Ann Thorac Surg 2007;83:678-680
© 2007 The Society of Thoracic Surgeons
Case Reports
Modified Technique for the Surgical Treatment of Severe Tricuspid Valve Deformity in Ebsteins Anomaly
David P.V. Bichell, MDa,*,
Bassem N. Mora, MDa,
James W. Mathewson, MDb,
Stanley K. Kirkpatrick, MDb,
Jeffrey J. Tyner, MDc,
Terri McLees-Palinkas, MSb
a Department of Surgery, Section of Cardiac and Thoracic Surgery, University of Chicago, Chicago, Illinois
b Division of Cardiology, Childrens Hospital, San Diego, San Diego, California
c Scripps Clinic & Foundation, La Jolla, California
Accepted for publication July 10, 2006.
* Address correspondence to Dr Bichell, University of Chicago, Section of Cardiac and Thoracic Surgery, 5841 S Maryland Ave, MC 5040, Chicago, IL 60637. (Email: dbichell{at}uchicago.edu).
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Abstract
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Distortion of septal and atrial anatomy in Ebsteins anomaly places the atrioventricular node and His bundle at risk for injury at operation. We present a novel technique for creating a robust neo-annulus, remote from conduction tissue, adding to the armamentarium of techniques available for the reconstruction or replacement of the tricuspid valve. Three cases are described. All the patients were in sinus rhythm, with competent native or bioprosthetic valves, at short-term follow-up.
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Introduction
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Apical displacement of the septal leaflet in Ebsteins anomaly poses challenges to surgical repair or replacement, among them protection of the atrioventricular node (AVN) and His bundle. Sutures across the septal portion of the true annulus traverse the area of the His bundle when there is no leaflet, annulus, or nonmuscular tissue to engage the sutures. Strategies to avoid damage to the conduction system of the heart include placement of sutures posterior to the AVN [1], supraannular suture placement, draining the coronary sinus to the right ventricle [2], and obliteration of the tricuspid valve toward a univentricular palliation [3].
Subannular sutures within the muscle of the septum or pressure from a sewing ring on the subjacent septum can cause partial or complete heart block, even when the AVN is successfully circumnavigated. Supraannular approaches have the disadvantage of placing the coronary sinus on the ventricular side, possibly affecting coronary circulation, and may promote pressure injury from a prosthesis. We describe a method for the reconstruction of the septal portion of the tricuspid annulus that provides an armature to support a repair or replacement, remote from the AVN and His bundle and elevated from the septum, to prevent prosthesis pressure injury to the conduction system.
A patch of 0.8-mm polytetrafluoroethylene (PTFE) (W. L. Gore & Associates, Inc, Flagstaff, Ariz) is cut to a triangular shape defined by the area delimited by the ideal anteroseptal commissure, the ideal posteroseptal commissure, and a point lateral to the tendon of Todaro. One edge is sculpted to conform to the contour of the coronary sinus (Fig 1A). The patch is sewn to the surface of the atrium to cover the area of the AVN and His bundle entirely, with its free edge constituting a new posterior annulus that floats above the true annulus; thus, all sutures and the PTFE neo-annulus remain remote from the area of the AVN (Fig 1B). The coronary sinus remains committed to the right atrium. Repair sutures or sutures to attach the sewing ring of a prosthetic valve are passed through the neo-annulus, which is contiguous with the native anterior true annulus (Fig 1C). The patch can be designed to create an annulus of any desired dimension, according to the goals of the repair or replacement.

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Fig 1. (A) Depiction of Ebsteins anomaly, with sail-like anterior leaflet of the tricuspid valve (TV) and absent or severely displaced septal leaflet. The relationships of the atrioventricular node (AVN), and the coronary sinus (CS), are indicated. Superior vena cava (SVC), and inferior vena cava (IVC) are shown for orientation. (B) Patch of polytetrafluoroethylene tailored to form a new septal annulus, attached to the atrium remote from the area of the AVN. Accommodation for the CS is made by placing a notch in the patch. (C) Tailored patch in place, defining a new septal annulus and an armature, onto which leaflet advancement is performed, attached at the plane of the reconstructed annulus. All suture material is remote from the AVN and His bundle.
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Case Reports
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Patient 1
The patient is a 13-year-old girl with Ebsteins anomaly, coarctation of the aorta. A coarctation repair was performed at 1 week of age. Surgical intervention was made at age 13 for severe tricuspid regurgitation (TR) and interval right heart enlargement. At operation, the septal leaflet was severely displaced, with tight septal attachments. A leaflet detachment was performed, with re-advancement, transitioning the leaflet reattachment onto a septal PTFE neo-annulus to complete the repair. Where leaflet was attached to PTFE, the suture line was reinforced with an autologous pericardial strip. Intraoperative testing revealed a competent valve and a postoperative echocardiogram demonstrated trace TR and no stenosis.
Four months later, the repair was intact, with 1+ TR by echocardiogram. At the 23-month follow-up examination, she was asymptomatic and taking no medications. An echocardiogram demonstrated normal right ventricular size and function, and 1 to 2+ (mild) TR.
Patient 2
The patient is a 16-year-old boy with Ebsteins malformation and Wolff-Parkinson-White syndrome. He underwent a catheter-based ablation of a right posterolateral accessory pathway at age 11 for symptomatic supraventricular tachycardia. No subsequent evidence of residual accessory pathways had been demonstrated, but his exercise tolerance had been progressively compromised, as serial echocardiograms revealed severe TR and cardiomegaly. The septal leaflet was displaced into the apical one third of the septum, with negligible mobility.
An operative repair was performed at age 14, with annuloplasty and leaflet advancement. Competent in early follow-up, progressive TR evolved to severe, with cardiomegaly and exercise intolerance. At age 16, reoperation revealed a deficient anterior leaflet. A 27-mm porcine bioprosthetic valve replacement was commenced. Valve sutures were affixed to a PTFE neo-annulus, transitioning onto the true annulus anteriorly.
The patient had an uneventful 4-day hospital stay. At echocardiogram 6 months after the procedure, the prosthesis was competent, and ventricular size was normal. At the 16-month follow-up, he was asymptomatic, taking no medications, and had a normal cardiac silhouette. He remains in normal sinus rhythm.
Patient 3
A 41-year-old man with severe Ebsteins malformation, presented with dyspnea and palpitations. An accessory pathway in the posterior septal region of the atrialized ventricle was cryoablated by catheter for orthodromic reentrant tachycardia. By echocardiogram, TR was severe, and a septal leaflet was displaced to the apex of the right ventricle.
At operation, the support to the anterior leaflet was muscular, no secondary chordae were divisible, and there was no functional septal leaflet. A PTFE septal neo-annulus was constructed, and a 33-mm porcine bioprosthesis was seated onto the resultant annulus. The right ventricle was plicated, and cryoablation lesions were placed empirically.
The patients hospital course was prolonged by episodic self-limited supraventricular tachycardia. He was prescribed metoprolol and discharged in sinus rhythm on postoperative day 6. An echocardiogram at 5 months demonstrated a well-seated bioprosthetic valve with trace regurgitation. Results of treadmill and Holter examinations demonstrated no arrhythmia.
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Comment
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This approach to tricuspid repair avoids the placement of any suture material in the muscle surrounding the AVN and His bundle, while providing a robust neo-annulus onto which to attach the anterior leaflet or sewing ring. This technique permits the preservation of coronary sinus drainage into the right atrium. Short-term follow-up is encouraging in the three reported cases.
Limitations to this report include the small number of patients and the short follow-up. It will be important to track the durability of a tricuspid repair where the hinge point of the leaflet is an interface between leaflet and PTFE. In addition, the reported patients are all teenagers or older, and no accounting for how this technique might apply in the infant or child is suggested.
The placement of the PTFE neo-annulus covers the area of the septal annulus, and although protecting from surgical injury to the conduction system, it could limit access to that region in catheter-based ablations of adjacent pathways. Patient 3 underwent preoperative mapping and catheter-based ablations as well as direct cryoablation of reentrant pathways concomitant with the tricuspid procedure, anticipating that surgery might obscure access for later ablations.
Ebsteins anomaly of the tricuspid valve presents a spectrum of disease, and no single approach can be applied to all patients. When septal leaflet displacement is severe, the conduction system may be at particular risk when seating a valve prosthesis or advancing the anterior leaflet against the septal portion of the true annulus. This technique for constructing a neo-annular armature of PTFE is simple, remote from the atrioventricular node and His bundle, and holds promise as a durable addition to the armamentarium of surgical techniques.
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References
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- Barnard CN, Schrire Y. Surgical correction of Ebsteins malformation with a prosthetic tricuspid valve Surgery 1963;54:302.[Medline]
- Kiziltan HT, Theodoro DA, Warnes CA, OLeary PW, Anderson BJ, Danielson GK. Late results of tricuspid valve replacement in Ebsteins anomaly Ann Thorac Surg 1998;66:1539-1545.[Abstract/Free Full Text]
- Starnes VA, Pitlick PT, Berstein D, Griffin ML, Choy M, Shumway NE. Ebsteins anomaly appearing in the neonateA new surgical approach. J Thorac Cardiovasc Surg 1991;101:1082-1087.[Abstract]