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.

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.

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.

Factors affecting mortality in Ebstein's anomaly of the tricuspid valve.

Ebstein's anomaly of the tricuspid valve is a relatively uncommon congenital heart defect. Twenty-one patients (11 boys and 10 girls) with Ebstein's anomaly were reviewed regarding clinical characteristics and factors related to the outcome of this lesion. Their ages at presentation ranged from 1 day to 13 years (median = 11 months). Eleven patients presented in infancy period, 6 of them were neonates. Common clinical findings were systolic murmur (85.7%) and cyanosis (57.1%). Laboratory findings included cardiomegaly on chest X-rays (95.2%), right bundle branch block pattern (76.2%) and right atrial enlargement (61.9%) on electrocardiography. Diagnosis and grading of severity were established by echocardiography. Among 21 patients, 4 were lost during follow-up. Seventeen patients were followed for 3-72 months. Six patients (28.6%) required surgery, 5 of whom died following surgery. Two patients died during the medical follow-up. Factors affecting cardiac death were the younger age at presentation, onset of cyanosis in infancy period, associated PS or PA, the lower insertion of the septal leaflet of the tricuspid valve and the higher ratio of the combined area of right atrium and atrialized right ventricle to that of functional right ventricle and left heart chambers.

Ebstein's anomaly. Clinical profile en 174 patients

La población estudiada está formada por 148 pacientes sin tratamiento quirúrgico y 26 que fueron operados durante el periodo de observación la mayor parte de ellos diagnosticados después de la edad de 2 años con, un seguimiento de 6 meses a 25.3 años. Los pacientes fueron divididos en tres Grupos de deterioro clínico tomando en cuenta la clase funcional y el ICT. El seguimiento en 148 pacientes no operados mostró diferencias significativas para la mortalidad entre los Grupos I y III (p < 0.001), y entre los Grupos II y III (p< 0.02). Los factores predictores de muerte incluyeron la asociación entre clase funcional III o IV más ICTò65 por ciento asociados a cianosis o arritmias (p< 0.05). El análisis multivariado mostró que el deterioro clínico (p < 0.0001), ICT (p < 0.0002) y clase funcional (p< 0.001), tuvieron valores significativos para la mortalidad. La curva actuarial mostró sobrevida de 81 por ciento en los pacientes que no fueron tratados quirúrgicamente. De acuerdo a la curva actuarial, la tasa de sobrevida fue menor en pacientes con ICT ò de 65 por ciento (63.5 por ciento), en pacientes que tenían clase funcional IV (52.5 por ciento) en el Grupo III de deterioro clínico (38.2 por ciento). Si bien la asociación entre clase funcional III o IV y ICTò de 65 por ciento asociados a cianosis o arritmia son factores predictivos para la mortalidad en pacientes que presentaron solamente una de esas variables fué poco importante. Los pacientes incluidos en el Grupo II de deterioro clínico, en condiciones clínicas estables, presentaron una larga sobrevida con el tratamiento médico. Debido a la alta tasa de mortalidad encontrada en el Grupo III, el tratamiento quirúrgico de la Anomalía de Ebstein debe ser realizado antes de esta etapa de deterioro clínico, de acuerdo a la expresión de la Institución y comparando la tasa de mortalidad quirúrgica en la tasa de sobrevida sin operación