Ann Thorac Surg 1998;66:1571-1574
© 1998 The Society of Thoracic Surgeons
Surgical management of isolated congenital tricuspid regurgitation
Toshiyuki Katogi, MDa,
Ryo Aeba, MDa,
Tsutomu Ito, MDa,
Tetsuya Goto, MDa,
Yasunori Cho, MDa,
Toshihiko Ueda, MDa,
Shiaki Kawada, MDa
a Division of Cardiovascular Surgery, Keio University, Tokyo, Japan
Accepted for publication May 18, 1998.
Address reprint requests to Dr Katogi, Division of Cardiovascular Surgery, Keio University, 35 Shinanomachi, Shinjuku, Tokyo 160, Japan
e-mail: (katogi{at}mc.med.keio.ac.jp)
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Abstract
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Background. Isolated congenital tricuspid regurgitation without downward displacement of the leaflet is a rare clinical entity. Degenerative cusps and lack of chordae may preclude valvuloplasty and require valve replacement.
Methods. Three consecutive patients with isolated congenital tricuspid regurgitation underwent surgical repair between May 1995 and April 1997. Their ages were 23, 15, and 8 years old. Tricuspid valvuloplasty was feasible in all of them, with use of a gathering suture of the anterior leaflet, artificial chordae implantation, and ring annuloplasty.
Results. All 3 patients survived and recovered well after the operation. The cardiothoracic ratios on their chest roentgenograms decreased from 0.64 to 0.52 in patient 1, from 0.58 to 0.48 in patient 2, and from 0.60 to 0.44 in patient 3. Postoperative echocardiograms showed competent tricuspid valves and the disappearance of regurgitation in all cases.
Conclusions. Although malformation of the valve is extensive in isolated congenital tricuspid regurgitation, application of artificial chordae with conventional valvuloplasty technique can avoid the use of prosthetic valves by establishing the competence of the tricuspid valve.
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Introduction
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Congenital tricuspid regurgitation without downward displacement of the leaflet (ie, other than Ebstein’s anomaly) is a very uncommon cardiac lesion [1, 2]. If patients’ status is tolerable, they should be treated medically without surgery during infancy and childhood. The extensive nature of their valve abnormality may preclude valvuloplasty, and valve replacement is a procedure of choice. The natural history of congenital tricuspid regurgitation is unclear because of its rarity, but it may be considered the same as Ebstein’s anomaly [1, 3, 4].
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Material and methods
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Patients
From May 1995 to April 1997, 3 consecutive patients required operations for isolated congenital tricuspid regurgitation.
Patient 1
Patient 1 was a 23-year-old woman. Shortly after birth she had shortness of breath and failed to thrive. The diagnoses were tricuspid valve regurgitation and small atrial septal defect. After 6 months of medical treatment, her congestive heart failure disappeared and she grew up normally afterward. Although her cardiac status was stable except for a mild limitation on exercise, her cardiothoracic ratio gradually increased to 0.64. Echocardiography demonstrated massive tricuspid valve regurgitation with a huge right ventricle and a so-called pancakelike small left ventricle. With cardiac catheterization, the right ventricular end-diastolic volume index was 224.7 mL/m2 with an ejection fraction of 0.64. The left ventricular end-diastolic volume index was 36.1 mL/m2 with an ejection fraction of 0.72.
Patient 2
Patient 2 was a 15-year-old boy. He had deep cyanosis after birth, with respiratory distress. After 3 months of medical care, his cyanosis improved and he has grown up normally. His cardiac status was stable except for occasional supraventricular arrhythmia. His cardiothoracic ratio was 0.58 on chest roentgenogram. Echocardiography showed a huge right ventricle, small left ventricle, and massive tricuspid regurgitation without downward displacement of the septal leaflet. His right ventricular end-diastolic volume index was 140.1 mL/m2 with a right ventricular ejection fraction of 0.46, and his left ventricular end-diastolic volume index was 46.1 mL/m2 with a left ventricular ejection fraction of 0.31. His left ventricular function deteriorated, likely because of extreme enlargement of the right ventricle.
Patient 3
Patient 3 was an 8-year-old boy. He had deep cyanosis and congestive heart failure after birth and was referred to our hospital with a diagnosis of Ebstein’s anomaly. After 3 months of medical treatment, his cardiac failure was reversed, leaving moderate cyanosis. His growth was normal except that his peripheral arterial oxygen saturation was around 80%. His cardiothoracic ratio according to chest roentgenogram was 0.60. Echocardiography revealed a large right ventricle, small left ventricle, right to left shunting through an atrial septal defect, and massive tricuspid regurgitation without downward displacement of the valve. With cardiac catheterization, his right ventricular end-diastolic volume index was 136.5 mL/m2 with a right ventricular ejection fraction of 0.40, and the left ventricular end-diastolic volume index was 38.2 mL/m2 with a left ventricular ejection fraction of 0.69.
Operative technique
After a median sternotomy, standard cardiopulmonary bypass with bicaval and aortic cannulas was established. Under moderate hypothermia, the aorta was clamped and crystalloid cardioplegic solution was administered for myocardial protection. The right atrium was opened parallel to the atrioventricular groove. Inspection of the tricuspid valve revealed that the leaflets were very thin in all 3 patients. Medial and posterior papillary muscle were present, but the anterior papillary muscle was not observed in all 3 patients. The anterior and posterior leaflets were joined because of a lack of the anteroposterior commissure. Although the anteroseptal commissure and posteroseptal commissure were suspended by a few chordae, almost all components of the anterior and posterior leaflets had no chordae (Fig 1). Only the septal leaflet was suspended by scarce chordae. The tricuspid annulus were enlarged enormously: 50 mm in diameter in patient 1, 60 mm in patient 2, and 45 mm in patient 3. The redundant central portion of the anterior–posterior leaflet complex was secured by a gathering suture in a triangular fashion using 5–0 Prolene (Ethicon, Somerville, NJ) reinforced by autologous pericardium. Annuloplasty was done by a Carpentier–Edwards annuloplasty ring: 36 mm in patients 1 and 2 and 30 mm in patient 3. The competence of the valve was checked at this stage, but showed massive regurgitation because of an unsuspended anterior–posterior leaflet complex in each case. Double armed 4–0 expanded polytetrafluoroethylene suture was anchored to the trabecullar portion of the right ventricle, passed through the gathered and reinforced portion of the anteroposterior leaflet, and again anchored to the right ventricle. In total, four artificial chordae were implanted. The proper length of the artificial chordae was slightly less than that of the chordae of the septal leaflet. Confirmation of valve competence at this stage showed trivial or no regurgitation in every case. An atrial septal defect existed only in patient 3, and it was closed by direct suture.
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Fig 1. (a) Gathering plasty of anterior-posterior leaflet complex with continuous suture of 5–0 Prolene reinforced by autologous pericardial strip. (b) Implantation of artificial chordae of expanded polytetrafluoroethylene suture. (c) Completed procedures with Carpentier–Edwards ring, gathering valvuloplasty, and artificial chordae.
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Results
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The postoperative course was uneventful in each patient. Chest roentgenograms at discharge showed a remarkable decrease in cardiothoracic ratio: from 0.64 to 0.52 in patient 1, from 0.58 to 0.48 in patient 2, and from 0.60 to 0.44 in patient 3 (Fig 2). Postoperative echocardiograms revealed good coaptation with no regurgitation of the tricuspid valve and a remarkable decrease in the size of the right ventricle (Fig 3). All patients are in New York Heart Association functional class I, 6 months to 2 years postoperatively.
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Fig 2. (A) Preoperative chest roentgenogram of patient 3. (B) Postoperative chest roentgenogram of the same patient.
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Fig 3. (A) Preoperative echocardiogram shows prolapsed anterior leaflet of the tricuspid valve, and dilatation of the right atrium and right ventricle. (B) Postoperative echocardiogram shows competent tricuspid valve and decrease in the size of right heart chambers and increase in the size of left heart chambers.
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Comment
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Isolated congenital tricuspid regurgitation is a rare clinical entity and should be distinguished from Ebstein’s anomaly. These two groups may be clinically similar but are anatomically different. The prominent features of Ebstein’s anomaly are downward displacement of the septel leaflet and the existence of an atrialized right ventricle [3, 5]. In isolated congenital tricuspid regurgitation, basal attachment of the tricuspid valve leaflets is of a normal level, an atrialized right ventricle does not exist, and the integrity of the muscular structure of the right ventricle is preserved. Aaron and colleagues [1] described the deformity of the tricuspid valve in this entity as follows: (1) focal or diffuse thickening of the valve leaflets; (2) deficient development of the chordae tendineae and papillary muscles, most often binding down or tethering the valve margin; (3) improper separation of valve components from the ventricular wall; and (4) focal agenesis of valvular tissue. Our 3 patients showed all of these features except thickening of the leaflet. The leaflets of our patients were thin and flimsy and the chordae were also thin and weak. Anterior and posterior leaflets normally are not separated and consist of a large cusp opposite the septal leaflet. Because most of this leaflet was not suspnded by chordae tendineae, valvuloplasty using the conventional technique is inadequate to form a competent tricuspid valve. It was thought to be unsuccessful to transfer the chordae from the septal leaflet because those chordae were thin and weak in these patients. The gathering suture reinforced by autologous pericardium at the center of anterior and posterior leaflet continuity offers an ideal site for chordae insertion because it is also the center of the unsupported area. Although the use of expanded polytetrafluoroethylene sutures for artificial chordae in the mitral position is an established technique [6, 7], its application to tricuspid valve was reported only by Zussa in 1996 [8]. Our experience demonstrates that implantation of artificial chordae is very effective in patients with this anomaly. Patients get great benefit by avoiding artificial valve replacement. Our patients showed uneventful recoveries, the volume of the right ventricle decreased dramatically, and their tricuspid valves are functioning well up to 2 years later. Although our patients’ results are promising, the long-term fate of artificial chordae is not yet clear, especially in the tricuspid position. We have to pay close attention to the function of the valves in these patients.
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Acknowledgments
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We thank Dr Katsuhisa Onoguchi for his artistic help with Figure 1.
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References
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- Aaron B.L., Mills M., Lower R.R. Congenital tricuspid insufficiency: definition and review. Chest 1976;69:637-641.[Abstract/Free Full Text]
- Yokoyama S., Akimoto T., Takemura T., Harada Y. A surgical case report of isolated congenital tricuspid valve insufficiency. J Jpn Assn Thorac Surg 1995;43:1063-1068.
- Becker A.E., Becker M.J., Edwards J.E. Pathologic spectrum of dysplasia of the tricuspid valve. Arch Pathol 1971;91:167-178.[Medline]
- Celermajer D.S., Bull C., Till J.A., et al. Ebstein’s anomaly: presentation and outcome from fetus to adult. J Am Coll Cardiol 1994;23:170-176.[Abstract]
- Quaegebeur J.M., Sreeram N., Fraser A.G., et al. Surgery for Ebstein’s anomaly: the clinical and echocardiographic evaluation of a new technique. J Am Coll Cardiol 1991;17:722-728.[Abstract]
- Zussa C., Polesel E., Rocco F., Galloni M., Frater R.W.M., Valfré C. Surgical technique for artificial mitral chordae implantation. J Cardiol Surg 1991;6:432-438.
- Zussa C., Polesel E., Da Col U., Galloni M., Valfré C. Seven-year experience with chordal replacement with expanded polytetrafluoroethylene in floppy mitral valve. J Thorac Cardiovasc Surg 1994;108:37-41.[Abstract/Free Full Text]
- Zussa C. Different applications of ePTFE valve chodae: surgical technique. J Heart Valve Dis 1996;5:356-361.[Medline]
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Abstract
Introduction
