Ann Thorac Surg 2001;71:1677-1679
© 2001 The Society of Thoracic Surgeons
Case report
Cardiac papillary fibroelastoma on the pulmonary valve: a rare cardiac tumor
Kenji Okada, MDa,
Taijiro Sueda, MDa,
Kazumasa Orihashi, MDa,
Masanobu Watari, MDa,
Yuichiro Matsuura, MDa
a First Department of Surgery, Hiroshima University School of Medicine, Hiroshima, Japan
Accepted for publication June 12, 2000.
Address reprint requests to Dr Okada, First Department of Surgery, Hiroshima University School of Medicine, 1-2-3 Kasumi, Minami-ku, Hiroshima, 734-8551, Japan
e-mail: okaken{at}mcai.med.hiroshima-u.ac.jp
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Abstract
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We report the case of a patient with a rare papillary fibroelastoma on the pulmonary valve visualized before surgical intervention. The tumor was an encapsulated, rounded mass. The gelatinous membrane on the surface tore easily, and multiple fronds appeared. This case emphasizes that when there is an encapsulated mass attached to a valve, the initial excision of valve tissue should be as minimal as possible. To avoid unnecessary injury to the valve, it is simple and practical to confirm that the tumor has the appearance of a sea anemone, thus identifying it as a papillary fibroblastoma, a benign tumor.
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Introduction
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Papillary fibroelastoma (PFE) is a rare cardiac tumor frequently formed from valve endocardium [1]. Such tumors have often been described on the left side of the heart in association with embolism, but PFEs on the right side have typically been discovered as incidental findings at postmortem examination. We describe the case of a patient in whom a PFE on the pulmonary valve was visualized before surgical intervention.
A 71-year-old woman had had occasional slight fever and effort dyspnea during the preceding year. Chest radiography demonstrated a bronchitislike shadow in the right lung. Computed tomography revealed a thumb-sized mass in the pulmonary trunk. Subsequently the diagnosis of a cardiac tumor was made, and the patient was referred to our hospital.
On admission, body temperature was 37.1°C, and auscultation revealed no heart murmur. Transesophageal echocardiography demonstrated a heterogeneous mobile mass in the pulmonary trunk. Computed tomography of the chest revealed a solitary rounded mass 20 mm in diameter that had an irregular pattern and no contrast effect (Fig 1).
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Fig 1. Preoperative computed tomographic scan demonstrating a round shape with an irregular pattern in the pulmonary artery trunk.
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After a median sternotomy, cardiopulmonary bypass was instituted. The ascending aorta was cross-clamped, and cardiac arrest was achieved. A vertical incision was made in the pulmonary trunk. Special attention was paid to avoid damage to the pulmonary valve and to minimize manipulation of the heart. Through the incision, a rounded, encapsulated yellow tumor attached to the pulmonary valve was visible. The tumor had a very short stalk and was closely adherent to the edge of the central portion of the left cusp of the pulmonary valve. The tumor was carefully excised just at the point of attachment so as not to injure the cusp. The surface of the tumor was smooth and translucent. The gelatinous membrane on the surface tore easily, and multiple fronds were visible (Fig 2). No abnormal findings were detected in the right atrium or ventricle or on the tricuspid valve. Cardiopulmonary bypass was discontinued without difficulty. Intraoperative transesophageal echocardiography revealed no pulmonary insufficiency.
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Fig 2. (A) Intraoperative photograph demonstrating the encapsulated mass with a relatively smooth surface attached to pulmonary valve. (B) Putting the excised tumor in physiological saline solution immediately after excision resulted in the "sea anemone"–like appearance, thus providing easy verification of the tumor type.
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Pathological examination of the mass revealed a PFE with no features of malignancy. Postoperative whole-body computed tomography and transthoracic echocardiography showed no other masses in the heart. The fever gradually subsided after the operation.
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Comment
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In most series involving cardiac tumors, primary valve tumors comprise less than 10% of cases. In their multiinstitutional experience, Edwards and associates [2] found that 73.2% of heart valve tumors were PFEs. In a literature review of 198 cases of PFEs, Grinda and colleagues [3] indicated that the rate of occurrence at the aortic valve was more than twice that at the pulmonary valve. To our knowledge, symptomatic PFE at the pulmonary valve visualized before surgical intervention is extremely rare.
Papillary fibroelastomas are usually solitary tumors; multiple PFEs have been noted only a few times [3]. Recurrence after surgical excision has not been reported. In one patient, a diagnosis of PFE was made intraoperatively on the basis of gross appearance, and only the pulmonary trunk and two chambers were examined. To avoid incomplete removal, and recurrence, identification of the site of attachment and inspection of the cardiac chambers are important. We think that intraoperative transesophageal echocardiography and video-assisted devices are very useful for the identification of the entire lesion [4].
As most cardiac valve tumors are PFEs, which are benign tumors, and are rarely associated with valvular dysfunction, it has been suggested that such tumors can be excised locally without the need of valve replacement. Intraoperative transesophageal echocardiography is a useful adjunct in assessing any damage to the valve and the adequacy of valve repair. In the case of one patient, to make a diagnosis first, the encapsulated mass was excised just at the point of attachment to the valve. The tumor surface tore easily, and the similarity in appearance between the tumor and a sea anemone, key to the identification of a PFE, was easily verified. Once the diagnosis of a PFE, a benign tumor, was confirmed, no further resection of the valve was performed because extra resection might have necessitated valve replacement. The case of our patient emphasizes that during operation for a cardiac tumor, when the encapsulated mass is attached to a valve, the initial excision of valve tissue should be as minimal as possible. Once the "sea anemone"–like appearance has been confirmed, no further resection is needed. However, if this specific appearance is not verified, we recommend a greater resection of the valve.
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References
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Reynen K. Cardiac myxomas. N Engl J Med 1995;333:1610-1617.[Free Full Text]
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Edwards F.H., Hale D., Cohen A., Thompson L., Pezzella A.T., Virmani R. Primary cardiac valve tumors. Ann Thorac Surg 1991;52:1127-1131.[Abstract]
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Grinda J.M., Couetil J.P., Chauvaud S., et al. Cardiac valve papillary fibroelastoma: surgical excision for revealed or potential embolization. J Thorac Cardiovasc Surg 1999;117:106-110.[Abstract/Free Full Text]
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Allen K.B., Goldin M., Mitra R. Transaortic video-assisted excision of a left ventricular papillary fibroelastoma. J Thorac Cardiovasc Surg 1996;112:199-201.[Free Full Text]
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Abstract
Introduction
