Asian Cardiovasc Thorac Ann 1999;7:59-61
© 1999 Asia Publishing EXchange Pte Ltd
Right Atrial Myxoma Originating from Tricuspid Septal Leaflet
C Levent Birincio
lu, MD,
A Tulga Ulus, MD,
S Fehmi Katirciolu, MD,
Birol Yamak, MD,
Arzum Kale, MD,
Gülden Aydog, MD,
Ouz Ta
demir, MD
Department of Cardiovascular Surgery Türkiye Yüksek  htisas Hospital Sihhiye, Ankara, Turkey
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For reprint information contact: C Levent Birinciolu, MD Tel: 90 312 310 3080 Fax: 90 312 466 3202 email: ulus{at}escortnet.com Department of Cardiovascular Surgery, Türkiye Yüksek htisas Hospital, Sihhiye, Ankara 06100, Turkey.
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Abstract
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A unique case of right atrial myxoma originating from the septal leaflet of the tricuspid valve is described. The tumor was detected by echocardiography and resected along with part of the septal leaflet, followed by primary repair.
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Introduction
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Right atrial myxomas are rare, being three to four times less frequent than those occurring in the left atrium and usually arising from the atrial septum in the region of the fossa ovalis. Cardiac myxoma usually presents as a benign pedunculated tumor that can be resected with relative ease. However, it has been reported that these tumors sometimes exhibit malignant behaviour.1–4 We recently encountered a case of right atrial myxoma originating from the septal leaflet of the tricuspid valve, which to the best of our knowledge has never been reported previously.
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Case Report
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A 13-year-old girl was referred to our hospital because of dyspnea and palpitations during the previous 12 months. There was a history of postural hypotension and syncope in the supine position. Physical examination revealed a blood pressure of 110/60 mm Hg, a heart rate of 80 beats•min–1, and the patient was afebrile. Auscultation detected normal first and second heart sounds and a grade 4/6 holosystolic murmur at the left sternal border; the lungs were clear. She had a 2-cm hepatomegaly without pain. There was no jugular venous dilation. The electrocardiogram showed normal sinus rhythm. Cardiomegaly was not seen on chest radiography. Her hematocrit was 37.9%, her hemoglobin level was 126 g•L–1, and platelet and white cell counts were within normal ranges. The erythrocyte sedimentation rate was 48 mm•h–1. Arterial blood gas analysis revealed mild hypoxia, pH 7.42, partial pressure of oxygen 80 mm Hg, partial pressure of carbon dioxide 37 mm Hg, and bicarbonate 23.7 mEq•L–1. Immunologic examinations detected no abnormality except for elevation of antistreptolysin O to 263 lU•mL–1 (normal range, 0.00 to 200 lU•mL–1). Cultures of both arterial and venous blood were negative. Echocardiography showed normal left ventricular size and systolic function, dilation of the right atrium, and a large lobulated mass in the right atrium prolapsing into the right ventricle during diastole (Figure 1A and 1B
). Mild tricuspid regurgitation and a patent foremen ovale were observed. In view of the presence of a large lobulated mass, urgent surgical intervention was undertaken.
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Figure 1. Echocardiography in apical four-chamber view demonstrating a large mass prolapsing into the right ventricle (A) during diastole and (B) moving back into the right atrium during systole.
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The patient underwent a median sternotomy and the right atrium, right ventricle, and the venae cavae were examined by surface echocardiography. This indicated that both venae cavae were suitable for cannulation so a right-angled cannula was passed directly into the vena cava, avoiding the right atrium. After institution of cardio-pulmonary bypass, a right atriotomy was performed. A 6 x 5-cm nonuniform myxomatous mass was found with a pedicle attached to the septal leaflet of the tricuspid valve and prolapsing into the right ventricle during diastole. The other leaflets of the valve were intact. A patent foremen ovale was seen in the interatrial septum. The myxoma was excised with part of the septal leaflet that was than repaired primarily. The tricuspid valve was assessed by filling the right ventricle with saline solution. Additional reconstructive procedures on the tricuspid valve, such as patching, were not necessary. Transeso-phageal echo-cardiography showed that there was minimal tricuspid insufficiency. The patent foremen ovale was closed primarily. There was no complication in the postoperative period and the patient made a good recovery.
The excised myxoma, measuring 6 x 5 cm, was smooth and lobulated with a soft friable gelatinous appearance and with hemorrhagic areas seen on the cut surface. On histologic examination, the mass was composed of an abundant extracellular myxoid matrix containing small polygonal stellate spindle cells (Figure 2). There were small blood vessels, areas of hemosiderin-laden macrophages, and other chronic inflammatory cells (Figure 3).
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Figure 2. Histologic appearance of the myxoma. The nests of small polygonal cells were widely spaced by abundant myxoid stroma (hematoxylin and eosin stain, original magnification x100).
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Figure 3. The tumor had stellate spindle cells and chronic inflammatory cells (hematoxylin and eosin stain, original magnification x400).
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Discussion
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Myxomas are usually benign but life-threatening symptoms may develop as they enlarge. Diagnosis of the tumor is usually not made until the patient has significant symptoms. Atrial myxomas are typically solitary pedunculated tumors that arise from the fossa ovalis.5 On diagnosis, urgent surgery is indicated because of the risk of embolic events or destruction of a cardiac valve. Myxomas may produce symptoms of hemodynamic derangement due to obstruction of flow within the cardiac chambers or deformation of a valve with resultant incompetence. Symptoms may be caused by embolization and less commonly, there may be constitutional symptoms. There are reports of embolic events involving the brain, spleen, kidney, finger, aorta, as well as the coronary, iliac, and mesenteric vessels, which led to a diagnosis of atrial myxoma.6–8
Forty-seven patients with left atrial myxoma underwent surgery in the cardiovascular surgery clinic at Türkiye Yüksek Ihtisas Hospital between 1971 and 1998. The myxomas arose from the interatrial septum in 45 patients, from the anulus of the posterior leaflet of the mitral valve in one patient, and from the free wall of the left atrium in one patient. Preoperatively, all patients had dyspnea or tachycardia. Clinical evidence of systemic embolism was detected in 9 patients. The diagnosis was established by angiocardiography in 6 patients and by echocardiography in 41 patients. All patients were treated surgically under cardiopulmonary bypass and the myxomas were resected along with their atrial attachments. The iatrogenic septal defects were closed by a patch in 12 patients and primarily in 34 patients. In addition, 3 patients with right atrial myxomas underwent surgery during this period. Two of these tumors originated from the fossa ovalis; the other one is the subject of this case study.
Review of the literature revealed only 2 case reports of a calcified right atrial myxoma that destroyed the tricuspid valve requiring replacement with a prosthetic valve.1,2 Our patient had a myxomatous mass with a pedicle attached to the septal leaflet of the tricuspid valve. However, it did not cause total destruction of the tricuspid valve. Transesophageal echocardiography was extremely useful in identifying the myxoma, its point of attachment, and the anatomy of the tricuspid valve.
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References
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Abstract
Introduction
