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.

Anesthetic management for patient with severe cyanosis following bioprosthetic valve stenosis / Manejo anestésico de paciente com cianose grave após estenose de valva bioprotética

Abstract We presented a 39-year-old female patient with life-threatening hypoxemia after tricuspid valve replacement because of Ebstein's anomaly. And the severe cyanosis is due to bioprosthetic valve stenosis and atrial septal defect. Anesthetic management of a patient with severe obstructive prosthetic valve dysfunction can be challenging. Similar considerations should be given to patients with Ebstein's anomaly to maintain the pressure equalized between the right and left atrial. Transesophageal echocardiography and cerebral oxygen saturation provided real time information in perioperative care.

Resumo Apresentamos o caso de uma paciente de 39 anos, com hipoxemia em risco de vida após a substituição da valva tricúspide devido à anomalia de Ebstein e cianose grave devido à estenose de valva bioprotética e comunicação interatrial. O manejo anestésico de um paciente com disfunção obstrutiva grave de prótese valvar pode ser um desafio. Os pacientes com anomalia de Ebstein também precisam de atenção especial para manter a pressão equalizada entre o átrio direito e o esquerdo. A ecocardiografia transesofágica e a saturação cerebral de oxigênio forneceram informações em tempo real nos cuidados perioperatórios.

Ebstein's anomaly: "The one and a half ventricle heart"

Abstract Objective: Ebstein's anomaly remains a relatively ignored disease. Lying in the 'No Man's land' between congenital and valve surgeons, it largely remains inadequately studied. We report our short-term results of treating it as a 'one and a half ventricle heart' and propose that the true tricuspid annulus (TTA) 'Z' score be used as an objective criterion for estimation of 'functional' right ventricle (RV). Methods: 22 consecutive patients undergoing surgery for Ebstein's anomaly were studied. Echocardiography was performed to assess the type and severity of the disease, tricuspid annular dimension and its 'Z' score. Patients were operated by a modification of the cone repair, with addition of annuloplasty, bidirectional cavopulmonary shunt (BCPS) and right reduction atrioplasty to provide a comprehensive repair. TTA 'Z' score was correlated later with postplication indexed residual RV volume. Results: There was one (4.5%) early and no late postoperative death. There was a significant reduction in tricuspid regurgitation grading (3.40±0.65 to 1.22±0.42, P<0.001). Residual RV volume reduced to 71.96±3.8% of the expected volume and there was a significant negative correlation (rho −0.83) between TTA 'Z' score and indexed residual RV volume. During the follow-up of 20.54±7.62 months, the functional class improved from 2.59±0.7 to 1.34±0.52 (P<0.001). Conclusion: In Ebstein's anomaly, a higher TTA 'Z' score correlates with a lower postplication indexed residual RV volume. Hence, a complete trileaflet repair with offloading of RV by BCPS (when the TTA 'Z' score is >2) is recommended. The short-term outcomes of our technique are promising.

Consideraciones anestésicas en la anomalía de Ebstein / Anesthetic considerations in Ebstein's anomaly

Introducción: la anomalía de Ebstein es una rara malformación congénita cardiaca, definida por un desplazamiento apical de las valvas septal y posterior de la válvula tricúspide hacia el interior del ventrículo derecho, en vez de hacerlo a nivel del anillo auriculoventricular, lo cual conlleva un aumento del volumen de la aurícula derecha (megaurícula) a expensas de una reducción del tamaño del ventrículo del mismo lado (auriculización del ventrículo). Causa una significante regurgitación tricuspídea y reducción de la capacidad funcional del ventrículo, dilatación atrioventricular derecha, con arritmias auriculares y ventriculares, las que tienden a hacerse resistentes en ocasiones, o de difícil tratamiento. El desplazamiento del orificio valvular tricuspídeo produce una división del ventrículo derecho en una porción integrada al atrio derecho, lo que constituye la porción atrializada del ventrículo derecho, en tanto la porción apicotrabecular y de salida constituye su parte funcional. Objetivo: presentar la evolución clínico-anestesiológica de una paciente con anomalía de Ebstein. Caso clínico: paciente de 69 años de edad programada para cirugía electiva en dos ocasiones por diferentes enfermedades oncológicas, portadora de anomalía de Ebstein. Antecedentes de tromboembolismo pulmonar, tres cirugías cardiacas, diabetes mellitus e hipertensión arterial, enfermedad arterial aterosclerótica estable e infarto cerebral media derecha embólico. Se evitaron aquellas condiciones que aumenten el trabajo cardiaco y el consumo de oxígeno. Conclusiones: el desafío que representa para el anestesiólogo, aquellos pacientes que sufren anomalía de Ebstein puede variar de paciente a paciente, en dependencia del estado físico, el tipo de enfermedad y las técnicas de monitorización(AU)

Introduction: Ebstein's anomaly is a rare congenital cardiac malformation, defined by an apical displacement of the septal and posterior leaflets of the tricuspid valve into the right ventricle, instead of at the level of the atrioventricular ring, which leads to an increase of the volume of the right atrium (mega atrium) at the expense of a size reduction for the ventricle on the same side (auriculization of the ventricle). It causes a significant tricuspid regurgitation and reduction of the functional capacity of the ventricle, right atrioventricular dilation, with atrial and ventricular arrhythmias, which tend to become resistant at times, or difficult to treat. The displacement of the tricuspid valve orifice produces a division of the right ventricle in a portion integrated to the right atrium, which constitutes the atrialized portion of the right ventricle, while the apico-trabecular and exit portion constitutes its functional part. Objective: To present the clinical-anesthesiological evolution of a patient with Ebstein's anomaly. Clinical case: A 69-year-old patient, with Ebstein's anomaly, who was scheduled for elective surgery on two occasions for different oncological diseases. History of pulmonary thromboembolism, three cardiac surgeries, diabetes mellitus and arterial hypertension, stable atherosclerotic arterial disease, and right middle cerebral infarction. Conditions that increase cardiac work and oxygen consumption were avoided. Conclusions: The challenge patients suffering from Ebstein's anomaly represent for the anesthesiologist can vary from patient to patient, depending on the physical state, the type of disease, and the monitoring techniques(AU)

Alternativas de estimulação cardíaca definitiva em portadores de anomalia de Ebstein recém-operados pela cirurgia do cone / Alternatives for definitive cardiac pacing in patients with Ebstein's anomaly recently operated by cone surgery

A passagem do cabo-eletrodo ventricular pela valva tricúspide pode ser um problema em pacientes com anomalia de Ebstein recém-operados. Neste trabalho, em que analisamos os prontuários de 3 pacientes, entre 1998 e 2012, são apresentadas alternativas de implante de marcapasso definitivo nesses casos. A estimulação cardíaca artificial preservando a valva tricúspide na anomalia de Ebstein corrigida pode ser feita de maneira eficaz pelas técnicas apresentadas

Passing the ventricular lead through the tricuspid valve may be a problem in patients recently operated for Ebstein's anomaly. In this study, which evaluated hospital charts of 3 patients from 1998 to 2012 we report alternatives for definitive pacemaker implantation. Artificial cardiac pacing preserving the tricuspid valve in corrected Ebstein anomaly may be performed effectively using the described techniques

Tricuspid Valve Replacement in an Adult with Destroyed Tricuspid Valve and Refractory Right Heart Failure.

22 year old woman with no previous heart disease history, presented with progressive worsening of right heart failure symptoms due to severe tricuspid valve regurgitation which had become refractory to medical management. Echocardiogram revealed probable rare case of dysplastic tricuspid valve with large calcified mobile masses attached to leaflets. Calcified masses were thought to be due to healed vegetations from silent infective endocarditis of abnormal tricuspid valve which she had suffered in the past. There were no known acquired causes of tricuspid valve endocarditis. She had successfully undergone tricuspid valve replacement with bio-prosthetic valve along with a right atrial reduction surgery after which her heart failure symptoms improved markedly.

Anomalia de Ebstein: relato de caso / Ebstein Anomaly: a case report

Justificativa e objetivos: a anomalia de Ebstein é a doença congênita mais comum da valva tricúspide. É caracterizada por aderência de porção variável das cúspides posterior e septal de uma valva tricúspide redundante para o interior da parede ventricular direita e implantada mais caudalmente em direção ao ápice. Suas manifestações clínicas bem como seu tratamento são variáveis conforme o grau de anomalia valvar. Relato do caso: paciente de 11 anos com diagnóstico de anomalia de Ebstein desde o período pós-natal imediato. Apresentava percentil 10 de peso e estatura; desenvolvimento normal. Foi indicada intervenção cirúrgica devido a quadro de dispneia aos médios esforços, piora da cianose e diaforese. Submetido à plicatura do VD atrializado, plastia da valva tricúspide com diminuição do anel tricúspide, correção da CIA e CIV sob anestesia geral balanceada associada à peridural torácica. Antes de entrar em CEC, apresentou TSV após manipulação do AD. A CEC durou 89 min e o CAo, 60 min. Saiu de CEC com adrenalina a 0,3 mcg/kg/min e milrinona a 0,75 mcg/kg/min. Aproximadamente 15 minutos após receber uma bolsa de concentrado de hemácias, duas unidades de plaquetas e 10 mL de gluconato de cálcio 10 mL a 10%, cursou com TV sem pulso revertida a ritmo juncional após a segunda desfibrilação. Foi transferido para o CTI em ritmo juncional, FC: 130 BPM, PIA: 56/40 mmHg. Conclusões: portadores de anomalia de Ebstein com mais de 10 anos frequentemente apresentam arritmias cardíacas. Há forte associação com vias de condução acessórias ­ fenômenos de pré-excitação ­ WPW (ressalta-se que no caso em questão o estudo eletrofisiológico pré-CEC não evidenciou qualquer via anômala ou fenômeno de pré-excitação). É comum a coexistência de defeitos septais. O tratamento clínico-cirúrgico deve ser individualizado, sendo mandatório conhecer alterações cardiovasculares e hemodinâ- micas associadas para otimizar o ato anestésico-cirúrgico

Justification and objectives: Ebstein anomaly is the most common congenital disorder in the tricuspid valve. It is characterized by a variable portion of the posterior and septal leaflets of a redundant tricuspid valve adhering the right ventricular wall and caudally towards the apex. Its clinical manifestations and treatments vary according to the degree of valve anomaly. Case report: An eleven-year-old patient diagnosed with Ebstein anomaly since the immediate postnatal period. The patient was in the 10th percentile of height and weight, and had normal development. Surgery was indicated due to effort dyspnea, worsening cyanosis, and diaphoresis. The patient underwent plicature of the atrialized right ventricle; tricuspid valve plastic surgery to reduce the tricuspid ring, correction of interatrial and interventricular communication with the aid of balanced, general anesthesia associated with thoracic epidural. Before initial- izing extracorporeal circulation (ECC), the patient had supraventricular tachycardia upon manipulation of the right atrium. ECC and aortic clamping lasted 89 and 60 minutes respectively. CEC stopped with 0.3 mcg/ kg/min adrenalin and 0.75 mcg/kg/min milrinone. Approximately 15 minutes after receiving a red blood cell concentrate bag, two plaque units and 10 mL of 10 % calcium gluconate, he had ventricular tachycardia without pulse that was reverted to junctional rhythm upon the second defibrillation.The patient was transferred to ICU in junctional rhythm, heart rate: 130 BPM; intra-abdominal pressure: 56/40 mmHg. Conclusions: Elbstein anomaly patients aging over 10 years old frequently have cardiac arrhythmias. There is strong association with accessory conduction pathways ­ pre-excitation phenomena ­ WPW (in the case under scrutiny, a pre-CEC electrophysiological study did not point to any anomalous pathway or pre-excitation phenomena). Coexistence of septal defects is common. Clinical and surgical treatment should be customized, and the practitioners must be aware of the associated cardiovascular and hemodynamic alterations in order to optimize both anesthetic and surgical procedures.

A técnica do cone para correção da anomalia de Ebstein: resultados imediatos e em longo prazo / Cone reconstruction in Ebstein's anomaly repair: early and long-term results

FUNDAMENTO: As principais correções da anomalia de Ebstein (AE) baseiam-se na reconstrução monocúspide da valva tricúspide e são limitadas pela frequente necessidade de substituição ou pela alta reincidência de insuficiência valvar. OBJETIVO: Avaliar a viabilidade e os efeitos da correção anatômica da anomalia de Ebstein com a técnica do cone na evolução clínica dos pacientes, na função da valva tricúspide e na morfologia do ventrículo direito. MÉTODOS: Foram comparados os dados clínicos, ecocardiográficos e radiológicos de 52 pacientes consecutivos, com idade média de 18,5 ± 13,8anos, submetidos à técnica do cone, obtidos nos períodos pré-operatório, pós-operatório imediato (POI) e em longo prazo (POL). RESULTADOS: Houve dois óbitos hospitalares (3,8 por cento) e mais dois durante o seguimento. A classe funcional média de insuficiência cardíaca pré-operatória de 2,2 melhorou para 1,2 após 57 meses de seguimento médio de 97 por cento dos pacientes (p < 0,001). O grau médio de insuficiência tricúspide pré-operatória de 3,6 diminuiu para 1,6 no POI (p < 0,001), mantendo-se em 1,9 no POL (p > 0,05). A área funcional indexada do VD aumentou de 8,53 ± 7,02 cm2/m2 no préoperatório para 21,01±6,87 cm2/m2 no POI (p < 0,001), mantendo-se inalterada em 20,28 ± 5,26 cm2/m2 no POL (p > 0,05). O índice cardiotorácico médio foi reduzido de 0,66 ± 0,09 para 0,54 ± 0,06 (p < 0,001) em longo prazo. CONCLUSÃO: A técnica do cone apresentou baixa mortalidade hospitalar, corrigindo a insuficiência tricúspide de maneira eficaz e duradoura, com a restauração da área funcional do ventrículo direito, permitindo o remodelamento reverso do coração e a melhora clínica na maioria dos pacientes em longo prazo.

BACKGROUND: The main Ebstein anomaly (EA) repairs are based on the monocusp reconstruction of the tricuspid valve and are limited by the frequent need for replacement or the high recurrence of valve regurgitation. OBJECTIVE: To evaluate the feasibility and effects of anatomical repair of Ebstein's anomaly using the cone reconstruction technique on patients' clinical evaluation, tricuspid valve function and right ventricular morphology. METHODS: We compared the clinical, echocardiographic and radiological data of 52 consecutive patients, with a mean age of 18.5 ± 13.8 years, submitted to the cone reconstruction technique, obtained in the preoperative, early postoperative (EPO) and long-term (LPO) periods. RESULTS: There were two in-hospital deaths (3.8 percent) and two more during the follow-up. Mean functional class of pre-operative heart failure improved from 2.2 to 1.2 after 57 months of mean follow up of 97 percent of patients (p <0.001). The mean degree of preoperative tricuspid regurgitation decreased from 3.6 to 1.6 in the EPO (p <0.001), remaining at 1.9 in LPO period (p> 0.05). The indexed RV functional area increased from 8.53 ± 7.02 cm2/m2 preoperatively to 21.01 ± 6.87 cm2/m2 in the EPO (p <0.001) and remained unchanged at 20.28 ± 5.26 cm2/m2 in LPO period (p> 0.05). The mean cardiothoracic ratio was decreased from 0.66 ± 0.09 to 0.54 ± 0.06 (p <0.001) in the long term. CONCLUSION: The cone technique showed low in-hospital mortality, resulting in an effective and long-lasting repair of tricuspid regurgitation, restoring the functional area of the right ventricle and allowing reverse remodeling of the heart and clinical improvement in most patients in the long term.

Anomalía de Ebstein “tipo B”: caso clínico / Ebstein’s anomaly “type B”: case report

La anomalía de Ebstein es una rara enfermedad cardíacacongénita, con incidencia 1/200.000 nacidos vivos, presenta un espectro morfológico amplio caracterizado por diferentes grados de desplazamiento y adherencia de la valvas septal y posterior de la válvula tricúspide hacia el ventrículo derecho, comunicación interauricular o foramen oval permeable, asociada a degeneración del miocardio subyacente a las valvas posterior y septal incriminadas. El reservorio venoso auricular aumenta su volumen al desplazarse el orificio tricuspídeo efectivo dentro de la cámara de entrada del ventrículo derecho, afectándose su función sistolo-diastólica y aparece cianosis secundaria a cortocircuito a nivel auricular. En 20% - 30% se asocia a síndrome de Wolff-Parkinson-White (WPW). Presentamos el caso clínico de un adolescente de 16 años con anomalía de Ebstein y taquicardia supraventricular, quién presentó evento cerebrovascular isquémico a los 7 años, la evaluación clínica y ecocardiográfica resultó en anomalía de Ebstein tipo B, se realizó ablación por radiofrecuencia eficaz de vía accesoria posterolateral derecha guiada por sistema de navegación, sin embargo, 7 meses después reingresa por disnea progresiva y acentuación de la cianosis, se realiza resonancia magnética y cateterismo cardíaco; para calcular volumen ventricular funcional del ventrículo derecho y medir presiones pulmonares, realizándole corrección bi-ventricular con técnica de Carpentier y reemplazo valvular tricuspídeo (Prótesis Medtronic Advantage N°23), con resultado satisfactorio y evolución clínica a clase funcional I-II NYHA.

Ebstein’s anomaly is a rare congenital heart disease, with incidence 1/200.000 live births, has a broad morphologics pectrum characterized by different degrees of displacement and adherence of the septal and posterior leaflets of the tricuspid valve into the right ventricle atrial septal defect or patent foramen ovale associated with myocardial degeneratio nunderlying the posterior and septal leaflets incriminated. The atrial venous reservoir increases in volume by moving the effective tricuspid orifice in the right ventricle inlet chamber, affecting systolic-diastolic function and appears cyanosis secondary to shunt at the atrial level. In 20% - 30% is associated with Wolff-Parkinson-White (WPW). We report the case of a 16-year Ebstein’s anomaly and supraventricular tachycardia, who presented ischemic stroke event at age 7-year, the clinical and echocardiographic assessment of Ebstein’s anomaly resulted in type B, was performed radiofrequency ablation of accessory pathway effective right postero-lateral guided navigation system, but seven months later readmitted because of progressive dyspnoea and cyanosis accentuation is performed cardiac MRI and catheterization to calculate volume right ventricle and measure functional pulmonary pressures, performing bi-ventricular correction technique Carpentier tricuspid valve replacement (Medtronic Advantage prosthesis No. 23), with satisfactory results and clinical functional class NYHA I-II.

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.

Nova técnica cirúrgica para a correção da anomalia de Ebstein: resultados imediatos e em longo prazo / New surgical technique for Ebsteins anomaly repair: early and long term results

Introdução: As principais operações para correção da anomalia de Ebstein baseiam-se na reconstrução da valva atrioventricular direita (AVD) em formato monovalvular, tendo resultados comprometidos pela necessidade de substituição ou alta reincidência de insuficiência valvar. Uma nova técnica foi desenvolvida, diferenciado-se das anteriores, pela correção anatômica da valva AVD, resultando na coaptação plena das válvulas no fechamento valvar. O objetivo deste estudo é avaliar a aplicabilidade dessa técnica, estudando os seus efeitos na evolução clínica, função da valva, restauração do ventrículo direito funcional e remodelamento reverso do coração no pós-operatório imediato (POI) e no pós-operatório em longo prazo (POL). Métodos: Estudo retrospectivo de 52 pacientes consecutivos, com idade média de 18,5±13,8anos, submetidos técnica do cone para correção da anomalia de Ebstein, entre novembro 1993 e dezembro de 2006, cujos principais detalhes cirúrgicos são: as válvulas anterior e posterior da valva AV direita são mobilizadas das suas implantações anômalas no ventrículo direito (VD), a borda livre desse conjunto é rodada no sentido horário para ser suturada à, previamente mobilizada, borda septal da válvula anterior, formando um cone cujo vértice permanece fixo ao ápice do VD e a base é suturada no nível do anel atrioventricular verdadeiro, reduzido ao mesmo tamanho da base do cone. A válvula septal, sempre que possível, é incorporada à parede do cone. A porção atrializada do VD é reduzida por plicatura longitudinal. Os dados clínicos, ecocardiográficos e os índices cardiotorácicos, obtidos nos períodos pré-operatório (PREOP) e pós-operatório, foram analisados. Resultados: Houve dois óbitos...

Background: The main operations for Ebsteins anomaly repair are conceived to reconstruct the tricuspid valve (TV) in a monocusp format, but their results are restricted either by the need for valve replacement or by high incidence of postoperative valve regurgitation. A new surgical technique was developed, that performs an anatomical reconstruction of the tricuspid valve, realizing a leaflet-to-leaflet coaptation at the TV closure. The objective of this study is to access the feasibility of this technique, evaluating its effects in clinical outcome, tricuspid valve function, right ventricle (RV) morphology and reverse remodeling of the heart.Methods: Retrospective study on 52 consecutive patients, mean age of 18,5+- 13,8 years, treated with a new surgical technique for Ebsteins anomaly repair (the cone technique), between November 1993 and December 2006, which principal details are: a) the anterior and posterior tricuspid valve leaflets re mobilizedfrom their anomalous attachments in the RV, the free edge of this complex is rotated clockwise to be sutured to the septal border of anterior leaflet, creatind a cone which vertex remains fixed at RV apex and whose base is the sutured to a true tricuspid annulus, plicated to match it to base of said cone. The septal leaflet is incorporated into the cone wall ewhenever possible. The atrialized chamber is reduced by longitudinal placation. The clinical and echocardiographic data and the patients cardiothoracic ratios, collected at the preoperative, early and late postoperative periods, were analyzed. Results: There were two hospital deaths (3.8 %) and two more deaths in the long term followup.

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