Interact CardioVasc Thorac Surg 2008;7:1134-1136. doi:10.1510/icvts.2008.189332
© 2008 European Association of Cardio-Thoracic Surgery
Negative results - Valves |
Aortic valve papillary fibroelastoma that developed rapidly after open-heart surgery
Yuki Okamoto*,
Masahiko Matsumoto,
Hidenori Inoue and
Kisyo Shimura
Second Department of Surgery, Yamanashi University, 1110 Shimokato, Chuo City, Yamanashi, 409-3898, Japan
Received 25 July 2008;
received in revised form 4 September 2008;
accepted 8 September 2008
Corresponding author. Tel.: +81-55-273-1111; fax: +81-55-273-6767.
E-mail address: yamanashimedical{at}yahoo.co.jp (Y. Okamoto).
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Abstract
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The patient was a 76-year-old woman who underwent left atrial thrombectomy in November 2003 at our hospital. A mass adhered to the non-coronary aortic cusp was found during annual echocardiography, and the patient was admitted to our hospital. Transthoracic echocardiography detected two masses with pendulum-like movement: a 7-mm mass adhered to the non-coronary cusp and a 5-mm mass on the right coronary cusp. Papillary fibroelastoma was diagnosed based on the absence of inflammatory reactions or valvular destruction. Anticoagulant therapy was continued to prevent left atrial thrombus formation. At surgery, a 7-mm mass was adhered to the belly of the non-coronary cusp and a 5-mm pediculate mass was attached to a site near the nodule of Arantius of the right coronary cusp. Movable fibrous connective tissue 4 mm in length was present at two sites near the nodule of Arantius of the left coronary cusp, and these were also excised. All excised specimens, including those considered to be connective tissue, were found to be papillary fibroelastoma on pathological examination. Four papillary fibroelastomas that developed in all the cusps, including two small connective tissue-like morphologies and two sea anemone-like morphologies, indicated the process of papillary fibroelastoma growth and looked like ‘a family’.
Key Words: Valve disease; Papillary fibroelastoma; Cardiac tumor
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1. Introduction
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Papillary fibroelastoma (PFE) is a rare heart tumor [1–8]. Its mechanism of development has not been determined. Many cases are identified as complications of thromboembolism adhered to a tumor embolism and tumors or in valvular disease, or are found incidentally in a medical check-up [1–8]. Here, we report a case of aortic valve papillary fibroelastoma that developed in all cusps over a short period.
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2. Case report
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The patient was a 76-year-old woman who underwent left atrial thrombectomy in November 2003 at our hospital and was subsequently followed for atrial fibrillation by a local physician. A mass adhered to the non-coronary aortic cusp was found by echocardiography performed annually and the patient was admitted to our hospital. No apparent fever or inflammatory reaction was noted in blood tests. Transthoracic echocardiography detected two masses with pendulum-like movement: a 7-mm mass adhered to the non-coronary cusp and a 5-mm mass on the right or left coronary cusp (Fig. 1). However, there was no aortic insufficiency and cardiac function was normal (Videos 1 and 2). PFE was diagnosed based on the absence of fever, inflammatory reactions, or valvular destruction or abnormal valvular function. Anticoagulant therapy was continued to prevent left atrial thrombus formation and surgery was performed.
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Fig. 1. Transthoracic echocardiography shows two high dense masses attached to the non-coronary cusp (arrowhead) and the right or left coronary cusp (arrow).
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Moderate adhesion due to the previous surgery was noted in an approach by median sternotomy. After establishing extracorporeal circulation via the ascending aortic blood supply and right arterial blood withdrawal, aortic crossclamping was performed and cardioplegia was used to induce cardiac arrest. A transverse incision of approximately 3 cm in the ascending aorta was made to observe the three cusps of the aortic valve. A 7-mm mass was adhered to the belly of the non-coronary cusp and a 5-mm yellowish-white pediculate mass was attached to a site near the nodule of Arantius of the right coronary cusp. Movable fibrous connective tissue 4 mm in length was present at two sites near the nodule of Arantius of the left coronary cusp, and these were also excised. The excised tumors were immersed in physiological saline. Those excised from the non-coronary and right coronary cusps showed a sea anemone-like morphology, based on which PFE was diagnosed (Fig. 2). Only tumorectomy was performed because no recurrent case has been reported and no residual tumor was apparent macroscopically. The aorta was sutured and blockage of the aorta was released. No abnormal aortic valve function, such as aortic regurgitation, was detected by transesophageal echocardiography. The durations of extracorporeal circulation and aortic cross-clamping were 89 and 19 min, respectively.
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Fig. 2. Macroscopic specimens arising from the non-coronary cusp (a) and the right coronary cusp (b) show the appearance of the tumor immersed in saline solution resembling a sea anemone. However, two excised specimen attached to the left coronary cusp reveals the thin fibrous connective tissue-like appearance (c).
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No residual tumor was detected on postoperative transthoracic echocardiography and aortic valve function was good. All excised specimens, including those considered to be connective tissue were found to be PFE on pathological examination. The patient recovered smoothly and was discharged 20 days after surgery.
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3. Discussion
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PFE is a rare benign tumor with an incidence that is the third highest among heart tumors following myxoma and lipoma [3, 7]. PFE develops most frequently in the aortic valve and may develop in multiple cusps, as in this case; however, development in all three cusps is very rare [1–4, 8]. Pediculate movable tumors and those with a sea anemone-like morphology are likely to be imaged by echocardiography. However, when the size is small and the morphology is not sea anemone-like, as in the PFE adhered to the left coronary cusp in this case, imaging by echocardiography is difficult [3, 8].
Persistent turbulent flow in the heart or endothelial cell disorder due to a particular cause is generally thought to induce endothelial cell hyperplasia and lead to development of PFE [1–7].
Kurup et al. have suggested that the procedure of open-heart surgery may also cause PFE and have reported that among surgically excised PFEs, non-iatrogenic tumors were likely to be single and iatrogenic tumors were likely to be multiple [6].
Our patient had undergone previous open-heart surgery, in addition, the blood supply and cardioplegia infusion route were established via the ascending aorta around the aortic valve during previous surgery. However, it is unclear whether this procedure and subsequent turbulent blood flow impaired the function of endothelial cells. Moreover, Kurup et al. reported the mean interval year at tumor excision to be 16 years (range 8–31 years) in 22 iatrogenic cases [6]. In comparison with these 22 cases, our case of PFE that developed over a short period is extremely rare.
PFE can be diagnosed when the tumor floats on saline and shows a sea anemone-like morphology, but some PFE cases do not show this morphology and macroscopically appear as fibrous connective tissue, as seen in this case. Since PFE may develop in any region, it is difficult to identify PFE completely, and therefore an explanation of the possibility of recurrence, new development, and overlooking of PFE should be given to patients. A quite rare case of four PFEs that developed in all the cusps, including two small connective tissue-like morphologies and two sea anemone-like morphologies, indicated the process of PFE growth and looked like ‘a family’. Since identification of PFE is difficult when the morphology is not typical or the tumor size is small, sufficient follow-up is necessary to investigate potential development of a new tumor.
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References
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- Ngaage DL, Mullany CJ, Daly RC, Dearani JA, Edwards WD, Tazelaar HD, McGregor CG, Orszulak TA, Puga FJ, Schaff HV, Sundt TM 3rd, Zehr KJ. Surgical treatment of cardiac papillary fibroelastoma: a single center experience with eighty-eight patients. Ann Thorac Surg 2001;80:1712–1718.
- Gowda RM, Khan IA, Nair CK, Mehta NJ, Vasavada BC, Sacchi TJ. Cardiac papillary fibroelastoma: a comprehensive analysis of 725 cases. Am Heart J 2003;146:404–410.[CrossRef][Medline]
- Shahian DM. Papillary fibroelastomas. Semin Thorac Cardiovasc Surg 2000;12:101–110.[Medline]
- Sun JP, Asher CR, Yang XS, Cheng GG, Scalia GM, Massed AG, Griffin BP, Rastliff NB, Stewart WJ, Thomas JD. Clinical and echocardiographic characteristic of papillary fibroelastoma: a retrospective and prospective study in 162 patients. Circulation 2001;103:2687–2693.[Abstract/Free Full Text]
- Grandmougin D, Fayad D, Moukassa D, Decoene C, Abolmaali K, Bodart JC, Limousin M, Warembourg H. Cardiac valve papillary fibroelastomas: clinical, histological and immunohistochemical studies and a physiopathogenic hypothesis. J Heart Valve Dis 2000;9:832–841.[Medline]
- Kurup AN, Tazelaar HD, Edwards WD, Burke AP, Virmani R, Klarich KW, Orszulak TA. Iatrogenic cardiac papillary fibroelastoma: a study of 12 cases (1990–2000). Hum Pathol 2002;33:1165–1169.[CrossRef][Medline]
- Sumino S, Paterson HS. No regrowth after incomplete papillary fibroelastoma excision. Ann Thorac Surg 2005;79:e3–e4.[Abstract/Free Full Text]
- Touati GD, Carmi D, Sevestre H, Poulain H. Multiple aortic valve papillary fibroelastoma: do not miss the other one. Eur J Cardiothorac Surg 2002;21:595–598.[Free Full Text]
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Abstract
1. Introduction
