Papillary Fibroelastoma of Mitral Valve Chorda Ricardo Gallo, MD, Naresh Kumar, FRCS, Ganga Prabhakar, FRCS, Adnan Awada, MD, Yousef Maalouf, MD, and Carlos M. G. Duran, MD, PhD King Faisal Specialist Hospital and Research Center and King Fahad National Guard Hospital, Riyadh, Saudi Arabia A case of papillary fibroelastoma arising from a mitral valve chorda, presenting with right-sided hemiparesis, that was successfully treated by chordal excision is herein reported. (Ann Thorac Surg 2993;55:2576-7) P rimary tumors of the heart valves are rare with important clinical significance. Their incidence in large series is reported to be less than 10%of all cardiac tumors [l, 21. We report here a primary papillary fibroelastoma originating in a strut chorda of the anterior leaflet of the mitral valve in a young man presenting with an embolic cerebrovascular episode. The fibroelastoma was diagnosed by echocardiography and successfully operated on with conservation of the valve. A 21-year-old male patient was admitted to another hospital with sudden-onset right-sided hemiparesis and aphasia 2 weeks before transfer to our hospital. He was investigated at the referring hospital, where a transthoracic and transesophageal echocardiogram showed an 8 x 8-mm nodular mass (Fig 1) in the strut chorda originating from the posterior papillary muscle and inserting into the anterior cusp of the mitral valve close to its cuspal insertion. He had no evidence of other valve pathology or a left-sided thrombus. Hemodynamically he was in stable condition, and a computed tomographic scan of the brain showed infarction in the region of the left middle cerebral artery distribution, possibly embolic. Heparin infusion was commenced, and the patient was transferred to our hospital. On admission the presence of the chordal mass and absence of any other cardiac valvular or cavitary pathology were confirmed by repeat echocardiography. His left ventricular ejection fraction was estimated to be 0.66. The patient underwent operation on cardiopulmonary bypass using standard techniques and retrograde blood cardioplegia. The left atrium was small and was opened by an extended transseptal approach, and the mitral valve was visualized. The cusps and commissures were normal with no evidence of left atrial thrombus. There was a small (8 x 8-mm) myxoid mass attached to the chorda extending from the posterior papillary muscle to the anterior cusp with normal chordal tissue above and below the mass (Fig 2). As there were two other chordae on Accepted for publication Aug 21, 1992. Address reprint requests to Dr Gallo, King Faisal Specialist Hospital and Research Center, MBC 16, Riyadh 11211, Saudi Arabia. 0 1993 by The Society of Thoracic Surgeons either side of the involved chord, inserting into the anterior cusp, it was resected with the mass. The patient was weaned from bypass without difficulty, and intraoperative transesophageal echocardiography showed no evidence of valvar regurgitation. Recovery from the operation was uneventful. The histologic diagnosis was confirmed to be papillary fibroelastoma. Comment Primary cardiac valve tumors are uncommon, with an incidence of less than 10% of all cardiac tumors. In a recent review from the Walter Reed Institute of Pathology, 56 valve tumors occurring in 53 patients over a 58-year period were documented [3]. Each of the four cardiac valves was involved with equal frequency, and tumor size ranged from 3 mm to 7 cm with an average of 1.15 cm. Forty-one (73.21%)of these tumors were papillary fibroelastomas. These tumors are rare, diagnosed mostly at autopsy, and can occur on the leaflets, papillary muscles, chordae tendineae, ventricular septum, or the endocardia1 surface [2]. It was initially believed that these tumors were not clinically significant, but now it has been well proved that they are responsible for coronary and cerebral emboli [4, 51. In our patient, there was an embolism of the left middle cerebral artery with a nonfatal but dense rightsided hemiplegia. Embolization may occur from the frag- Fig 1 . Echocardiogram showing mitral valve chordal tumor. Arrow points to the tumor. The portion of the chorda abozv the tumor is seen clearly. (Lv = left ventricle..) 0003-4W5/93/$6.00 CASE REPORT Ann Thorac Surg 1993;55:157&7 GALLO ET AL VALVE TUMOR 1577 chorda supporting the anterior cusp of the mitral valve made conservation of the valve possible. In conclusion, we report a patient with a papillary fibroelastoma occurring on a chorda of the mitral valve treated by a conservative valve operation. The reports of this rare condition will certainly increase with more cases of even small tumors being referred for evaluation after echocardiographic identification. We are grateful to Alison Silkstone for her help in the preparation of the manuscript and Dr John Antonius for the histopathologic examination. Fig 2 . Surgical photograph showing the 8 X 8-mm tumor on the strut chorda of the mitral valve. References ile papillary fronds [3, 61 of the tumor itself or from a thrombus forming on the tumor [5]. The exact cause of cerebral embolization in our patient was difficult to ascertain. Before the advent of echocardiography, diagnosis of valve tumors were made at autopsy as an incidental finding. At present these tumors are being increasingly recognized by echocardiography. When the mitral valve is involved, the tumor arises most often on the atrial side of the valve [3]. In our patient the mass was attached to one chorda of the mitral valve. Valve tumors should be managed surgically by complete excision to avoid recurrence of tumor or thrombi. In our patient the location of the tumor on a nonexclusive strut 1. Dein JR, Frist WH, Stinson EB, et al. Primary cardiac neoplasms. Early and late results of surgical treatment in 42 patients. J Thorac Cardiovasc Surg 1987;93:502-11. 2. McAllister HA, Fenoglio JJ. Tumors of the cardiovascular system. In: Atlas of tumor pathology, 2nd series. Washington, DC: Armed Forces Institute of Pathology, 1978. 3. Edwards FH, Hale D, Cohen A, Thompson L, Pezzella T, Virmani R. Primary cardiac valve tumors. Ann Thorac Surg 1991;52:1127-31. 4. Kasarskis EJ, OConnor W, Earle G. Embolic stroke from cardiac papillary fibroelastoma. Stroke 1988;19:1171-3. 5. Fowles RE, Miller DC, Egbert BM, Fitzgerald JW, Popp RL. Systemic embolization from a mitral valve papillary endocardial fibroma detected by two-dimensional echocardiography . Am Heart J 1981;102128-30. 6 . Heath D, Best PV, Davis BT. Papilliferous tumors of the heart valves. Br Heart J 1961;23:204.