Removal of a ventricular mitral valve fibroelastoma by an aortic approach
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《血管的通路杂志》
a Department of Thoracic and Cardiovascular Surgery, Dupuytren University Medical Hospital, 2 Av. M. L. King, 87042 University of Limoges, France
b Department of Cardiology, Dupuytren University Medical Hospital, University of Limoges, France
Presented at the 55th International Congress of the European Society for Cardiovascular Surgery, St Petersburg, Russian Federation, May 11–14, 2006.
Abstract
Cardiac papillary fibroelastomas are rare neoplasms usually incidentally found by echocardiography for unrelated problems. Otherwise they could be diagnosed following cardiac symptoms or after an embolic complication. The treatment is only surgical, whatever their size, to prevent any complications. A patient is described who presented acute psychiatric symptoms as a consequence of cerebral embolism of a mitral valve papillary fibroelastoma. The surgical excision was performed through an aortotomy with videoscopy used to help exposure and removal.
Key Words: Valve disease; Tumor; Mitral valve repair
1. Introduction
Cardiac papillary fibroelastomas (CPFEs) are rare neoplasms that represent the third etiology of benign cardiac primitive tumors. They are usually small, singular and do not recur after surgical excision. Most CPFEs are asymptomatic and are incidentally found during echography or surgery. Otherwise they could be diagnosed following cardiac symptoms or after an embolic complication. The treatment is only surgical to prevent any complications. We report the unusual case of a papillary fibroelastoma located on a mitral valve chorda presenting acute psychiatric symptoms as a consequence of vertebrobasilar cerebral territory embolism. The surgical excision was performed through an aortotomy with videoscopy used to help exposure and removal.
2. Case report
A 55-year-old man, suffering from depression and loss of interest at work, unexplainedly ran away from work on two occasions, driving aimlessly and drinking until he was found without aforethought. Clinical examination just pointed out memory disorders. Neurological investigations found a right temporal lobe epilepsy via an electroencephalogram and a right hypothalamic hole without any localized tumor via an MRI, suggesting a posterior cerebral artery territory embolism. Further investigations (CT scan, ultrasounds of carotid arteries) performed to search for an embolic etiology were normal but a transesophageal echocardiography showed a single one centimeter diameter mobile mass on the ventricular side of the anterior mitral valve (Fig. 1a). The diagnosis was thought to be a myxoma and, therefore, the patient was referred to surgery.
Under cardiopulmonary bypass, an exploration was first performed through an aortotomy and the aortic valve with the help of a thoracoscope to ensure the mass nature and the possibility of its resection. It revealed a small myxoid mass attached to one anterior mitral valve second-order chorda. A resection of the tumor preserving the mitral valve was decided based on the macroscopic aspect of the mass suggesting a benign tumor and its single location on a second-order chorda. Excision of the tumor and the chorda was performed using this same approach with thoracoscopic instruments (Fig. 2). The patient was weaned from bypass without difficulty and intraoperative transesophageal echography showed neither evidence of valvular regurgitation nor remaining mass after resection (Fig. 1b).
The histological diagnosis was confirmed to be papillary fibroelastoma, with clear margins. Recovery from the operation was uneventful and the patient was discharged on postoperative day 12. He had no cardiac or new neurological problems during the past 6 years of follow-up. His epilepsy is stabilized under treatment, and his cognitive function assessment has improved with time.
3. Discussion
CPFEs were first described by Yater [1] in 1931 and are the most common cardiac valve tumors and the third most frequent benign cardiac primitive neoplasms after myxomas and fibromas [2]. This tumor is single in 90% of cases and more than 95% occur in the left heart. Its frequency varies from 2.9 to 7.9% but it might be more frequent for two reasons: first because nowadays tumors are discovered incidentally by echocardiography in patients being evaluated for unrelated problems and second because a new review of the pathological anatomy classification has included different tumors as papillary fibroelastomas.
CPFE has a characteristic flower-like appearance or sea anemone with multiple papillary fronds attached to the endocardium by a short pedicle. Papillary fronds are narrow, elongated and branching and are best visualized when immersed in water. The majority of tumors are around 15 mm in their greatest dimension [2], but tumors as large as 40 mm have been reported [3]. On microscopic analysis, CPFEs are avascular papillomas with a single layer of endocardial cells covering the papillary surface. The matrix consists of proteoglycans and some elastic fibers that are longitudinally oriented [4]. Thrombi attached on the tumor could be discovered with microscopic analysis.
Its histogenesis and pathogenesis remain controversial. Immunohistochemical investigations are in favor of a hamartomatous origin [5], whereas a recent finding of cytomegalovirus in a tumor suggested the possibility of a virus-induced tumor mechanism [6]. Most CPFEs are acquired lesions, as demonstrated by at least two cases of new diagnosed tumors eight and twelve years after cardiac surgery. However, there are few congenital cases reported in the literature but no familial occurrence has been noticed.
Mean age of patients at time of diagnosis is 50 years old, with a wide range from birth to 92 years old, without sex ratio. No recurrence has been reported after surgery in patients followed by echocardiography with the longest available follow-up of 11 years.
The potential for life-threatening complications of CPFEs, even asymptomatic ones, is an indication for their surgical excision whatever their size, as long as there are no major contraindications to the operation. Surgery is performed under cardiopulmonary bypass, usually through the left atrium when the mitral valve is concerned. Surgical excision must be complete, and when the valvular tissue is involved, the valve should be preserved if possible. As in our case, a single location on a mitral valve chorda could be removed without mitral valve regurgitation checked by an echography at the end of surgery [7]. The use of a thoracoscope has been described to visualize and remove a CPFE posterior to the mitral valve through the left atrium [8]. We preferred to first have a general look at the left ventricle by an aortotomy with the help of a thoracoscope to make sure there was no other location undetected by the preoperative echography and to visualize directly the mass. This approach also ensured its resectability, followed by its excision without any difficulty using the same approach with thoracoscopic instruments. This particular surgical method seems very interesting to us when the tumor is located in the left ventricle.
Our case is also unusual in its diagnosis. Usually brain embolic complications occur in the middle cerebral artery territory, whereas in our case it concerned the posterior cerebral artery of the vertebrobasilar territory. Clinical features of thalamic infarction could include impaired memory, perseveration, altered social skills and personality as described in our case [9].
4. Conclusion
We present this single case to highlight this particular approach of a ventricular mitral valve mass diagnosed by echocardiography. The exposure, the analysis of the mass and finally, its resection could easily be performed through the aortic valve with the use of videoscopy.
References
Yater WM. Tumors of the heart and pericardium: pathology, symptomatology and report of nine cases. Arch Intern Med 1931; 48:627–666.
Shahian D. Papillary fibroelastomas. Semin Thorac Cardiovasc Surg 2000; 12:101–110.
Crestanello JA, Orszulak TA. Giant papillary fibroelastoma of the right atrium: an unusual presentation. Ann Thorac Surg 2002; 74:1252–1254.
Burke A, Virmani R. Papillary fibroelastoma: tumors of the heart and great vessels. AFIP Atlas of Tumor Pathology 1996; 16:47–54.
Paraf F. Pathology of primary cardiac neoplasms. Ann Pathol 1999; 19:212–222.
Grandmougin D, Fayad G, 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.
Gallo R, Kumar N, Prabhakar G, Awada A, Maalouf Y, Duran C. Papillary fibroelastoma of mitral valve chorda. Ann Thorac Surg 1993; 55:1576–1577.
Espada R, Talwalker NG, Wilcox G, Kleiman NS, Verani MS. Visualization of ventricular fibroelastoma with a video-assisted thoracoscope. Ann Thorac Surg 1997; 63:221–223.
Schmahmann JD. Vascular syndromes of the thalamus. Stroke 2003; 34:2264–2278.(Alexandre Le Guyader, Kar)
b Department of Cardiology, Dupuytren University Medical Hospital, University of Limoges, France
Presented at the 55th International Congress of the European Society for Cardiovascular Surgery, St Petersburg, Russian Federation, May 11–14, 2006.
Abstract
Cardiac papillary fibroelastomas are rare neoplasms usually incidentally found by echocardiography for unrelated problems. Otherwise they could be diagnosed following cardiac symptoms or after an embolic complication. The treatment is only surgical, whatever their size, to prevent any complications. A patient is described who presented acute psychiatric symptoms as a consequence of cerebral embolism of a mitral valve papillary fibroelastoma. The surgical excision was performed through an aortotomy with videoscopy used to help exposure and removal.
Key Words: Valve disease; Tumor; Mitral valve repair
1. Introduction
Cardiac papillary fibroelastomas (CPFEs) are rare neoplasms that represent the third etiology of benign cardiac primitive tumors. They are usually small, singular and do not recur after surgical excision. Most CPFEs are asymptomatic and are incidentally found during echography or surgery. Otherwise they could be diagnosed following cardiac symptoms or after an embolic complication. The treatment is only surgical to prevent any complications. We report the unusual case of a papillary fibroelastoma located on a mitral valve chorda presenting acute psychiatric symptoms as a consequence of vertebrobasilar cerebral territory embolism. The surgical excision was performed through an aortotomy with videoscopy used to help exposure and removal.
2. Case report
A 55-year-old man, suffering from depression and loss of interest at work, unexplainedly ran away from work on two occasions, driving aimlessly and drinking until he was found without aforethought. Clinical examination just pointed out memory disorders. Neurological investigations found a right temporal lobe epilepsy via an electroencephalogram and a right hypothalamic hole without any localized tumor via an MRI, suggesting a posterior cerebral artery territory embolism. Further investigations (CT scan, ultrasounds of carotid arteries) performed to search for an embolic etiology were normal but a transesophageal echocardiography showed a single one centimeter diameter mobile mass on the ventricular side of the anterior mitral valve (Fig. 1a). The diagnosis was thought to be a myxoma and, therefore, the patient was referred to surgery.
Under cardiopulmonary bypass, an exploration was first performed through an aortotomy and the aortic valve with the help of a thoracoscope to ensure the mass nature and the possibility of its resection. It revealed a small myxoid mass attached to one anterior mitral valve second-order chorda. A resection of the tumor preserving the mitral valve was decided based on the macroscopic aspect of the mass suggesting a benign tumor and its single location on a second-order chorda. Excision of the tumor and the chorda was performed using this same approach with thoracoscopic instruments (Fig. 2). The patient was weaned from bypass without difficulty and intraoperative transesophageal echography showed neither evidence of valvular regurgitation nor remaining mass after resection (Fig. 1b).
The histological diagnosis was confirmed to be papillary fibroelastoma, with clear margins. Recovery from the operation was uneventful and the patient was discharged on postoperative day 12. He had no cardiac or new neurological problems during the past 6 years of follow-up. His epilepsy is stabilized under treatment, and his cognitive function assessment has improved with time.
3. Discussion
CPFEs were first described by Yater [1] in 1931 and are the most common cardiac valve tumors and the third most frequent benign cardiac primitive neoplasms after myxomas and fibromas [2]. This tumor is single in 90% of cases and more than 95% occur in the left heart. Its frequency varies from 2.9 to 7.9% but it might be more frequent for two reasons: first because nowadays tumors are discovered incidentally by echocardiography in patients being evaluated for unrelated problems and second because a new review of the pathological anatomy classification has included different tumors as papillary fibroelastomas.
CPFE has a characteristic flower-like appearance or sea anemone with multiple papillary fronds attached to the endocardium by a short pedicle. Papillary fronds are narrow, elongated and branching and are best visualized when immersed in water. The majority of tumors are around 15 mm in their greatest dimension [2], but tumors as large as 40 mm have been reported [3]. On microscopic analysis, CPFEs are avascular papillomas with a single layer of endocardial cells covering the papillary surface. The matrix consists of proteoglycans and some elastic fibers that are longitudinally oriented [4]. Thrombi attached on the tumor could be discovered with microscopic analysis.
Its histogenesis and pathogenesis remain controversial. Immunohistochemical investigations are in favor of a hamartomatous origin [5], whereas a recent finding of cytomegalovirus in a tumor suggested the possibility of a virus-induced tumor mechanism [6]. Most CPFEs are acquired lesions, as demonstrated by at least two cases of new diagnosed tumors eight and twelve years after cardiac surgery. However, there are few congenital cases reported in the literature but no familial occurrence has been noticed.
Mean age of patients at time of diagnosis is 50 years old, with a wide range from birth to 92 years old, without sex ratio. No recurrence has been reported after surgery in patients followed by echocardiography with the longest available follow-up of 11 years.
The potential for life-threatening complications of CPFEs, even asymptomatic ones, is an indication for their surgical excision whatever their size, as long as there are no major contraindications to the operation. Surgery is performed under cardiopulmonary bypass, usually through the left atrium when the mitral valve is concerned. Surgical excision must be complete, and when the valvular tissue is involved, the valve should be preserved if possible. As in our case, a single location on a mitral valve chorda could be removed without mitral valve regurgitation checked by an echography at the end of surgery [7]. The use of a thoracoscope has been described to visualize and remove a CPFE posterior to the mitral valve through the left atrium [8]. We preferred to first have a general look at the left ventricle by an aortotomy with the help of a thoracoscope to make sure there was no other location undetected by the preoperative echography and to visualize directly the mass. This approach also ensured its resectability, followed by its excision without any difficulty using the same approach with thoracoscopic instruments. This particular surgical method seems very interesting to us when the tumor is located in the left ventricle.
Our case is also unusual in its diagnosis. Usually brain embolic complications occur in the middle cerebral artery territory, whereas in our case it concerned the posterior cerebral artery of the vertebrobasilar territory. Clinical features of thalamic infarction could include impaired memory, perseveration, altered social skills and personality as described in our case [9].
4. Conclusion
We present this single case to highlight this particular approach of a ventricular mitral valve mass diagnosed by echocardiography. The exposure, the analysis of the mass and finally, its resection could easily be performed through the aortic valve with the use of videoscopy.
References
Yater WM. Tumors of the heart and pericardium: pathology, symptomatology and report of nine cases. Arch Intern Med 1931; 48:627–666.
Shahian D. Papillary fibroelastomas. Semin Thorac Cardiovasc Surg 2000; 12:101–110.
Crestanello JA, Orszulak TA. Giant papillary fibroelastoma of the right atrium: an unusual presentation. Ann Thorac Surg 2002; 74:1252–1254.
Burke A, Virmani R. Papillary fibroelastoma: tumors of the heart and great vessels. AFIP Atlas of Tumor Pathology 1996; 16:47–54.
Paraf F. Pathology of primary cardiac neoplasms. Ann Pathol 1999; 19:212–222.
Grandmougin D, Fayad G, 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.
Gallo R, Kumar N, Prabhakar G, Awada A, Maalouf Y, Duran C. Papillary fibroelastoma of mitral valve chorda. Ann Thorac Surg 1993; 55:1576–1577.
Espada R, Talwalker NG, Wilcox G, Kleiman NS, Verani MS. Visualization of ventricular fibroelastoma with a video-assisted thoracoscope. Ann Thorac Surg 1997; 63:221–223.
Schmahmann JD. Vascular syndromes of the thalamus. Stroke 2003; 34:2264–2278.(Alexandre Le Guyader, Kar)