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Aortic valve papillary fibroelastoma
PII: S0967-2109(01)00093-X
Cardiovascular Surgery, Vol. 10, No. 1, pp. 65–67, 2002 2002 The International Society for Cardiovascular Surgery Published by Elsevier Science Ltd. All rights reserved 0967-2109/02 $22.00
www.elsevier.com/locate/cardiosur
CASE REPORT Aortic valve papillary fibroelastoma Abdallah Alawi1, Elie B. Kassabian, Ramzi Ashoush and Victor A. Jebara Department of Cardiovascular and Thoracic Surgery, Hoˆtel Dieu de France, Rue Adib Ishac, Beirut, Lebanon Cardiac papillary fibroelastomas are rare tumors of the heart and affect primarily the cardiac valves. These lesions are responsible for embolic accidents that clinically manifest as neurological and cardiovascular symptoms. We describe the case of a 40-yr-old woman who presented with a neurological deficit caused by a papillary tumor of the aortic valve. Diagnosis was made by transesophageal echocardiaphy and the tumor was surgically removed. 2002 The International Society for Cardiovascular Surgery. Published by Elsevier Science Ltd. All rights reserved Keywords: papillary fibroelastoma, embolic accidents, transoesophageal echocardiography
Case report Primary tumors of heart valves are rare. The most commonly encountered are papillary fibroelastomas. Although benign, these tumors are potential embolic sources to the brain and the coronary circulation. Many have been associated with transient ischemic attacks, stroke, angina, myocardial infarction and sudden death [1–3]. A 40-yr-old female was admitted for sudden onset of dysarthria and left upper limb hemiparesis. Upon admission, she was in normal sinus rhythm. Her vital signs were stable and no other abnormalities were noted. Her past medical history was uneventful. A computed tomographic scan of the brain showed ischemic lesions in the region of the right middle cerebral artery distribution. A duplex scan of the carotids revealed total occlusion of the right common carotid artery at its origin, the other vessels were normal. An arteriogram confirmed the occlusion of the right common carotid and showed retrograde filling of the distal cerebral vessels through the anterior communicating and ophtalmic branches. The other brachiocephalic vessels were normal.
Correspondence to: Dr. Victor A. Jebara. Tel.: +96-1-3272747; Fax: +96-1-1426181; e-mail: [email protected] 1 Present address: Department of Cardiology, Central Military Hospital, Beirut, Lebanon
CARDIOVASCULAR SURGERY
FEBRUARY 2002 VOL 10 NO 1
The patient had no stigmates of Takayasu’s disease and an embolic origin was suspected. A two-dimensional transesophageal echocardiogram was obtained and showed a 6×8 mm pedunculated mass arising from the non-coronary cusp of the aortic valve at the level of the non-coronary to right coronary commissure (Figure 1). A papillary fibroelastoma was suspected. There was no evidence of left sided thrombus and the rest of the examination was normal. Neurologic symptoms improved progressively, and at one week, the patient made a total recovery. A CT scan of the brain one month after the initial event was normal. Surgery was decided to resect the cardiac tumor in order to avoid further embolic events. Under cardiopulmonary bypass and using standard techniques, the aorta was opened. As was shown by the TEE a 7 mm polypoid mass attached with a small pedicle to the edge of the non coronary cusp of the aortic valve was found and resected with care taken not to injure the aortic cusp. Pathology showed a papillary fibroelastoma.
Comment Primary cardiac tumors are rare, and include both benign and malignant lesions. The reported prevalence in autopsy varies among authors. It ranges from 0.0017–0.28% to 0.02–0.33% [3, 4]. Eighty percent of primary cardiac tumors are 65
Aortic valve papillary fibroelastoma: A. Alawi et al.
Figure 1 Transoesophageal echocradiogram showing the pedunculated mass arising from the aortic valve
benign. The most common type is myxoma, which represents approximately 50% of all cases [4]. Cardiac papillary fibroelastomas (CPFE) are the third most common benign tumors of the heart after myxomas and lipomas, but represent the most common valvular tumors of the heart [1, 3–5]. They most often originate from the valvular endocardium, and affect men and women equally. Mean age at the time of the diagnosis is 60 yr, but CPFE has been described in patients aged from 6 days to 92 yr [4, 5]. CPFE are small avascular tumors ranging in size from 0.1 to 5.7 cm with a mean size of 1.5 cm. They are derived from the normal components of the endocardium and are ligned by a single layer of hyperplastic endothelial cells. The mycopolysaccharide matrix contains fibrous connective tissue, elastic fibers and smooth muscle cells. The tumor has the characteristic ‘sea anemone’ appearance produced by the multiple papillary fronds [1–4]. Eighty-four percent of known cases of CPFE originate from cardiac valves. In a series studied by Ryan and associates, 88% of valvular tumors were CPFE. Edwards and associates found that CPFE represented 73.21% of the cardiac-valvular tumors in their series [1, 4]. Each of the four cardiac valves is affected with equal frequency. Nonvalvular locations of the tumor occur in 15.7% [4]. They include the ventricular 66
endocardial wall, the atrial endocardium, papillary muscle and chordæ tendinæ. Most surgically resected CPFE are located in the left side of the heart although the tumor affects equally both the right and left sides. Multiple CPFE are rare. They usually occur as solitary tumors. Only 7.5% of collected cases were multiple [4, 6]. Prior to the availability of transthoracic and transesophageal echocardiography, these tumors were described as incidental findings at autopsy or during cardiac surgical procedures [3, 6]. The clinical implications of CPFE are now well known. There is sufficient evidence to state that CPFE can cause serious symptoms. Common presentations include transient ischemic attacks, stroke, angina, myocardial infarction and death [2, 3, 5]. These complications are mostly due to embolic debris that originate either from the tumor itself by release of papillary fragments into the circulation, or from a thrombus that forms around the tumor, since its surface may become a nidus for platelet aggregates. In the case of intermittent ischemic heart symptoms, prolapse of a pedunculated tumor into the coronary ostia has been described [4, 6]. The most reliable diagnostic tool is transesophageal echocardiography. TEE can also guide the surgical resection and assess the adequacy of valve repair both pre- and postoperatively [2–4]. Whether to perform routine preoperative coronary CARDIOVASCULAR SURGERY
FEBRUARY 2002 VOL 10 NO 1
Aortic valve papillary fibroelastoma: A. Alawi et al.
angiogram prior to resection of a CPFE is subject to debate due to the friable nature of the lesion and to the potential risk of embolization [6]. Surgical resection of CPFE is warranted even in asymptomatic in order to avoid the life threatening complications secondary to embolic events. A conservative surgical approach to the culprit heart valve is generally possible. Should valvular damage occur during resection of the tumor, standard techniques of valve repair are effective. Eckstein and associates [3] used an autologous pericardial patch to reconstruct an aortic valve, whereas, Grinda and associates [4] reported using a cryopreserved aortic homograft cusp to achieve restoration of normal cusp architecture in one patient. Recurrence after surgical resection is unknown to date [4].
Conclusion CPFE should be treated surgically because of its potentially dangerous complications. Transesophagal echocardiography is the best mean in diagnosing CPFE. Most patients present with clini-
CARDIOVASCULAR SURGERY
FEBRUARY 2002 VOL 10 NO 1
cal manifestations of an embolic nature originating from the tumor. Surgical cure is effective in preventing serious complications. Recurrence after surgery has not been reported but careful follow-up is necessary.
References 1. Edwards, F. H., Hale, D., Cohen, A. et al., Primary cardiac valve tumors. Annals of Thoracic Surgery, 1991, 52, 1127–1131. 2. Gallo, R., Kumar, N., Prabhakar, G. et al., Papillary fibroelastoma of mitral valve chordæ. Annals of Thoracic Surgery, 1993, 55, 1576–1577. 3. Eckstein, F. S., Scha¨ fers, H. J., Groten, J. et al., Papillary fibroelastoma of the aortic valve presenting with myocardial infarction. Annals of Thoracic Surgery, 1995, 60, 206–208. 4. Grinda, J. M., Chauvaud, S., D’Attellis, N. et al., Cardiac valve papillary fibroelastoma: surgical excision for revealed or potential embolization. Journal of Thoracic and Cardiovascular Surgery, 1999, 117, 106–110. 5. Wolfe, J. T., Finck, S. J., Safford, R. E. et al., Tricuspid valve papillary fibroelastoma: echocardiographic characterization. Annals of Thoracic Surgery, 1991, 51, 116–118. 6. Sahian, D. M., Labib, S. B. and Chang, G., Cardiac papillary fibroelastoma. Annals of Thoracic Surgery, 1995, 59, 538–541. Paper accepted 15 May 2001
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Cardiovascular Surgery, Vol. 10, No. 1, pp. 65–67, 2002 2002 The International Society for Cardiovascular Surgery Published by Elsevier Science Ltd. All rights reserved 0967-2109/02 $22.00
www.elsevier.com/locate/cardiosur
CASE REPORT Aortic valve papillary fibroelastoma Abdallah Alawi1, Elie B. Kassabian, Ramzi Ashoush and Victor A. Jebara Department of Cardiovascular and Thoracic Surgery, Hoˆtel Dieu de France, Rue Adib Ishac, Beirut, Lebanon Cardiac papillary fibroelastomas are rare tumors of the heart and affect primarily the cardiac valves. These lesions are responsible for embolic accidents that clinically manifest as neurological and cardiovascular symptoms. We describe the case of a 40-yr-old woman who presented with a neurological deficit caused by a papillary tumor of the aortic valve. Diagnosis was made by transesophageal echocardiaphy and the tumor was surgically removed. 2002 The International Society for Cardiovascular Surgery. Published by Elsevier Science Ltd. All rights reserved Keywords: papillary fibroelastoma, embolic accidents, transoesophageal echocardiography
Case report Primary tumors of heart valves are rare. The most commonly encountered are papillary fibroelastomas. Although benign, these tumors are potential embolic sources to the brain and the coronary circulation. Many have been associated with transient ischemic attacks, stroke, angina, myocardial infarction and sudden death [1–3]. A 40-yr-old female was admitted for sudden onset of dysarthria and left upper limb hemiparesis. Upon admission, she was in normal sinus rhythm. Her vital signs were stable and no other abnormalities were noted. Her past medical history was uneventful. A computed tomographic scan of the brain showed ischemic lesions in the region of the right middle cerebral artery distribution. A duplex scan of the carotids revealed total occlusion of the right common carotid artery at its origin, the other vessels were normal. An arteriogram confirmed the occlusion of the right common carotid and showed retrograde filling of the distal cerebral vessels through the anterior communicating and ophtalmic branches. The other brachiocephalic vessels were normal.
Correspondence to: Dr. Victor A. Jebara. Tel.: +96-1-3272747; Fax: +96-1-1426181; e-mail: [email protected] 1 Present address: Department of Cardiology, Central Military Hospital, Beirut, Lebanon
CARDIOVASCULAR SURGERY
FEBRUARY 2002 VOL 10 NO 1
The patient had no stigmates of Takayasu’s disease and an embolic origin was suspected. A two-dimensional transesophageal echocardiogram was obtained and showed a 6×8 mm pedunculated mass arising from the non-coronary cusp of the aortic valve at the level of the non-coronary to right coronary commissure (Figure 1). A papillary fibroelastoma was suspected. There was no evidence of left sided thrombus and the rest of the examination was normal. Neurologic symptoms improved progressively, and at one week, the patient made a total recovery. A CT scan of the brain one month after the initial event was normal. Surgery was decided to resect the cardiac tumor in order to avoid further embolic events. Under cardiopulmonary bypass and using standard techniques, the aorta was opened. As was shown by the TEE a 7 mm polypoid mass attached with a small pedicle to the edge of the non coronary cusp of the aortic valve was found and resected with care taken not to injure the aortic cusp. Pathology showed a papillary fibroelastoma.
Comment Primary cardiac tumors are rare, and include both benign and malignant lesions. The reported prevalence in autopsy varies among authors. It ranges from 0.0017–0.28% to 0.02–0.33% [3, 4]. Eighty percent of primary cardiac tumors are 65
Aortic valve papillary fibroelastoma: A. Alawi et al.
Figure 1 Transoesophageal echocradiogram showing the pedunculated mass arising from the aortic valve
benign. The most common type is myxoma, which represents approximately 50% of all cases [4]. Cardiac papillary fibroelastomas (CPFE) are the third most common benign tumors of the heart after myxomas and lipomas, but represent the most common valvular tumors of the heart [1, 3–5]. They most often originate from the valvular endocardium, and affect men and women equally. Mean age at the time of the diagnosis is 60 yr, but CPFE has been described in patients aged from 6 days to 92 yr [4, 5]. CPFE are small avascular tumors ranging in size from 0.1 to 5.7 cm with a mean size of 1.5 cm. They are derived from the normal components of the endocardium and are ligned by a single layer of hyperplastic endothelial cells. The mycopolysaccharide matrix contains fibrous connective tissue, elastic fibers and smooth muscle cells. The tumor has the characteristic ‘sea anemone’ appearance produced by the multiple papillary fronds [1–4]. Eighty-four percent of known cases of CPFE originate from cardiac valves. In a series studied by Ryan and associates, 88% of valvular tumors were CPFE. Edwards and associates found that CPFE represented 73.21% of the cardiac-valvular tumors in their series [1, 4]. Each of the four cardiac valves is affected with equal frequency. Nonvalvular locations of the tumor occur in 15.7% [4]. They include the ventricular 66
endocardial wall, the atrial endocardium, papillary muscle and chordæ tendinæ. Most surgically resected CPFE are located in the left side of the heart although the tumor affects equally both the right and left sides. Multiple CPFE are rare. They usually occur as solitary tumors. Only 7.5% of collected cases were multiple [4, 6]. Prior to the availability of transthoracic and transesophageal echocardiography, these tumors were described as incidental findings at autopsy or during cardiac surgical procedures [3, 6]. The clinical implications of CPFE are now well known. There is sufficient evidence to state that CPFE can cause serious symptoms. Common presentations include transient ischemic attacks, stroke, angina, myocardial infarction and death [2, 3, 5]. These complications are mostly due to embolic debris that originate either from the tumor itself by release of papillary fragments into the circulation, or from a thrombus that forms around the tumor, since its surface may become a nidus for platelet aggregates. In the case of intermittent ischemic heart symptoms, prolapse of a pedunculated tumor into the coronary ostia has been described [4, 6]. The most reliable diagnostic tool is transesophageal echocardiography. TEE can also guide the surgical resection and assess the adequacy of valve repair both pre- and postoperatively [2–4]. Whether to perform routine preoperative coronary CARDIOVASCULAR SURGERY
FEBRUARY 2002 VOL 10 NO 1
Aortic valve papillary fibroelastoma: A. Alawi et al.
angiogram prior to resection of a CPFE is subject to debate due to the friable nature of the lesion and to the potential risk of embolization [6]. Surgical resection of CPFE is warranted even in asymptomatic in order to avoid the life threatening complications secondary to embolic events. A conservative surgical approach to the culprit heart valve is generally possible. Should valvular damage occur during resection of the tumor, standard techniques of valve repair are effective. Eckstein and associates [3] used an autologous pericardial patch to reconstruct an aortic valve, whereas, Grinda and associates [4] reported using a cryopreserved aortic homograft cusp to achieve restoration of normal cusp architecture in one patient. Recurrence after surgical resection is unknown to date [4].
Conclusion CPFE should be treated surgically because of its potentially dangerous complications. Transesophagal echocardiography is the best mean in diagnosing CPFE. Most patients present with clini-
CARDIOVASCULAR SURGERY
FEBRUARY 2002 VOL 10 NO 1
cal manifestations of an embolic nature originating from the tumor. Surgical cure is effective in preventing serious complications. Recurrence after surgery has not been reported but careful follow-up is necessary.
References 1. Edwards, F. H., Hale, D., Cohen, A. et al., Primary cardiac valve tumors. Annals of Thoracic Surgery, 1991, 52, 1127–1131. 2. Gallo, R., Kumar, N., Prabhakar, G. et al., Papillary fibroelastoma of mitral valve chordæ. Annals of Thoracic Surgery, 1993, 55, 1576–1577. 3. Eckstein, F. S., Scha¨ fers, H. J., Groten, J. et al., Papillary fibroelastoma of the aortic valve presenting with myocardial infarction. Annals of Thoracic Surgery, 1995, 60, 206–208. 4. Grinda, J. M., Chauvaud, S., D’Attellis, N. et al., Cardiac valve papillary fibroelastoma: surgical excision for revealed or potential embolization. Journal of Thoracic and Cardiovascular Surgery, 1999, 117, 106–110. 5. Wolfe, J. T., Finck, S. J., Safford, R. E. et al., Tricuspid valve papillary fibroelastoma: echocardiographic characterization. Annals of Thoracic Surgery, 1991, 51, 116–118. 6. Sahian, D. M., Labib, S. B. and Chang, G., Cardiac papillary fibroelastoma. Annals of Thoracic Surgery, 1995, 59, 538–541. Paper accepted 15 May 2001
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