Ann Thorac Surg 1995;59:320-322
© 1995 The Society of Thoracic Surgeons
Unstented Semilunar Homograft Replacement of Tricuspid Valve in Ebstein's Malformation
Naresh Kumar, FRCS,
Ricardo Gallo, MD,
Zohair Al-Halees, FRCS(C),
Fadel Al-Fadley, FRCP,
Carlos M. G. Duran, MD, PhD
Department of Cardiovascular Diseases, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia
Accepted for publication July 27, 1994.
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Abstract
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Tricuspid valve pathology in Ebstein's malformation requires replacement when it is not possible to repair or reconstruct this valve. In smaller children, in whom the right-sided atrioventricular valve is severely dysplastic and right ventricular volume is prohibitive, prosthetic replacement is not always possible. We report here on 3 patients who underwent stentless semilunar homograft replacement (top-hat procedure) of tricuspid valve for Ebstein's anomaly with good short-term outcome. This provides an attractive alternative in the management of a certain difficult subset of patients, avoids long term anticoagulation and probably is more durable.
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Introduction
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Ebstein's malformation, characterized by downward displacement of septal or posterior tricuspid valve leaflets (or both), is a rare congenital anomaly [1]. The tricuspid valve is often incompetent but is sometimes stenotic. This valve pathology together with the atrialized right ventricle, atrial septal defect (ASD), and ventricular septal defect (VSD), which often are associated, produce right-sided failure, arrhythmias, and cyanosis.
We report on 3 patients with Ebstein's anomaly who underwent stentless semilunar homograft replacement (top-hat procedure) of the tricuspid valve for management of their tricuspid valve pathology.
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Case Reports
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Patient 1
A 3-year-old female child with a history of cyanosis since birth was diagnosed by echocardiogram as having VSD and ASD with moderately severe tricuspid valve regurgitation due to Ebstein's malformation of the valve. The child underwent closure of the VSD and ASD with replacement of the tricuspid valve using a pulmonary homograft placed inside Dacron tubing with a pericardial skirt on the atrial side (top-hat procedure). The child had no immediate tricuspid regurgitation according to transesophageal echocardiogram after homograft implantation, had an uneventful hospital recovery, and was doing well at last follow-up, 1 year after the operation.
Patient 2
A 6-year-old female child with echocardiographic and catheter diagnosis of Ebstein's malformation of the tricuspid valve with moderately severe regurgitation and ASD with cyanosis and elevated hemoglobin was referred for operation. The tricuspid valve was replaced with an aortic homograft placed inside a 26-mm Gelseal tube and the ASD was closed with a pericardial patch. She had an uneventful recovery and is doing well 6 months after the operation with no echocardiographic evidence of tricuspid regurgitation.
Patient 3
A 7-month-old male child with a diagnosis of ASD/VSD and Ebstein's malformation of the tricuspid valve with severe regurgitation was referred for operation. He underwent closure of the ASD and the VSD with an attempted repair of the tricuspid valve with plication of the atrialized portion of the ventricle and annulus stabilization. Passive testing showed only minimal regurgitation and this was accepted. In the immediate postoperative period he did well and was extubated within 24 hours, but on the second postoperative day he went into low cardiac output state and required reintubation and inotropic agent support. He also went into acute oliguric renal insufficiency, requiring temporary peritoneal dialysis. Transesophageal echocardiogram showed severe tricuspid valve insufficiency and he was reoperated on the third postoperative day. The tricuspid valve was replaced with a 21-mm aortic homograft in a 26-mm Dacron tube. The chest was initially left open, but it was closed after attaining hemodynamic stability 3 days after the second operation. The child recovered from renal insufficiency and had an otherwise uneventful convalescence in the hospital. He is doing well 2 months after the operation, with no echocardiographic evidence of tricuspid regurgitation or right-sided failure symptoms.
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Operative Technique
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All 3 children underwent operation using standard cardiopulmonary techniques with moderate systemic hypothermia and antegrade cold blood cardioplegia for myocardial protection. The VSD was closed with interrupted pledgeted sutures and bovine pericardial patch. The roof of the coronary sinus was cut back, leaving it open to the left atrium. The ASD was then closed with a patch. Based on the echocardiographic analysis, an appropriately sized aortic homograft was prepared by placing it inside a Dacron tube, the caliber of which was a few millimeters larger than the widest part of the homograft. The distal end of the homograft was sewn to the wall of the Dacron tube and the proximal end of the homograft was incorporated in a suture line with a wide skirt of bovine pericardium and the Dacron tube. The tricuspid valve was trimmed and the distal end of the homograft prosthesis was sutured to the annulus and the valve remnants, taking care to prevent injury to the conduction tissue. The pericardial skirt was then sutured to the floor of the right atrium, avoiding the inflow of both cavae and forming a smooth transition to the homograft inlet. Care was taken to avoid torsion to the Dacron tube, as this would lead to incompetence of the homograft (Fig 1
).
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Fig 1. . Echocardiogram of unstented aortic homograft inside a Dacron tube in tricuspid position (left) in systole and (right) in diastole. (RA = right atrium; RV = right ventricle.)
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Comment
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Ebstein's malformation with its protean anatomy still remains a challenge to the surgeon. Various operations, including tricuspid valve replacement [2–4], valve repair techniques [5, 6], and right heart reconstructive techniques [7, 8] have been used to correct this anomaly.
Although conservation of the tricuspid valve by repair techniques is preferable, it is not always possible. Carpentier and colleagues [7] and Danielson and Fuster [8] have tried to classify this difficult malformation based on the surgical anatomy and have described repair techniques to reconstruct the right ventricle and the tricuspid valve. The severity of the malformation sometimes requires replacement of the tricuspid valve to alleviate right-sided regurgitation and failure.
The malformed tricuspid valve has been replaced with mechanical [3, 4], homograft [9, 10], and bioprosthetic [11] valves, with no apparent differences in intermediate or long-term outcome [12]. With the inherent problem of long-term anticoagulation, especially in growing children, for mechanical prosthesis and of accelerated calcific degeneration of the tissue valves in this age group, we chose to use a non-stented homograft valve. All 3 children were maintained on low-dose cyclosporine (0.5 mg • kg-1 • day-1) therapy [13] to obviate immunologic degeneration of the homograft. We postulate that these valves should last longer than stented homograft or allografts.
This operation provides a competent valve with a central laminar flow which requires no anticoagulation and can be performed safely, even in small children. The size of the homograft that can be accomodated allows for growth well into adolescence and early adulthood without becoming stenotic. The prosthesis does not encroach onto the right ventricle and thus does not interfere with its function. The relocation of the coronary sinus by unroofing it to the left atrium does not cause any major disturbances and can be achieved easily and safely.
We conclude that this procedure offers an elegant alternative to this rather distressing malformation, and we report that 3 of our patients, including a very young child had good short-term outcomes with this operation.
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Footnotes
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Address reprint requests to Dr Kumar, Department of Cardiovascular Diseases (MBC # 16), King Faisal Specialist Hospital, PO Box 3354, 11211 Riyadh, Saudi Arabia.
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References
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- Keith JO, Rowe RD, Vlad P. Heart disease in infancy and childhood. New York: Macmillan, 1958:314.
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- Bove EL, Kirsh MM. Valve replacement for Ebstein's anomaly of the tricuspid valve. J Thorac Cardiovasc Surg 1979;78:229–32.[Abstract]
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- Hardy KL, May IA, Webster CA, Kimball KG. Ebstein's anomaly: A functional concept and successful definitive repair. J Thorac Cardiovasc Surg 1964;48:927–40.
- Carpentier A, Chavaud S, Mace L, et al. A new reconstructive operation for Ebstein's anomaly of the tricuspid valve. J Thorac Cardiovasc Surg 1988;96:92–101.[Abstract]
- Danielson GK, Fuster V. Surgical repair of Ebstein's anomaly. Ann Surg 1982;196:499–504.[Medline]
- Senoo Y, Ohishi K, Nawa S, Teramoto S, Sunada T. Total correction of Ebstein's anomaly by replacement with a biological aortic valve without plication of the atrialized ventricle. J Thorac Cardiovasc Surg 1976;72:243–8.[Abstract]
- McKay R, Sono J, Arnold RM. Tricuspid valve replacement using an unstented pulmonary homograft. Ann Thorac Surg 1988;46:58–62.[Abstract]
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- Mair DD, Seward JB, Driscoll DJ, Danielson GK. Surgical repair of Ebstein's anomaly: Selection of patients and early and late operative results. Circulation 1985;72(Suppl 2):70–6.
- Clarke DR, Bishop DA. Allograft degeneration in infant pulmonary valve allograft recipients. Eur J Cardiothorac Surg 1993;7:365–70.[Abstract]
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Abstract
Introduction
