Anomalía de Ebstein y preeclampsia, asociación infrecuente: reporte de caso y revisión de la literatura / Ebstein's anomaly and preeclampsia, a rare association: case report and literature review

Resumen Se presenta el caso de una gestante con cardiopatía congénita no estudiada que acudió al servicio de urgencias en la semana 25 + 4 por palpitaciones, con evidencia de hipertensión arterial desde el ingreso. Los estudios diagnósticos revelaron anomalía de Ebstein, con gran compromiso de cavidades derechas y asociado a comunicación interauricular. También se documentó preeclampsia lejos del término, con restricción grave del crecimiento intrauterino. Durante la estancia hospitalaria, y ante negativa de la paciente a finalizar la gestación, desarrolló síntomas de congestión pulmonar por sobrecarga. Una vez falleció el feto in utero y después de inducir el parto, remitieron los síntomas cardiovasculares y se controló la hipertensión.

Abstract We present the case of a pregnant woman with previously undiagnosed congenital heart disease, who presented to the emergency department at week 25 + 4 due to palpitations, with evidence of arterial hypertension from admission. Diagnostic studies revealed Ebsteins anomaly, with great involvement of the right cavities and associated with atrial septal defect. Preeclampsia was also documented far from term, associated with severe intrauterine growth restriction. During the hospital stay and due to the refusal of the patient to end the pregnancy, she developed symptoms of pulmonary congestion due to overload, once the fetus died in utero and after inducing labor, the cardiovascular symptoms remitted and hypertension was controlled.

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.

Use of dopamine infusion improved oxygenation in a patient of Ebstein's anomaly with atrial septal defect.

We present the successful perioperative management of an adult patient with Ebstein's anomaly for abdominal rectopexy surgery. The patient developed mild hypotension and a fall in peripheral oxygen saturation (SpO 2 ) after administration of a graded epidural block. Correction of the fall in the blood pressure; however, did not improve the SpO 2 . The patient was administered an intravenous infusion of dopamine to improve the cardiac output and this led to improvement in the SpO 2 .

Anomalia de Ebstein com ventrículo esquerdo não compactado e comunicação interatrial do tipo seio coronário: um relato de caso / Ebstein's anomaly with left ventricular noncompaction and coronary sinus atrial septal defect: a case report

As cardiopatias congênitas envolvem um grupo de malformações que podem ser frequentes ou raras. Anomalias de Ebstein, ventrículo esquerdo não compactado e comunicação interatrial, tipo seio coronário, são condições incomuns na população e a ocorrência dos três defeitos, em um mesmo paciente, não possui relato prévio na literatura médica. Neste trabalho, é relatado o caso de um homem, com 37 anos de idade, que teve o diagnóstico feito em nosso serviço, por meio do ecocardiograma, dessa extremamente rara associação.

Eletrocardiograna na doença de Ebstein / Electrocardiogram in Ebstein disease

A anomalia de Ebstein é uma cardiopatia congênita rara, ocorrendo em ~1:200.000 nascidos vivos e correspondendo a <1 por cento de todas as causas de cardiopatia congênita. Constitui uma malformação da valva tricúspide e do ventrículo direito, caracterizada principalmente pelo deslocamento apical da valva tricúspide, dilatação da porção atrializada do ventrículo direito com variados graus de hipertrofia e afilamento da parede, além de dilatação da junção atrioventricular direita (annulus tricuspideo). Dois terços dos corações com anomalia de Ebstein mostram o venrículo direito diltado e o eletrocardiograma (ECG) normal na maioria dos pacientes. Pode demonstrar ondas P altas e largas como resultado do crescimento atrial direito, bem como bloqueio de ramo direito completo ou incompleto. A onda R nas derivações V1 e V2 são pequenas. Morfologias bizarras da porção terminal do QRS resultam do distúrbio de condução infra-Hissiana e da ativação anormal do ventrículo direito atrializado.

Ebstein’s anomaly is a rare congenital heart disease occurring in ~1:200,000 live births and accounting for <1% of all causes of congenital heart disease. It isa malformation of the tricuspid valve and the right ventricle characterized mainly by the downward (apical) displacement of the tricuspid valve, dilation of the atrialized portion of the right ventricle with various degrees of wall thinning and hypertrophy, and dilation of the right atrioventricular junction (true tricuspid annulus) Two thirds of hearts with Ebstein’s anomaly show dilated right ventricles. The ECG is abnormal in most patients with Ebstein’s anomaly and may display tall broad P waves as a result of right atrial enlargement, as well as complete or incomplete right bundle branch blocks. The R waves in lead V1and V2 are small. Bizarre morphologies of the terminal QRS pattern result from infra-Hisian conduction disturbances and abnormal activation of the atrializedright ventricle.

Anesthetic management for surgical repair of Ebstein's anomaly along with coexistent Wolff-Parkinson-White syndrome in a patient with severe mitral stenosis.

Ebstein's anomaly (EA) is the most common cause of congenital tricuspid regurgitation. The associated anomalies commonly seen are atrial septal defect or patent foramen ovale and accessory conduction pathways. Its association with coexisting mitral stenosis (MS) has uncommonly been described. The hemodynamic consequences and anesthetic implications, of a combination of EA and rheumatic MS, have not so far been discussed in the literature. We report successful anesthetic management of a repair of EA and mitral valve replacement in a patient with coexisting Wolff-Parkinson-White (WPW) syndrome.

Doença de Ebstein: ausência de bloqueio de ramo direito como preditor de via anômala manifestada / Ebstein disease: absence of right branch block as a predictor of manifested anomalous way

O desenvolvimento anormal da válvula tricúspide na doença de Ebstein resulta em uma série de anormalidades na ativação, que inclui condução intra-atrial demorada, bloqueio de ramo direito(BRG) e pré-excitação ventricular. O objetivo desse tabalho era definir as características do ECG antes e depois da ablação de uma via anômala atrioventricular. De 226 pacientes consecutivos com anomalia de Ebstein, 64 apresentavam taquicardia documentada...

Ablación transquirúrgica de vía accesoria atrioventricular en anomalía de Ebstein / Surgical radioerequency catheter ablation of accessory pathways in Ebstein's anomaly

Fueron estudiados 13 pacientes con anomalía de Ebstein con taquicardia por reentrada atrioventricular y al menos una vía accesoria. En 7 pacientes (53.8%) existía una vía accesoria y en 6 pacientes (46.1%) vías múltiples. En 5 pacientes (38.4%) se encontró vía accesoria tipo Mahaim. Un total de 22 vías accesorias fueron identificadas de las cuales 2 (9%) eran ocultas. El 100% se localizaron en el anillo tricuspídeo. Los sitios más frecuentes de ubicación fueron: región lateral derecha con 11 (50%) y postero-septal derecha con 5 (22.7%). Diez pacientes recibieron tratamiento mediante ablación con radiofrecuencia vía transquirúrgica y tres mediante sección quirúrgica. Se obtuvo un éxito de 100%. No se presentaron complicaciones durante los procedimientos. Conclusión: La ablación transquirúrgica de vías accesorias atrioventriculares en pacientes con anomalía de Ebstein es un procedimiento seguro y eficaz.

Thirteen patients with Ebstein anomaly and atrioventricular reentrant tachycardia and at least one accessory pathway were included in this study. One accessory pathway in 7 (53.8%) patients and multiple accessory pathways in 6 (46.1%) were found. Mahaim accessory pathway was observed in 5 (38.4%) patients. A total of 22 accessory pathways was found, 2 (9%) were concealed. In all (100%), the accessory pathways were located in the tricuspid ring. The most frequent regions were right lateral free wall with 11 (50%) and 5 right posteroseptal (22.7%). Ten patients underwent surgical radiofrequency catheter ablation and three subjected to surgical section. We obtained success in 100%. No complications were observed during the procedures. In conclusion, surgical ablation therapy in patients with accessory pathways and Ebstein's anomaly is safe and effective.

Cierre percutáneo de foramen oval permeable con dispositivo de Amplatzer: Presentación de dos casos / Percutaneous closure of patent foramen ovale with Amplatzer device: Report of two cases

Propósito: Se presentan los 2 casos de foramen oval permeable tratados con dispositivo de Amplatzer por vía percutánea. Método: El primero del sexo femenino de 48 años y con antecedentes de enfermedad de Ebstein moderada a severa y de 3 accidentes vasculares cerebrales, siendo el último bajo efecto anticoagulante, manejada con antiarrítmicos a pesar de lo cual presentó bloqueo auriculoventricular (a-v) de primer grado, aumento del automatismo supraventricular y ventricular de diferente foco y bloqueo de rama derecha. El segundo paciente masculino de 22 años con antecedente de un evento embólico cerebral a los 21 años con secuelas de crisis convulsivas. Ambos fueron tratados por vía percutánea para implante de dispositivo de Amplatzer, el primero para foramen oval permeable (FOP) y el segundo con dispositivo para comunicación interatrial por el diámetro del foramen. Los dos pacientes se encuentran asintomáticos. Conclusiones: El cierre de FOP por vía percutánea está indicado en pacientes con antecedentes de eventos cerebrales transitorios o permanentes y se sospeche esta vía como fuente embolígena. La técnica es fácil de realizar pero se requiere de un mayor número de pacientes para concluir.

Objective: We present two cases of Patent Foramen Ovale (PFO) treated with percutaneous Amplatzer device. Method: The first case corresponds to 48 years old woman with Ebstein's disease with moderate to severe hemodynamic repercussion and three cerebrovascular accidents, the last one under coumarin treatment, she received antiarrhythmic medication and despite of it developed 1st degree AV block, supraventricular and ventricular ectopia, rigth branch block. The second case corresponds to a 22 years old man with antecedents of cerebrovascular accident at the age of 21 with sequelae of convulsive crisis. Both patients were percutaneously treated with Amplatzer devices. The first patient was treated with a foramen ovale device and second with septal occluder due to the diameter of the foramen. Both patients have remained asymptomatic during the follow-up period. Conclusions: The PFO devices are indicated for patients with a history of cerebrovascular accidents.

Transatrial permanent pacing lead implantation in a patient of Ebstein's anomaly after one and half repair.

Several innovative approaches have been described to achieve endocardial pacing in patients with operated complex congenital heart diseases. We report herein a case of Ebstein's anomaly who underwent a Hardy's repair with a bidirectional Glenn shunt, tricuspid valve annuloplasty and atrial septal defect closure following which she developed complete heart block. The chest was reopened through the previous midsternotomy and a screw-in lead implanted transatrially that resulted in optimal pacing thresholds. This technique offers a viable alternative for endocardial pacing in peri-operative patients requiring permanent pacing.

Unusual ST-t changes in Ebstein's anomaly with occlusion of a nondominant coronary artery.

We present a case report of a patient of Ebstein's anomaly presenting with unusual ECG changes during acute coronary syndrome. The patient had undergone radiofrequency ablation of right posteroseptal accessory pathway. Two years later, he presented with acute chest pain. His ECG revealed ST elevation of 6-7 mm in leads III, aVF. V3R and V1-V4 with atrioventricular dissociation. He was thrombolysed for the same. He subsequently underwent an angiogram for continuing angina. His angiogram showed a nondominant right coronary artery with a 95% stenosis. The left circumflex artery was dominant but without any stenosis. The left anterior descending artery was also normal. Angiogplasty and stenting were done for the right coronary artery lesion and the patient did well on follow-up. The ST segment elevation in the anterior precordial leads resulting from occlusion of a nondominant right coronary artery is unusual. The possible reason for this is the isolated right ventricular infarction in the absence of any left ventricular infarction. Thus the electrical current of injury resulting from the right ventricular infarction was unopposed by any counterbalancing current of injury from the inferior surface of the left ventricle.

Anomalia de Ebstein com valva tricúspide imperfurada diagnóstico pré-natal / Ebstein's anomaly with imperforate tricuspid valve. Prenatal diagnosis

Ebstein's anomaly is an uncommon congenital heart defect, with a prevalence of 0.3-0.5 percent. Its association with an imperforate tricuspid valve is an even more rare situation (less than 10 percent of cases). Prenatal diagnosis of this association by means of fetal echocardiography has not been reported. We describe here this association diagnosed before birth and confirmed after birth. The diagnostic potential and importance of fetal echocardiography during prenatal evaluation of cardiac malformations allows for adequate perinatal planning and management, with an obvious impact on morbidity and mortality

Anomalia de Ebstein com coarctaçäo de aorta: uma associaçäo rara / Ebstein's anomaly with coarctation of the aorta: an unusual association

Ebstein's anomaly with coarctation of the aorta is an extremely unusual condition. In this report, the clinical and surgical features of 3 male patients, aged 7 months, 4 years and 14 years, are discussed. All patients were in situs solittus. The first 2 patients had atrioventricular and ventriculoarterial discordance and progressed to heart failure in the neonatal period. The third had atrioventricular and ventriculoarterial concordance, as well as Wolf-Parkinson-White syndrome, with frequent episodes of paroxysmal tachycardia. The 3 patients underwent surgery for correction of the coarctation of the aorta. The patient with atrioventricular and ventriculoarterial concordance underwent tricuspid valvuloplasty using a De Vega-like technique. In addition, ablation of 2 anomalous pathways (Kent bundle), which were detected by the electrophysiologic study, was also subsequently performed. The 3 patients showed a good postoperative outcome for 2 years, although, in those with discordance, the surgical procedure did not influence the dysplasia of the tricuspid valve, because this valve showed light to moderate dysfunction.

Anomalías de Ebstein relacionada con derrame pericárdico importante. Informe y análisis de un caso / Ebstein's anomaly associated to important pericardial effusion. A case report

La anomalía de Ebstein es una malformación congénita de la válvula tricúspide que se caracteriza desde el punto de vista clínico por la presencia de disnea, cianosis evolutiva y fatiga. Desde el punto de vista anatómico se constituye por adosamiento de sus valvas a las paredes del ventrículo derecho, así como displasia de éstas, condicionando un atrio derecho funcionalmente mayor y un ventrículo derecho pequeño. La característica radiográfica más frecuente es una gran cardiomegalia con imagen de "Garrafa". El clínico experimentado, frente a un paciente joven relativamente asintomático y con dicho hallazgo radiográfico, piensa rápidamente en dicha posibilidad diagnóstica; sin embargo, el primer paso a seguir, es en general el descartar que dicha imagen radiográfica, por su gran similitud no sea consecuencia de un gran derrame pericárdico. El ecocardiograma viene casi siempre a complementar el diagnóstico diferencial, que suele descartar una y confirmar la otra patología. El caso que estamos presentando, inusual y hasta donde nosotros sabemos, único caso de la literatura, refiere a un paciente en quien la coexistencia de un gran derrame pericárdico y la anomalía de Ebstein eran condicionantes de una gran cardiomegalia radiográfica y de las manifestaciones clínicas. Permite además, analizar las alteraciones hemodinámicas de la anomalía, sometida a un incremento de la presión pericárdica. Hacemos a la vez un análisis de la posible correlación etiopatogénica de ambas

Ablaçäo por radiofrequência na Síndrome de Wolff-Parkinson-White com anomalia de Ebstein / Radiofrequency ablation on Wolff-Parkinson-White Syndrome with Ebstein's Anomaly

Os autores relatam o caso de um paciente de 9 anos, portador da Anomalia de Ebstein e Síndrome de Wolff-Pardinson-White, apresentando episódios de síncope precedidas por taquicardia, o qual foi submetido a ablaçäo por radiofrequência de uma via acessória médio-septal e outra pósteroseptal direita com sucesso. Estratégias eletrofisiológicas utilizadas para aumentar a eficácia do mapeamento e da ablaçäo das vias acessórias nestes pacientes säo revistas.