertrabeculation, and ventricular septal defect associated with a MYH7 mutation.

Am J Med Genet A, 12pp. Two-dimensional echocardiographic spectrum of Ebstein’s anomaly: J Am Coll Cardiol, 3pp.