Ebstein’s Anomaly Corrected by Tissue Valve: Case Report

Ebstein’s anomaly is a rare form of congenital heart disease with incidence of 1% of all congenital heart diseases. There are two modes of surgical correction of Ebstein’s anomaly: either biventricular repair with or without tricuspid valve replacement, or palliative univentricular repair consisting of bidirectional Glenn shunt or Fontan procedure. We treated a case of severe form of Ebstein’s anomaly with ASD secundum with WPW syndrome. Radiofrequency ablation was done to treat WPW syndrome preoperatively. Celermajer’s index is a prognostic indicator for tricuspid valve repair or replacement. On 04.03.08 tricuspid valve was replaced with 31 mm Carpentier-Edwards bovine pericardial valve under cardiopulmonary bypass. Postoperative period was uneventful. Follow up echo done on 01.06.08 which revealed normally functioning tissue valve found in tricuspid position TR Grade I. So, in conclusion, preoperative evaluation and workout of Celermajer’s index is essential before surgical intervention for decision of tricuspid valve repair or replacement. Last but not the least, any event of arrhythmia should be properly evaluated.Key words: Ebstein’s anomaly, WPW (Wolff- Parkinson-White) Syndrome, Celermajer’s index, Tissue valve. DOI: http://dx.doi.org/10.3329/cardio.v1i1.8213 Cardiovasc. j. 2008; 1(1) : 112-114

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Ebstein's anomaly repair in infant and adult: Report of two cases

Journal of Shaheed Suhrawardy Medical College ◽

10.3329/jssmc.v3i2.12079 ◽

2012 ◽

Vol 3 (2) ◽

pp. 48-52

Author(s):

Jahangir Kabir ◽

Rezoana Rima ◽

Soofia Khatoon ◽

Rezaul Hassan ◽

Md. Zakir Hossain

Keyword(s):

Tricuspid Valve ◽

Congenital Heart ◽

Heart Defects ◽

Young Infant ◽

Ventilatory Support ◽

Ebstein’S Anomaly ◽

Tricuspid Valve Repair ◽

Ebstein's Anomaly ◽

Bidirectional Glenn Shunt ◽

Grade 3

Ebstein's anomaly (EA) is a rare but fascinating congenital heart disorder accounting for <1% of all congenital heart defects. It is a complex congenital anomaly with a broad pathologic-anatomical and clinical spectrum and no two patients are alike. Moderate to severe Ebstein's malformation remains a surgical challenge. We report our institutional experience with the surgical management of Ebstein's anomaly in a severely symptomatic four months old infant and a twenty year old grown up adult. The young infant with Ebstein's anomaly had severe tricuspid valve dysplasia, cyanosis, and gross cardiomegaly, hepatomegaly. Echocardiography severity score was 1.1 in infant (grade 3/4). Repair consisted of tricuspid valve repair, reduction annuloplasty, partial closure of atrial septal defect, and correction of all associated cardiac defects (PDA ligation).Adult Ebstein's anomaly patient underwent One and half ventricle repair which includes De-Vega repair of tricuspid valve, plication of atrialized RV , closure of ASD and a bidirectional Glenn shunt as part of the repair. The postoperative course was uneventful in adult patient except mild RV diastolic dysfunction. The infant required prolonged mechanical ventilatory support. She also developed lungs collapse-consolidation following extubation and septicaemia. At 2 years follow-up both patients showed improved exercise tolerance, infant showed significant weight gain. Echocardiographic studies reveal satisfactory monocusp valve function in both patients, with mild non- coaptation of the anterior leaflet and the septal structures with mild tricuspid valve regurgitation. Ebstein's repair has a good functional and hemodynamic outcome in infant and adult patients. One and one-half ventricular repair can be performed with an acceptable risk in adult and a biventricular repair is feasible and safe in a symptomatic infant. J Shaheed Suhrawardy Med Coll, 2011;3 (2): 48-52 DOI: http://dx.doi.org/10.3329/jssmc.v3i2.12079

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A case of redo tricuspid valve repair after neonatal cone operation for Ebstein’s anomaly

General Thoracic and Cardiovascular Surgery ◽

10.1007/s11748-021-01742-x ◽

2021 ◽

Author(s):

Junya Nabeshima ◽

Masaaki Yamagishi ◽

Yoshinobu Maeda ◽

Hisayuki Hongu ◽

Hiroki Nakatsuji ◽

...

Keyword(s):

Tricuspid Valve ◽

Ebstein’S Anomaly ◽

Tricuspid Valve Repair ◽

Valve Repair ◽

Ebstein's Anomaly

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Tricuspid Valve Repair for Ebstein’s Anomaly in Young Children: A 30-Year Experience

The Annals of Thoracic Surgery ◽

10.1016/j.athoracsur.2005.08.011 ◽

2006 ◽

Vol 81 (2) ◽

pp. 690-696 ◽

Cited By ~ 48

Author(s):

Umar S. Boston ◽

Joseph A. Dearani ◽

Patrick W. O’Leary ◽

David J. Driscoll ◽

Gordon K. Danielson

Keyword(s):

Young Children ◽

Tricuspid Valve ◽

Ebstein’S Anomaly ◽

Tricuspid Valve Repair ◽

Valve Repair ◽

Ebstein's Anomaly

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Posterior annular plication: tricuspid valve repair in Ebstein's anomaly

The Annals of Thoracic Surgery ◽

10.1016/j.athoracsur.2003.11.025 ◽

2004 ◽

Vol 77 (6) ◽

pp. 2167-2171 ◽

Cited By ~ 17

Author(s):

Camille L Hancock Friesen ◽

Robert Chen ◽

Jonathan G Howlett ◽

David B Ross

Keyword(s):

Tricuspid Valve ◽

Ebstein’S Anomaly ◽

Tricuspid Valve Repair ◽

Valve Repair ◽

Ebstein's Anomaly

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Modified Starnes procedure as a bridge to 2-ventricle repair in neonatal Ebstein’s anomaly: A paradigm shift

10.1510/mmcts.2021.077 ◽

2021 ◽

Keyword(s):

Tricuspid Valve ◽

Single Ventricle ◽

Hemodynamic Instability ◽

Ebstein’S Anomaly ◽

Tricuspid Valve Repair ◽

Valve Regurgitation ◽

Valve Repair ◽

Tricuspid Valve Regurgitation ◽

Ebstein's Anomaly ◽

Single Ventricle Palliation

Severe tricuspid valve regurgitation secondary to Ebstein’s anomaly represents several challenges in neonates. It can result in significant respiratory and/or hemodynamic compromise that mandates urgent interventions. When conservative management fails, 2 surgical options are available: tricuspid valve repair or single ventricle palliation. The overall results of neonatal tricuspid valve repair are unsatisfactory especially in sick neonates and those with preoperative hemodynamic instability. Single ventricle palliation utilizing the Starnes procedure with right ventricular exclusion provides a quicker way to improve hemodynamics and allows rapid decompression of the right ventricle but carries the long-term disadvantages of the single ventricle pathway. We were recently faced with a challenging case of neonatal Ebstein’s anomaly resulting in severe tricuspid valve regurgitation (TR) and significant hemodynamic and respiratory instability. We performed an initial stage I palliation with a modified Starnes’ procedure, which allowed stabilization and rapid recovery of the patient to be followed 5 months later with conversion to 2-ventricle repair using the cone technique. We believe combining these 2 strategies for suitable neonatal candidates may be a useful technique that should be considered in the algorithm for neonatal Ebstein’s anomaly.

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