Ann Thorac Surg 1999;67:1803-1804
© 1999 The Society of Thoracic Surgeons
Case Reports
Tricuspid valve myxoma in a pediatric patient: 10-year follow-up after resection
Manoj Agny, MDa,
Mark Reller, MDa,
Adnan Cobanoglu, MDa
a Division of Cardiopulmonary Surgery, Oregon Health Sciences University, Portland, Oregon, USA
Accepted for publication November 19, 1998.
Address reprint requests to Dr Cobanoglu, Division of Cardiopulmonary Surgery, Oregon Health Sciences University, 3181 SW Sam Jackson Park Rd, L353, Portland, OR 97201-3098
e-mail: Cobanogl{at}ohsu.edu
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Abstract
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Cardiac myxomas are rarely encountered in pediatric patients. Tricuspid valve involvement in these cases is even more exceptional. We report the case of a 5-year-old girl operated on successfully 10 years ago for a tricuspid valve myxoma who continues to be asymptomatic and had an event free outcome.
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Introduction
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Primary cardiac valve tumors are extremely rare. Involvement of any part of the tricuspid valve apparatus is very uncommon. We describe the case of a 5-year-old girl with a myxoma arising from the septal leaflet of the tricuspid valve. She was operated on successfully in 1988. Her follow-up clinically and by serial echocardiograms since then reveals an optimal outcome.
In 1986, at 3 years of age, the patient was evaluated for fever and a new diastolic cardiac murmur. Chest x-ray and echocardiogram were normal and the child was presumed to have otitis media and was treated with amoxicillin. She returned 1 month later with recurrent fever and was noted to have a gallop rhythm. Two-dimensional echocardiography revealed an echo-dense lesion, measuring 1.4 cm2 on the septal leaflet of the tricuspid valve. There was no evidence of tricuspid stenosis or regurgitation. The lesion was interpreted as being a vegetation, given the history of recurrent fever and persistent cardiac murmur. The possibility of a tumor of the tricuspid valve was also entertained. Blood cultures done on eight different occasions were negative. The cause of this vegetation was believed to be endocarditis, which had presumably responded to amoxicillin. The child was discharged and echocardiograms were done at 3-month intervals. In October 1988, the patient was evaluated again for recurrent fever and lethargy. On clinical examination, a new soft systolic murmur was noted. The electrocardiographic findings were unchanged, but a chest roentgenogram was suggestive of a right atrial enlargement. A two-dimensional echocardiogram (Fig 1) revealed no change in the tricuspid valve lesion; however, on Doppler study mild to moderate tricuspid regurgitation was noted. Based on this new development of tricuspid regurgitation, excision of the tricuspid valve lesion was planned.
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Fig 1. Preoperative (1988) two-dimensional echocardiogram (apical four-chamber view): demonstrating the tricuspid valve mass (arrow) involving the septal leaflet. (LA = left atrium; LV = left ventricle; RA = right atrium; RV = right ventricle.)
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A bicaval cannulation was used. Right atriotomy was done, which revealed a large polypoidal, pedunculated mass measuring 2 x 2 cm and arising from the free margin of the septal leaflet. On gross examination it looked more like a tumor than a vegetation. Complete excision of the mass necessitated removal of the middle third of the septal leaflet and detachment of three chordae tendinae from the free margin of the leaflet. Leaflet reconstruction was done using a U-shaped autologous pericardial patch. Severed chordae were reattached to the free margin of the pericardial patch. A Wooler type of annuloplasty [1] was also done. Valve competency was tested and found to be optimal. Frozen section of the mass was consistent with the diagnosis of a myxoma. This was subsequently confirmed by histopathologic examination, which revealed a nodular and papillary fragment of whitish smooth tissue. The child had an uneventful recovery. An echocardiogram obtained before discharge showed normal tricuspid valve motion and function, with only a minimal degree of tricuspid regurgitation. The child was followed up at 3-month intervals for the first year and yearly thereafter. All findings at these evaluations were normal. On her most recent visit in April 1998, the patient was totally asymptomatic. The echocardiogram (Fig 2) revealed a normal tricuspid valvular motion and function, with trivial tricuspid regurgitation.
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Fig 2. Late follow-up (1998) two-dimensional echocardiogram (apical four-chamber view) demonstrating a mass-free septal leaflet of the tricuspid valve. Arrow indicates pericardial patch used for leaflet reconstruction. Abbreviations as in Figure 1.
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Comment
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Myxomas are the most commonly encountered cardiac tumors and are usually seen in the left atrium. Primary cardiac valvular tumors are rarely seen, accounting for less than 10% of all cardiac tumors; of these, myxomas constitute less than 10% of the total as noted by Edwards and associates [2]. Although myxomas are more frequently encountered on the left side of the heart, valvular involvement is more common on the right side [3]. Even so, myxomas of the tricuspid valve are exceptional, with only 16 cases reported to date [3, 4]; of these only 4 were in pediatric patients. In our patient, we thought that leaflet reconstruction was necessary for two reasons. First, because the septal leaflet is attached to the right fibrous skeleton, primary closure after resection of one third of the leaflet in this location, is not feasible and almost invariably reconstruction with a patch material is required. Second, severed chordae needed to be reattached in the proper anatomic alignment, and for this the use of a pericardial patch was deemed necessary. Most tricuspid valve myxomas arise from the septal leaflet and can be excised without valve replacement. Kinugasa and colleagues [5] reported a myxoma arising from the anterior papillary muscle of the tricuspid valve, which, was excised and the valve repaired by chordoplasty and annuloplasty. The follow-up revealed no recurrence or tricuspid regurgitation. A similar follow-up was reported by Almdahl and associates [6], who operated on a 62-year-old man. Both of these reports represent long-term results. Identical findings in our follow-up further substantiate the need for a radical excision to avoid recurrence. The pedunculated nature of the lesion makes this feasible. Parker and associates [7] reported sudden death due to tricuspid valve myxoma with massive myxomatous pulmonary embolism in a 15-month-old patient. Their outcome suggests that a high index of suspicion be warranted for a mass on the tricuspid valve, as possibly being myxomatous. Our follow-up of 10 years assures us that a radical, valve preserving excision of a tricuspid valve myxoma can give an excellent and sustained cure.
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References
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Wooler G.H., Nixon P.G., Grimshaw V.A., Watson D.A. Experiences with the repair of mitral valve in mitral incompetence. Thorax 1962;17:49-57.
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Edwards F.H., Hale D., Cohen A., Thompson L., Pezzella T., Virmani R. Primary cardiac tumors. Ann Thorac Surg 1991;52:1127-1131.[Abstract]
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Kuroda H., Nitta K., Ashida Y., Hara Y., Ishiguro S., Mori T. Right atrial myxoma originating from the tricuspid valve. J Thorac Cardiovasc Surg 1995;109:1249-1250.
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Pessoto R., Santini F., Piccin C., Consolaro G., Faggian G., Mazzucco A. Cardiac myxoma of the tricuspid valve: description of a case and review of literature. J Heart Valve Dis 1994;3:344-346.[Medline]
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Kinugasa S., Asada K., Kodama T., et al. Right ventricular myxoma: case report. Surg Today 1997;27:269-271.[Medline]
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Almdahl S.M., Myrmel T., Gjestvang F.T. Surgical treatment of a tricuspid valve myxoma. Tidsskr Norske Laegeforen 1996;116:2175.
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Parker K.M., Embry J.H. Sudden death due to a tricuspid valve myxoma with massive pulmonary embolism in a 15-month-old male. J Forensic Sci 1997;42:524-526.[Medline]
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Abstract
Introduction
