Anomalía de Ebstein: a propósito de un caso / Ebstein anomaly: about a case

La anomalía de Ebstein es una cardiopatía congénita rara y poco frecuente caracterizada por el adosamiento de los velos valvulares tricuspídeos; en la etapa prenatal se estima que su incidencia corresponde a un 3% de todas las cardiopatías diagnosticadas. Se presenta el caso de un feto con diagnóstico de anomalía de Ebstein a quien se le realizó un diagnóstico prenatal adecuado, lo que permitió planificar el nacimiento neonatal con un equipo multidisciplinario integral. Debido a la rareza del diagnóstico prenatal de esta entidad, se describe el caso clínico y los hallazgos imagenológicos representativos.

Ebsteins anomaly is a rare and infrequent congenital heart disease characterized by the attachment of the tricuspid valve leaflets; in the prenatal stage it is estimated that its incidence corresponds to 3% of all diagnosed heart diseases. We present the case of a fetus diagnosed with Ebsteins anomaly who underwent an adequate prenatal diagnosis, which made it possible to plan the neonatal birth with a comprehensive multidisciplinary team. Due to the rarity of the prenatal diagnosis of this entity, the clinical case and the representative imaging findings are described.

Analysis of Surgical Treatment of 20 Cases of Ebstein's Anomaly Combined with Atrial Septal Defect / 昆明医科大学学报

Objective To analyze the treatment strategy of the atrial septal defect in the surgical treatment of Ebstein's anomaly combined with the atrial septal defect and the short-term follow-up results of the treatment of Ebstein's anomaly.Methods A retrospective analysis of the clinical data and follow-up results of 20 patients with Ebstein's anomaly and atrial septal defect was conducted from September 2017 to February 2021.And the statistical analysis on the preoperative and postoperative echocardiography results of this group of patients was performed.Results Sixteen patients underwent the biventricular correction surgery,among whom two cases underwent the horizontal atrial tricuspid valvuloplasty(Danielsons procedure),four cases underwent the vertical atrial tricuspid valvuloplasty(Carpentier procedure),and ten cases underwent the conical reconstruction.Two patients were given a half ventricular correction surgery(tricuspid valve reconstruction combined with bidirectional Glenn surgery)and two patients underwent the bidirectional Glenn surgery.The combined atrial septal defects were closed in one stage during extracorporeal circulation for correction of deformitie in 20 patients.At 1,3,6,and 12 months after the surgery,the patient's right ventricular size significantly decreased compared to preoperative(P<0.05),and cardiac function(left ventricular ejection fraction)significantly improved(P<0.05).Conclusion The surgical treatment of Ebstein's anomaly combined with the atrial septal defect should follow the principle of individualized treatment.Biventricular correction is still the first choice for the treatment of Ebstein's anomaly,and the atrial septal defect should be closed at one stage,so as to obtain a good therapeutic effect.

Right heart function parameters in patients with Ebstein anomaly:Correlations of evaluation of echocardiography and MRI / 中国医学影像技术

Objective To explore the correlations of evaluations of right heart function parameters in patients with Ebstein anomaly(EA)using echocardiography and cardiac MRI.Methods Data of transthoracic echocardiography and cardiac MRI in 32 patients with EA confirmed by operation were retrospectively analyzed.The correlations of cardiac cavity size,right ventricular function and strain parameters obtained using echocardiography and the functional right ventricular(fRV)ejection fraction(EF)measured using MRI were explored.Results MRI fRV-EF in 32 cases of EA was(23.20± 7.61)％.Among echocardiographic parameters in 32 cases of EA,fractional area change(FAC)of fRV(r=0.347,P=0.015)was slightly,while global longitudinal strain(GLS)of fRV(r=0.801,P＜0.001)was highly positively correlated with MRI fRV-EF,respectively,whereas atrialized right ventricle(aRV)area/fRV area(r=-0.730,P=0.007)was highly negatively,aRV area/left ventricular area(r=-0.450,P=0.042)and right ventricular anterior-posterior diameter(r=-0.650,P=0.022)were both moderately negatively correlated with MRI fRV-EF.Both the left ventricular eccentricity index(r=-0.347,P=0.049)and Glasgow outcome scale extended(r=-0.336,P=0.024)obtained with echocardiography were slightly negatively correlated MRI fRV-EF.Conclusion Right heart function parameters in EA patients obtained with echocardiography were correlated with those of MRI fRV-GLS,among which aRV area/fRV area were highly positively correlated with MRI fRV-EF,hence having great value for evaluating right heart function in EA patients.

Morphologic and functional changes of ventricles between pre- and post- cone reconstruction surgery in children with Ebstein’s anomaly / 中华胸心血管外科杂志

Objective:To understand the morphologic and functional changes of ventricles between pre- and post- cone reconstruction(CR) surgery in children with Ebstein’s anomaly(EA).Methods:The clinical data of children with EA who underwent CR and cardiac magnetic resonance(CMR) in Shanghai Children’s Medical Center between July 2011 to April 2019 were collected and analyzed. Ventricular functions were assessed with the use of ejection fraction(EF), stroke volume index(SVI), cardiac output(CO), and cardiac index(CI). Ventricular morphologies were assessed with the use of end-diastolic ventricular volume(EDV), end-diastolic ventricular volume index(EDVI) and ventricular cine images. Paired student t tests and Wilcoxon rank sum tests were used for statistical analysis. Results:There were a total of 32 children with EA who underwent CR and CMR, with 13 males and 19 females, a median operative age of 2.9 years old(0.6-15.5 years old), and a mean follow-up time of(4.4±1.9) years. Seven patients had both preoperative and postoperative CMR, with a mean follow-up time of(3.3±1.4) years; Eleven patients had two or more postoperative CMR, with a mean interval time of(1.9±1.0) years. After the surgery, the median tricuspid-regurgitation grade decreased from 3 to 2, and the median New York Heart Association functional class improved from 2.5 to 1, the left ventricle(LV)-SVI, LV-EDV and LV-EDVI increased from 29.8 ml/m 2 to 43.2 ml/m 2( P=0.039), from 56.4 ml to 86.9 ml( P=0.004), from 50.5 ml/m 2 to 68.4 ml/m 2( P=0.022), respectively. And the long-term LV-EDV increased from 56.6 ml to 74.7 ml( P=0.002) when compared to that of early postoperative. There was no significant differences in right ventricle(RV)-EF, RV-SVI, RV-CO, RV-CI, RV-EDV and RV-EDVI between pre- and post- CR( P>0.05); but the long-term postoperative RV-CO and RV-EDV increased from 3.1 L/min to 4.1 L/min( P=0.008), from 67.5 ml to 96.5 ml( P<0.001), respectively, when compared with those of early postoperative. Conclusion:CR improves the function and morphology of both ventricles in children with EA. And although postoperative ventricles grow well, RV dysfunction persists.

Analysis of the surgical effect of one and a half ventricle repair in the Ebstein anomaly / 中华胸心血管外科杂志

Objective:To summarize the effect of one and a half ventricle repair in the treatment of Ebstein’s Anomaly.Methods:The data of 149 patients diagnosed with Ebstein’s Anomaly and received with surgical treatment in Pediatric Cardiac Surgery Department of Beijing Anzhen Hospital affiliated to Capital Medical University from January 2010 to December 2018 were continuously collected, and the survival rate, reoperation rate and tricuspid regurgitation were followed up in the medium-long term.Results:There were 68 males and 81 females, with a median age of 5.58 years. Patients were divided into Biventricular repair group and one and a half ventricle repair group. The operative age was significantly younger in the one and a half ventricle repair group (4.15 years vs. 6.71 years, P=0.019). There were also significant differences in patiens’ body length[(107.70±31.28)cm vs. (123.20±35.22)cm, P=0.014]and body weight[(19.69±12.22)kg vs. (29.65±20.41)kg, P=0.001], between the two groups, which may be related to the severity of the disease and the need for early surgical intervention. However, there was no significant difference between the two groups in preoperative cyanosis, hemoglobin level, cardiac function, arrhythmia, and common cardiac malformations such as atrial septal defect. Notably, the proportion of preoperatively complicated pulmonary stenosis (10.81% vs. 1.79%, P=0.016) and right ventricular dysplasia (16.22% vs. 3.57%, P=0.008) was significantly higher in the one and a half ventricle repair group. In intraoperative and postoperative indicators, as one and a half ventricle repair under the collateral circulation, extracorporeal circulation time was slightly longer [(125.51±37.35)min vs. (100.44±25.24)min, P<0.001], and other indicators such as aortic cross-clamp time, endotracheal intubation time, length of hospital stay, and mid-term follow-up results, including mid-term mortality, reoperation rate, cardiac function and valvular regurgitation, there was no significant difference between the two groups. However, the proportion of hospital mortality in half ventricular therapy group was slightly higher, which may be related to the poor right heart function and postoperative recovery difficulties. Conclusion:Good follow-up results have been achieved in the treatment of two surgical therapy. Patients with right ventricular dysplasia and pulmonary artery stenosis should be paid more attention to. Pulmonary artery pressure and pulmonary vascular development should be evaluated before surgery to make preparations for one and a half ventricle repair.

Atypical pompholyx presentation of secondary Staphyloccoccus and Klebsiella infections in a patient with premorbid Ebstein anomaly: A case report

Rationale: Pompholyx refers to pruritic vesicles or bullous rash that mainly distribute on the palms and lateral surfaces of the fingers. It is less common among Asians, and in a severe condition, secondary bacterial infection of pompholyx can happen and result in pain, swelling and pustules. Patient concerns: A 15-year-old girl complained of progressive wound and small bumps containing yellowish pus and crusts on her hands and feet for over 6 months and worsened in the last month before admission. She also had Ebstein anomaly. Diagnosis: Atypical pompholyx with secondary Staphylococcus and Klebsiella infections. Interventions: Wound care with wet dressing and applying moisturizer on crusts, application of antibiotics for Gram positive and negative bacteria and giving nutritional support with reckoning of proper calories. Outcomes: Skin lesions were completely healed and the patient was discharged after 10 days of hospitalization. Lessons: Atypical manifestation of pompholyx makes it hard to diagnose. The diagnosis can be confirmed with meticulous history-taking and physical examination. Wound caring and controlling of the infection should be done to earn an optimal outcome.

Anomalía de Ebstein en recién nacido / Ebstein's anomaly in newborn

Introducción: La anomalía de Ebstein es una rara malformación con presentación sintomática respiratoria leve a grave a causa de defectos cardiacos congénitos manifestados durante la niñez. Objetivo: Exponer el caso en el cual el diagnóstico oportuno de esta enfermedad permitió el tratamiento integral del paciente. Presentación del caso: Recién nacido a término, quien a las siete horas de su nacimiento presentó saturación de oxígeno disminuida, examen físico sin cianosis, soplo pansistólico en la auscultación y cardiomegalia detectada por rayos X de tórax. Se realizó ecocardiograma que permitió diagnosticar al bebé como portador de la enfermedad de Ebstein con manifestaciones leves. Una vez que tiene mejoría clínica, se le proporcionó el alta médica con indicación de valoración por cardiología pediátrica. Conclusiones: La anomalía de Ebstein puede comenzar en el período neonatal con sintomatología de severidad variable. La sospecha clínica permite la confirmación diagnóstica y el seguimiento estrecho, sobre todo en casos severos(AU)

Introduction: Ebstein's anomaly is a rare malformation with mild to severe respiratory symptomatic presentation due to congenital heart defects manifested during childhood. Objective: To present the case in which the timely diagnosis of this disease allowed the comprehensive treatment of the patient. Case presentation: Full-term newborn, who at seven hours of birth presented decreased oxygen saturation, physical examination without cyanosis, pansystolic murmur in auscultation and cardiomegaly detected by chest X-rays. An echocardiogram was performed to diagnose the baby as a carrier of Ebstein's disease with mild manifestations. Once he had clinical improvement, he was discharged with an indication for assessment by pediatric cardiology. Conclusions: Ebstein's anomaly may begin in the neonatal period with symptoms of variable severity. Clinical suspicion allows for diagnostic confirmation and close follow-up, especially in severe cases(AU)

Usefulness of VExUS in a pediatric patient with Ebstein's anomaly / Utilidad de VExUS en paciente pediátrico portador de anomalía de Ebstein

The venous excess ultrasound grading system (VExUS) is a relatively new application of point of care ultrasound. It has been successfully used to monitor for fluid overload in adult post-operative cardiac surgery patients. It has not been described in the management of congenital cardiac disease. We present a novel use of VExUS to diagnose decompensated Ebstein's anomaly in a pediatric patient. A 13 year-old female with known stable Ebstein's anomaly presented with new onset fluid overload and oliguria. Using abdominal venous Doppler ultrasound and VExUS, we were able to quickly differentiate the cause of the patient's fluid overload as Ebstein's anomaly instead of a primary renal etiology. This is the first reported use of VExUS to diagnose acute fluid overload in a pediatric patient with Ebstein's anomaly. VExUS should be considered as a supplemental tool to diagnose fluid overload in other congenital cardiac diseases.

El sistema de gradiente por ultrasonidos de exceso venoso (VExUS), es una aplicación relativamente nueva de los ultrasonidos en el punto de cuidado. Esto ha sido utilizado exitosamente para monitorizar la sobrecarga de fluidos en pacientes adultos en el período posoperatorio de cirugía cardíaca. No ha sido descripto en el manejo de las cardiopatías congénitas. Nosotros presentamos un novedoso uso de VExUS para diagnosticar una anomalía de Ebstein descompensada en un paciente pediátrico. Paciente femenina de 13 años de edad con una anomalía de Ebstein compensada en su evolución, se presenta con sobrecarga de fluidos y oliguria. Usando ultrasonidos Doppler de vasos abdominales (VExUS), fue posible diferenciar rápidamente la causa de la sobrecarga de fluidos en una anomalía de Ebstein que provoca insuficiencia renal. Este el primer caso reportado del uso del VExUS para el diagnóstico de sobrecarga de fluidos en pacientes pediátricos con anomalía de Ebstein. El score VExUS debería ser considerado como método complementario en el diagnóstico de la sobrecarga de fluidos en otras cardiopatías congénitas.

Neonatal Ebstein's anomaly with type B Wolff-Parkinson-White syndrome: a case report / 中华围产医学杂志

This paper reported a male newborn with Ebstein's anomaly complicated by type B Wolff-Parkinson-White syndrome. The baby was delivered at 39 weeks of gestation and diagnosed as Ebstein's anomaly complicated by type B Wolff-Parkinson-White syndrome 2 d after birth. He was improved and discharged after conservative management in the Department of Internal Medicine. No abnormality was found by fetal echocardiography at 20 weeks of gestation, while supraventricular tachycardia was diagnosed by M-mode ultrasonography at 38 weeks of gestation due to rapid fetal heart rate. In addition to hemodynamic changes,the abnormal cardiac structure in patients with Ebstein's anomaly may also lead to tachyarrhythmia. Therefore, in fetuses diagnosed with supraventricular tachycardia by prenatal ultrasound or children with type B Wolff-Parkinson-White syndrome detected by electrocardiogram after birth, tricuspid valve should be carefully scanned under echocardiography to avoid the missed diagnosis of Ebstein's anomaly.

Reoperative strategy and mid- to long-term surgical outcomes of Ebstein anomaly / 中国胸心血管外科临床杂志

@#Objective    To analyze the Ebstein anomaly's reoperative strategy and mid- to long-term results. Methods    We retrospectively reviewed the clinical data of 23 patients who diagnosed with Ebstein anomaly and underwent reoperation for tricuspid valve insufficiency between July 2002 and July 2017 in Fuwai Hospital. There were 9 (39.1%) males and 14 (60.9%) females, with a median age of 28.0 (19.0, 45.0) years. Results    Among the 23 patients, 8 (34.8%) underwent tricuspid valvuloplasty and 15 (65.2%) underwent tricuspid valve replacement. The rate of valvuloplasty was 16.7% before 2012, and 54.5% after 2012 (P=0.089) as Cone reconstruction procedure was used. In the valvuloplasty cohort, 3 (37.5%) patients were treated with Danielson or Carpentier technique, and 5 (62.5%) patients were treated with Cone reconstruction procedure. There was no operation-related death. Early complications occurred in 3 (37.5%) patients. The median follow-up was 6.9 years (range, 3.0-15.1 years), and no adverse cardiac events occurred. In the patients with valve replacement, 7 (46.7%) received mechanical prosthesis and 8 (53.3%) received bio-prosthesis. There was no operation-related death. And early complications were observed in 3 (20.0%) patients. The median follow-up was 6.5 years (range, 2.5-15.3 years). One (6.3%) patient died and 4 (26.7%) had long-term complications during the follow-up period. Conclusion    The mid- to long-term outcomes are convincing in patients who undergo the second operation due to recurrent tricuspid regurgitation of Ebstein anomaly. A low incidence of reoperation is observed. Cone reconstruction procedure provides possibilities of second tricuspid valvuloplasty, and this technique can reduce the rate of tricuspid valve replacement in the second operation. Tricuspid valve replacement is still an alternative method for the treatment of recurrent tricuspid regurgitation in patients with Ebstein anomaly. The bioprosthetic prosthesis may be a better choice than mechanical prosthesis.

Clinical analysis of outcomes of different surgery methods for Ebstein anomaly in a single center / 中国胸心血管外科临床杂志

@#Objective    To investigate the indications and clinical effects of tricuspid cone reconstruction and tricuspid valve replacement in the treatment of downward displacement of tricuspid valve (Ebstein anomaly). Methods    The clinical data of 22 patients with Ebstein anomaly who underwent surgical treatment in our hospital from January 2013 to March 2020 were collected. There were 7 males and 15 females, aged 4-56 (33.68±17.78) years. The patients were divided into two groups according to different surgical methods: a tricuspid cone reconstruction group (tricuspid valvuloplasty group, n=12) and a tricuspid valve replacement group (n=10). The curative effect of the two operative methods were compared. Results    Twenty-two patients underwent surgical treatment under general anesthesia and cardiopulmonary bypass. One patient died of severe low cardiac output syndrome during operation. Downward displacement of tricuspid septum was found in 22 patients, and downward displacement of tricuspid septum and posterior septum in 21 patients, downward displacement of tricuspid in 8 patients. Twelve patients were treated with tricuspid  valvuloplasty, 10 patients with tricuspid valve replacement, and patients with other intracardiac structural malformations were treated at the same time. Postoperative cardiac doppler ultrasound indicated no tricuspid regurgitation in 9 patients, mild regurgitation in 8 patients, and moderate regurgitation in 4 patients. No perivalvular leakage occurred in all patients undergoing valve replacement. Four patients developed degree Ⅲ atrioventricular block after operation, among whom 3 patients recovered to sinus rhythm and 1 patient implanted the permanent pacemaker. Twenty-one patients were cured and discharged after successful operation, and were followed up for 3 to 78 months, with a follow-up rate of 100.0%. During the follow-up period, there was no severe tricuspid regurgitation in tricuspid valvuloplasty group, but mild or moderate regurgitation was found. After tricuspid valve replacement, only 1 patient had mild regurgitation, and the rest patients had no valve regurgitation. Conclusion    Tricuspid valvuloplasty and tricuspid valve replacement are effective in the treatment of Ebstein anomaly, and the tricuspid regurgitation is less severe after tricuspid valve replacement operation. The operation method should be selected according to the different anatomical characteristics and condition of tricuspid valve. The combined intracardiac malformation or arrhythmia can be dealt with simultaneously.

Rare diagnostics of Ebstein anomaly in an adult patient in Northern Rio de Janeiro / Diagnóstico raro da anomalia de Ebstein no norte do Rio de Janeiro em um paciente adulto

Ebstein anomaly is the fourth most frequent cyanotic heart disease in the neonatal period, and its clinical course is slow compared to other diseases, which in turn results in late diagnosis of this condition. We present a case of a 37-year-old man who complained of palpitation for about 5 years, twice a week, at rest, with prolonged duration and low systemic output. Auscultation revealed irregular heart rhythm characterized by extrasystoles with a tricuspid systolic murmur grade 5+/6 and worsening on inspiration. Clinical spectrum is variable and depends on the severity of defects and associated anatomical changes. In patients who have reached adulthood, symptoms usually present as progressive cyanosis, exercise intolerance, right heart failure, and arrhythmias. In Brazil, identification of this congenital disease usually occurs in a more adult phase, affecting the prognosis and survival of patients. (AU)

Anomalia de Ebstein é a quarta cardiopatia cianótica com maior frequência no período neonatal, cuja evolução clínica é lenta em comparação com outros tipos de doenças, o que, por sua vez, resulta no diagnóstico tardio. Apresentamos o caso de um homem de 37 anos, com queixa de palpitações por cerca de cinco anos, duas vezes por semana, em repouso, com duração prolongada e com baixo débito sistêmico. A ausculta revelou ritmo cardíaco irregular por extra-sístoles com sopro sistólico na tricúspide, 5 + / 6 piora na inspiração. O espectro clínico é variável e ocorre de acordo com a gravidade dos defeitos e alterações anatômicas associadas. Pacientes que atingiram a idade adulta, os sintomas geralmente se apresentam como cianose progressiva, intolerância ao exercício, insuficiência cardíaca direita e arritmias. A realidade brasileira é que a identificação dessa doença congênita persiste em uma fase adulta, afetando o prognóstico e a sobrevida desses pacientes. (AU)

Válvula tricúspide displásica (Ebstein-like) / Tricuspid valve dysplasia (Ebstein-like)

Resumen La anomalía de Ebstein es una malformación caracterizada por el desplazamiento apical de las inserciones de las valvas septal y posterior de la válvula tricúspide, así como del anillo funcional tricúspide y por la atrialización del ventrículo derecho. Se han encontrado casos durante cirugías que no han cumplido con estos criterios, y que, por tanto, se han clasificado como "anomalías Ebstein-like". Algunos de ellos han sido descritos anatómicamente como válvulas displásicas. Se presenta el caso de una mujer de 20 años que presentó disnea clase III de la Asociación del Corazón de Nueva York y dolor punzante en el pecho, que se clasificó como una posible anomalía de Ebstein por ecocardiografía; sin embargo, los hallazgos intraoperatorios fueron compatibles con una válvula tricúspide displásica con dilatación del anillo funcional. Este caso destaca la importancia del uso adecuado de la ecocardiografía, así como la necesidad de estudios prospectivos sobre los casos de Ebstein-like.

Abstract The Ebstein anomaly is a malformation characterised by the apical displacement of the insertions of the septal valve and the posterior tricuspid valve, displaced tricuspid functional ring, and atrialisation of the right ventricle. Cases have been found during surgery that have complied with these criteria, and have therefore been classified as "Ebstein-like anomalies". Some of them have been described anatomically as valve dysplasia. The case is presented of a 20 year-old female that presented with dyspnoea Class III of the New York Heart Association, and a stabbing pain in the chest. This was classified as a possible Ebstein anomaly by cardiac ultrasound. However the findings during the surgery were compatible with tricuspid valve dysplasia with dilation of the functional ring. This case highlights the importance of the appropriate use of cardiac ultrasound, as well as the need to carry out prospective studies on Ebstein-like cases.

Caso clínico: adulto con anomalía de Ebstein / Clinical case: adult with Ebstein anomaly

Introducción: la anomalía de Ebstein se caracteriza por la implantación baja de las valvas septal y posterior de la tricúspide; por lo tanto, la aurícula derecha es amplia y su ventrículo es pequeño. Al ser una patología bastante rara en nuestro medio se encuentra subdiagnosticada y no recibe el tratamiento oportuno. Por ende, la importancia de conocerla para no retrasar su manejo clínico quirúrgico.Caso clínico: paciente de 46 años de edad, masculino, con antecedente de hipoacusia congénita, presentó disnea (clase funcional III) más palpitaciones y dolor torácico atípico hace 1 año aproximadamente. Al examen físico evidenció soplo holosistólico en foco tricuspídeo y uso moderado de musculatura accesoria. Al ecocardiograma transesofágico, se observó arritmia auricular más implante bajo de las valvas de la tricúspide y se inició tratamiento clínico más ablación del flutter. En la evolución presentó mejoría de la sintomatología y dependiendo del deterioro de la clase funcional, tratamiento quirúrgico Conclusiones: en el presente caso se ajustó tratamiento clínico y ablación del flutter de acuerdo a sintomatología del paciente, mostrando mejoría (AU);

Introduction: Ebstein's anomaly is characterized by low implantation of the septal and posterior tricuspid leaflets; therefore, the right atrium is wide and its ventricle is small. Being a rather rare pathology in our environment, it is underdiagnosed and does not receive the appropriate treatment. Therefore, it is important to know it to avoid a delay in its clinical surgical management.Clinical case: The patient is 46-year-old male, with a history of congenital hearing loss, who presented dyspnea (functional class III) plus palpitations and atypical chest pain approximately 1 year ago. In the physical examination, he revealed a holosystolic murmur in the tricuspid focus and moderate use of accessory muscles. A transesophageal echocardiogram revealed atrial arrhythmia plus implantation of the tricuspid leaflets and it was started a clinical treatment plus flutter ablation. In the evolution he presented improvement of the symptoms and depending on the deterioration of the functional class, surgical treatment.Conclusions: in this case, clinical treatment and flutter ablation were adjusted according to the patient's symptoms, showing improvement (AU);

Consideraciones útiles para el asesoramiento genético de pacientes con anomalía de Ebstein / Useful considerations for the genetic advice of patients with Ebstein anomaly

La anomalía de Ebstein es una rara enfermedad que consiste en un espectro variable de anomalías, resultado de una implantación anormal de la válvula tricúspide en el ventrículo derecho; por tal razón, es considerada una afección grave e incurable que origina una tendencia a la terminación voluntaria del embarazo como opción reproductiva en el marco del asesoramiento genético prenatal. Los adelantos en el diagnóstico y control clinicoquirúrgico de esta enfermedad han garantizado el incremento de la supervivencia y una mejor calidad de vida en estos pacientes. Además, han permitido ajustar factores clínicos que implican su evolución y pronóstico. Se realizan algunas valoraciones, a fin de proveer los argumentos necesarios que permitan a la pareja elegir un curso de acción apropiado con vistas a los riesgos y objetivos familiares basados en los principios éticos del asesoramiento genético.

Ebstein anomaly is a strange disease that consists on a variable spectrum of anomalies, due to an abnormal installation of the tricuspid valve in the right ventricle; reason why, it is considered a serious and incurable disorder that originates a tendency to the voluntary termination of pregnancy as reproductive option in the mark of prenatal genetic advice. The advances in the diagnosis and clinical surgical control of this disease have guaranteed the increment of survival and a better life quality in these patients. Also, they have allowed to adjust clinical factors that involve its clinical course and prognosis. Some valuations are carried out, in order to provide the necessary arguments that allow the couple to choose an appropriate action course aimed at the risks and family objectives based on the ethical principles of the genetic advice.

Clinical outcome of tricuspid valve replacement in children aged no more than 14 years / 中国胸心血管外科临床杂志

@#Objective    To summarize the clinical characteristics and outcome of tricuspid valve replacement (TVR) in children aged no more than 14 years, and to discuss the selection of prosthesis. Methods    From September 2002 to August 2019, 14 patients aged no more than 14 years who received TVR were included in our study. There were 9 males and 5 females, with a mean age of 9.8±4.3 years. Results    Mechanical prosthesis was implanted in 8 patients, and bioprosthesis in 6 patients. The mean cardiopulmonary time and aortic-clamp time was 170.3±109.8 min and 95.1±63.1 min, respectively. The mortality within 30 days after surgery was 21.4% (3/14), and all 3 patients died of severe low cardiac output syndrome. Eleven patients were followed up for 34-199 (100.1±57.4) months. During the follow-up, mechanical prosthesis dysfunction occurred in 3 patients, 2 of whom received secondary TVR. One patient died during the follow-up. Conclusion    The bioprosthesis is the first choice for TVR in children. Some long-term complications may occur after TVR, and close follow-up and timely intervention are needed.

Early and long-term outcomes of surgical treatment of ebstein's anomaly

Abstract Objective: This study aimed to evaluate Ebstein's anomaly surgical correction and its early and long-term outcomes. Methods: A retrospective analysis of 62 consecutive patients who underwent surgical repair of Ebstein's anomaly in our institution from January 2000 to July 2016. The following long-term outcomes were evaluated: survival, reoperations, tricuspid regurgitation, and postoperative right ventricular dysfunction. Results: Valve repair was performed in 46 (74.2%) patients - 12 of them using the Da Silva cone reconstruction; tricuspid valve replacement was performed in 11 (17.7%) patients; univentricular palliation in one (1.6%) patient; and the one and a half ventricle repair in four (6.5%) patients. The patients' mean age at the time of surgery was 20.5±14.9 years, and 46.8% of them were male. The mean follow-up time was 8.8±6 years. The 30-day mortality rate was 8.06% and the one and 10-year survival rates were 91.9% both. Eleven (17.7%) of the 62 patients required late reoperation due to tricuspid regurgitation, in an average time of 7.1±4.9 years after the first procedure. Conclusion: In our experience, the long-term results of the surgical treatment of Ebstein's anomaly demonstrate an acceptable survival rate and a low incidence of reinterventions.