Surgical treatment of Ebstein anomaly in pediatric patients: A 10‐year single‐center study

Abstract

Objective We report the early and long‐term results of the strategies and surgical methods used in our center to treat pediatric patients who underwent surgical intervention to correct Ebstein anomaly (EA) in our center. Materials and Methods In our study, a consecutive sample of 29 patients who underwent surgery for EA between February 2011 and February 2020 in our center were evaluated retrospectively. Results The 29 patients underwent a total of 40 operations. Univentricular repair was performed in 5 (17.2%), 1.5 ventricular repair in 5 (17.2%), and biventricular repair in the remaining 19 (65.5%) patients. Cone reconstruction (CR) was performed in eight (27.5%), non‐Cone tricuspid valve (TV) repair technique in five (17.2%), ring annuloplasty in two (6.9%), and TV replacement in two patients (6.9%) who had undergone biventricular repair. In two patients (6.9%), only close an atrial septal defect. Two (6.9%) patients underwent a second operation for advanced tricuspid regurgitation (TR) in the early period. None of the 15 patients who underwent CR and TV plasty had moderate or advanced TR before discharge. Early mortality was seen in 1 (3.4%) patient. The mean follow‐up period of the patients was 48.4±27.6 months. Three (10.7%) of the patients who were discharged after their first operation later underwent a second operation for TV regurgitation in the long term. No mortality was observed in any patient during long‐term follow‐up. Conclusion Surgical treatment of EA is difficult, but its overall results are good. The anatomical repair rate is lower in neonatal and infant patients requiring surgery, but most of these patients underwent biventricular repair. Our long‐term results demonstrated an acceptable survival rate, low mortality in the early postoperative period, and low incidence of re‐intervention and morbidity.

Review of surgical experience in 188 infants and young children with Ebstein anomaly

“…The optimal timing of surgical intervention for pEA remains inconclusive. The present study trends towards younger children (median age: 3 years) when compared to the prior paediatric-specific cohorts [ 11 , 22 ] (excluding those focusing on neonatal or infantile patients). In this series, no difference in the risk of recurrent TR or death was observed between the different age subgroups.…”

The Association of Pulmonary Flow Characteristics With Cardiac Function in Tricuspid Valve Malformation Fetuses

“…EA and TVD are rare CHDs with TR and right heart enlargement 18,30–33 . Although recent advances in prenatal diagnosis and surgical techniques have allowed earlier detection and treatment of this malformation, termination of pregnancy, and perinatal mortality in TVM fetuses remain high 34–36 .…”

“…EA and TVD are rare CHDs with TR and right heart enlargement. 18,[30][31][32][33] Although recent advances in prenatal diagnosis and surgical techniques have allowed earlier detection and treatment of this malformation, termination of pregnancy, and perinatal mortality in TVM fetuses remain high. [34][35][36] It is important to provide accurate information about the severity of fetal TVM, as this may better enable parents to make informed decisions about the pregnancy.…”

Estrategias y avances quirúrgicos en la anomalía Ebstein: 28 años de experiencia en un hospital pediátrico

“…A pesar de los buenos resultados reportados a mediano y largo plazo de estas técnicas, la incidencia de arritmias, disfunción del VD e IT residual o recurrente es alta, por lo que la necesidad de reoperaciones es frecuente [6][7][8][9] .…”