Ann Thorac Surg 2001;71:1350-1351
© 2001 The Society of Thoracic Surgeons
Case report
Cleft on tricuspid anterior leaflet
Naotaka Motoyoshi, MDa,
Motohisa Tofukuji, MD, PhDa,
Masahiro Sakurai, MDa,
Mikio Ohmi, MDa,
Koichi Tabayashi, MDa
a Department of Cardiovascular Surgery, Graduate School of Medicine, Tohoku University, Sendai, Japan
Accepted for publication April 24, 2000.
Address reprint requests to Dr Motoyoshi, Department of Cardiovascular Surgery, Graduate School of Medicine, Tohoku University, 1-1 Seiryo-machi, Aoba-ku, Sendai 980-8574, Japan
e-mail: naotaka5{at}hotmail.com
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Abstract
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We report a case of isolated congenital tricuspid regurgitation caused by a cleft in the anterior tricuspid leaflet associated with a patent foramen ovale. Preoperative echocardiography revealed severe tricuspid regurgitation resulting from anterior tricuspid leaflet prolapse. The patient underwent successful tricuspid valve repair with simple cleft suture and annuloplasty and direct closure of the patent foramen ovale.
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Introduction
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Pure congenital tricuspid regurgitation (TR) caused by a cleft is very rare, and only ten surgical cases with successful outcomes have been reported [1]. The embryological origin of the cleft on the tricuspid leaflet is not completely obvious, and this lesion can often be seen with atrial septal defect, atrioventricular block, and congestive heart failure. We present here the case of a patient with isolated congenital tricuspid regurgitation caused by a cleft in the anterior tricuspid leaflet.
A male infant was found to have TR and atrial septal defect shortly after birth. Physical examination revealed no other congenital abnormalities. He was observed for more than 20 years without apparent cardiac-related complaints, but recently was seen with dyspnea on exertion and palpitations despite treatment with digoxin and diuretics.
Physical findings included slight peripheral cyanosis and a grade 3/6 systolic murmur at the left sternal edge in the 4th intercostal space (4LSB) consistent with TR. Neither hepatomegaly nor peripheral edema was present. Electrocardiography revealed normal sinus rhythm with first-degree atrioventricular block, and chest roentgenography demonstrated a normal heart size. Echocardiography confirmed the diagnosis of solitary TR (Fig 1). Both the right atrium and the right ventricle were grossly enlarged with severe TR caused by anterior tricuspid leaflet prolapse and a dilated tricuspid annulus. It could not clearly be distinguished whether the chordae beneath the anterior leaflet were attached to the papillary muscle or not. Cardiac catheterization revealed grade 4/4 TR, pulmonary artery systolic pressure of 17 mm Hg (mean pressure, 7 mm Hg), and a mean pulmonary wedge pressure of 3 mm Hg (v wave, 3 mm Hg).
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Fig 1. Preoperative echocardiogram revealing pure tricuspid regurgitation, unlike Ebstein’s anomaly. (RA = right atrium; RV = right ventricle; TV = tricuspid valve.)
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At operation, the right atrium and the right ventricle were markedly dilated. A right atriotomy revealed a patent foramen ovale and severe TR resulting from anterior leaflet prolapse. No thickening of the leaflets was present. On the anterior leaflet there was a large cleft that effectively cut the leaflet into two dysfunctional smaller leaflets. Each one had a bundle of chordae attached to either the anterior papillary muscle or the conal papillary muscle. There was little tension on the chordae because the lateral deviation of each of the divided smaller leaflets caused prolapse (Fig 2). Few abnormalities of the chordae, papillary muscles, valve cusps, or sites of attachment of the leaflets were observed. A running suture was placed to close the cleft, and tricuspid annuloplasty was performed using a 35-mm Duran ring. No regurgitation was seen when the ventricle was filled with water or when transesophageal echocardiography was carried out after the patient was weaned from the pump. The large patent foramen ovale was closed directly.
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Fig 2. A cleft in the tricuspid anterior leaflet is a rare finding. In our patient, the anterior leaflet had a large cleft, which effectively cut the leaflet into two dysfunctional smaller leaflets.
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Extubation was accomplished in the operating room immediately after the operation. The patient was moved from the intensive care unit on the day after the operation and recovered without complications. Postoperative echocardiography showed trivial TR and a smaller right atrium than was present before surgical correction.
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Comment
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Solitary congenital TR is a rare finding. Becker and colleagues [2] reported that the anatomical operation for malformed tricuspid valves ranges from downward displacement of the basal valve attachment only through the combination of both downward displacement and dysplasia. They did not consider other anomalies of the tricuspid valve such as an additional leaflet, or clefts. The typical origins of isolated TR include myocardial fibrosis, endocarditis, trauma, myxoma, malformation, and a cleft tricuspid valve. Eichhorn and coworkers [3] first reported a 0.6% incidence of tricuspid valve cleft in patients with a diagnosis of congenital heart disease, and 5 patients (0.018%) with this anomaly were found through random sampling of 28,091 Doppler echocardiographic studies. In 50 patients, Silver and associates [4] found that an incomplete notch on the anterior leaflet was often seen but that the more complete (close to commissure zone) type was rare.
From the embryological standpoint, the tricuspid valve is formed from the inner wall of the right ventricle, a process referred to as undermining. Incomplete undermining makes the leaflet stick on the inner wall of the right ventricle, and dysfunctional, and it is a smaller leaflet. We call this malformation Ebstein’s anomaly when the anterior leaflet is seldom plaster and is often bigger like a curtain [5]. However, a tricuspid valve cleft originates from an anomalous type different from undermining. Carpentier and coauthors [6] called such a cleft or notch a "must-be-considered-normal anatomic variation." Eichhorn and colleagues [3] offered two possible explanations for this. First, all of the tricuspid valve cleft involved the anterior leaflet, which is close to the area where the lateral endocardial cushion meets the right dorsal conus swelling. Therefore, the cleft might be the result of an anomalous fusion. Our patient fits into this category because each of the divided leaflets had a bundle of chordae, which caused serious regurgitation. Second, the large variability in the anatomic structure of the tricuspid valve itself makes it difficult, even for the pathologist, to differentiate a cleft from additional valves.
Suturing the cleft seems appropriate for this type of anomaly. In our patient, we added annuloplasty with a Duran ring for a dilated annulus to prevent potential regurgitation caused by the enlarged right ventricle. Postoperative echocardiography revealed satisfactory results for this type of valve repair.
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References
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Katogi T., Aeba R., Ito T., et al. Surgical management of isolated congenital tricuspid regurgitation. Ann Thorac Surg 1998;66:1571-1574.[Abstract/Free Full Text]
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Becker A.E., Becker M.J., Edwards J.E. Pathologic spectrum of dysplasia of the tricuspid valve. Arch Pathol 1971;91:167-178.[Medline]
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Eichhorn P., Ritter M., Suetsch G., von Segesser L.K., Turina M., Jenni R. Congenital cleft of the anterior tricuspid leaflet with severe tricuspid regurgitation in adults. J Am Coll Cardiol 1992;20:1175-1179.[Abstract]
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Silver M.D., Lam J.H., Ranganathan N., Wigle E.D. Morphology of the human tricuspid valve. Circulation 1971;43:333-348.[Abstract/Free Full Text]
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Van Mierop L.H.S., Gessner I.H. Pathogenic mechanisms in congenital cardiovascular malformations. Prog Cardiovasc Dis 1972;15:67-72.[Medline]
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Carpentier A., Deloche A., McGoon D.C., et al. Surgical management of acquired tricuspid valve disease. J Thorac Cardiovasc Surg 1974;67:53-65.[Medline]
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Abstract
Introduction
