Cone reconstruction in children with Ebstein anomaly: the Mayo Clinic experience.

Published on May 1, 2014in Congenital Heart Disease2.007
· DOI :10.1111/CHD.12155
Joseph A. Dearani8
Estimated H-index: 8
 (Mayo Clinic)
Sources
Abstract
Objective. Valve repair for pediatric patients with Ebstein anomaly has historically yielded varied results. The cone reconstruction (CR) first described by Da Silva has revolutionized the surgical approach to these patients. This study reports our recent experience with CR in children and young adults with Ebstein anomaly. Design. Electronic medical records were reviewed for all patients 5 mm Hg) was present in 6 patients. There was one late death (motor vehicle accident) and one late re-repair of the tricuspid valve 4 years after initial operation. Conclusions. CR in children and young adults with Ebstein anomaly can be performed with low early mortality and excellent durability at short-term follow-up. CR represents an important surgical option for young patients. It is applicable to patients with a broad range of anatomic variability and precludes valve replacement in the vast majority. CR should be considered prior to the deleterious effects of chronic right ventricular volume overload and the development of systolic dysfunction, which hamper long-term prognosis. Therefore, early referral for surgical evaluation is recommended.
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References19
Newest
Jan 1, 2014·European Journal of Cardio-Thoracic Surgery4.19
#1Ymkje J. van Slooten (UG: University of Groningen)H-Index: 7
#2Hendrik G. Freling (UG: University of Groningen)H-Index: 8
Last. Petronella G. Pieper (UG: University of Groningen)H-Index: 42
view all 8 authors...
OBJECTIVES: In patients with acquired valvar disease, morbidity and mortality rates after tricuspid valve replacement (TVR) are high. However, in adult patients with congenital heart disease, though data concerning outcome after TVR are scarce, even poorer results are suggested in patients with Ebstein anomaly. To investigate the applicability of these results to a broader array of congenital heart disease patients, we report the long-term follow-up of prosthesis-related complications, including...
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Background Successful tricuspid repair for Ebstein malformation (EM) is challenging. Cone repair has recently emerged as the most anatomic repair technique. There are no data on tricuspid re-repair in EM. We reviewed our experience with tricuspid valve (TV) re-repair using a cone technique. Methods Between June 2007 and October 2012, 134 patients underwent cone repair. Prior TV repair was performed in 20 patients (10 female, 50%). Median age was 15 years (range, 4 to 68 years). Four patients (20...
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Jan 1, 2013·The Annals of Thoracic Surgery4.33
#1Joseph A. Dearani (Mayo Clinic)H-Index: 95
#2Sameh M. Said (Mayo Clinic)H-Index: 23
Last. Frank Cetta (Mayo Clinic)H-Index: 46
view all 6 authors...
Background Reproducible repair of Ebstein's malformation is challenging and numerous surgical techniques have been described. We reviewed our experience with the cone reconstruction. Methods Between June 2007 and December 2011, 89 patients (47 female; 53%) underwent cone reconstruction (median age 19 years; range, 19 days to 68 years). Indication for operation was progressive cardiomegaly in 43 (48%), cyanosis in 29 (33%), and heart failure in 13 (15%). Prior tricuspid valve repair was performed...
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Oct 1, 2012·The Annals of Thoracic Surgery4.33
#1S SataH-Index: 6
#2Peter MurinH-Index: 9
Last. Viktor HraskaH-Index: 23
view all 3 authors...
Neonatal repair of Ebstein's anomaly is challenging and should be considered only if medical measures to stabilize the circulation and provide antegrade pulmonary blood flow fail. Anatomic repair, based on the cone reconstruction technique, has demonstrated promising survival benefits in older patients; however, there are no data regarding neonatal repair. This is a report on a successful salvage operation using cone reconstruction of Ebstein's anomaly in a neonate who had required extracorporea...
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Jan 1, 2012·Congenital Heart Disease2.01
#8Emile A. Bacha (Boston Children's Hospital)H-Index: 65
Objectives. We report our analysis of conventional surgery and the cone procedure for Ebstein's malformation (EM) of the tricuspid valve at a single institution. Previous conventional surgery for EM, including use of bioprosthetic valves, has inherent problems especially in pediatrics. The newer cone procedure aims to construct a funnel-like valve out of native leaflets, obviating problems with artificial valves. Methods. This is a retrospective cohort study to examine short-term outcomes of bot...
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Aug 5, 2011·Arquivos Brasileiros De Cardiologia2.00
#1José Pedro da Silva (USP: University of São Paulo)H-Index: 13
#2Luciana da Fonseca da SilvaH-Index: 6
Last. Gláucia Maria Penha TavaresH-Index: 7
view all 8 authors...
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 rad...
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Jan 1, 2010·European Journal of Cardio-Thoracic Surgery4.19
#1CC Badiu (TUM: Technische Universität München)H-Index: 12
#2Christian Schreiber (TUM: Technische Universität München)H-Index: 43
Last. Rüdiger Lange (TUM: Technische Universität München)H-Index: 58
view all 8 authors...
Objective: Various surgical valve repair and replacement techniques have been developed over the past decades for patients with Ebstein’s anomaly. Determination of the appropriate moment for surgery, however, has not been elucidated clearly enough. Methods: From 1976 to 2007, 130 patients (mean age 23.8 17.8 years, range: 1 month to 73.6 years) underwent surgery for Ebstein’s anomaly at our centre. Four patients (3.0%), who underwentuniventricular palliation, and four (3.0%), who only had an atr...
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Jun 1, 2008·Operative Techniques in Thoracic and Cardiovascular Surgery
#1Joseph A. Dearani (Mayo Clinic)H-Index: 95
#2Emile Bacha (Mayo Clinic)H-Index: 1
Last. José Pedro da Silva (Mayo Clinic)H-Index: 1
view all 3 authors...
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Objective Our objective was to review the long-term outcomes of patients with Ebstein anomaly who underwent cardiac surgery at our institution. Methods Patient records were reviewed, and all patients were mailed a medical questionnaire or contacted by means of telephone. Patients who had pulmonary atresia with an intact ventricular septum, complex conotruncal abnormalities, and atrioventricular discordance with ventriculoarterial discordance were excluded. Results From April 1, 1972, to January ...
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May 1, 2007·The Journal of Thoracic and Cardiovascular Surgery5.21
#1Luis G. Quinonez (Mayo Clinic)H-Index: 9
#2Joseph A. Dearani (Mayo Clinic)H-Index: 95
Last. Gordon K. Danielson (Mayo Clinic)H-Index: 100
view all 7 authors...
Objective Repair of Ebstein anomaly and impaired right ventricular function pose challenges for the cardiac surgeon. The bidirectional cavopulmonary shunt may improve early outcomes. We reviewed our experience with the 1.5-ventricle repair in this patient population. Methods Between July 1999 and March 2006, 169 patients underwent operations to repair Ebstein anomaly. Fourteen patients had a bidirectional cavopulmonary shunt constructed. The median age at operation was 6 years (17 months-57.8 ye...
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12
Cited By49
Newest
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 perfor...
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Source
Cone repair (CR) uses native tissue for tricuspid valve (TV) repair and provides potential for growth. Results after CR were investigated in different age groups including several surgical modifications. Single institution retrospective analysis of all CR excluding neonatal procedures. Endpoints included TV reoperation, late tricuspid regurgitation (TR) and death. Between April 2006 and August 2019, 157 patients underwent CR at a median age of 11.7 years (range, 0.3-57.2). 20% (n=32) of patients...
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Source
#1Santosh Uppu (University of Texas Health Science Center at Houston)H-Index: 1
Congenital tricuspid valve anomalies are rare congenital heart defects (CHDs). Tricuspid atresia is more common among them followed by Ebstein anomaly. Tricuspid atresia results from the absence of the tricuspid valve along with right ventricular hypoplasia. Surgical approach for tricuspid atresia evolved over time from a palliative procedure to control pulmonary overcirculation to Fontan et al. describing a way to separate pulmonary and systemic venous return in the early 1970s. Ebstein anomaly...
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Nov 8, 2020·World Journal for Pediatric and Congenital Heart Surgery
#1Elizabeth H. Stephens (Mayo Clinic)H-Index: 25
#2Joseph A. Dearani (Mayo Clinic)H-Index: 95
Last. Joseph J. Maleszewski (Mayo Clinic)H-Index: 42
view all 5 authors...
Ebstein anomaly has a breadth of presentations, including "typical" and "atypical," and can be confused with congenital tricuspid dysplasia. We summarize how to differentiate within this spectrum of disease. Both typical and atypical Ebstein have an underlying failure of delamination, but atypical Ebstein does not have ≥8mm/m2 apical septal leaflet displacement. In congenital tricuspid dysplasia, delamination is normal, while the leaflets and subvalvar apparatus are abnormal. To summarize, the s...
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May 1, 2020·Journal of Cardiothoracic and Vascular Anesthesia2.63
#1Tiffany M. Williams (UCLA: University of California, Los Angeles)H-Index: 1
#2Gentian Lluri (UCLA: University of California, Los Angeles)H-Index: 10
Last. Wolf B. Kratzert (UCLA: University of California, Los Angeles)H-Index: 3
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Abstract Survival of patients with congenital heart disease has significantly improved over the last two decades confronting interventionalists, surgeons, anesthesiologists, cardiologists, and intensivists with often unfamiliar complex pathophysiology in the perioperative setting. Aside from cardiac catheterization, echocardiography has become the main imaging modality in the hospitalized adult with congenital heart disease. The great variety of congenital lesions and their prior surgical manage...
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Although the Cone procedure has improved outcomes for patients with Ebstein s anomaly (EA), neither RV systolic function recovery in long-term follow-up nor the best echocardiographic parameters to assess RV function are well established. Thus, we evaluated RV performance after the Cone procedure comparing two-dimensional (2DEcho) and three-dimensional (3DEcho) echocardiography to cardiac magnetic resonance (CMR). We assessed 27 EA patients after the Cone procedure (53% female, median age of 20 ...
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Feb 1, 2020·Heartrhythm Case Reports
#1Kyung Jin Ahn (Gachon University)H-Index: 2
#2Mi Kyoung Song (SNU: Seoul National University)H-Index: 9
Last. Eun Jung Bae (SNU: Seoul National University)H-Index: 21
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