Ann Thorac Surg 2003;76:1609-1613
© 2003 The Society of Thoracic Surgeons
Original article: cardiovascular
Primary cardiac valve tumors: early and late results of surgical treatment in 10 patients
Zhixiong Huang, MDa*,
Lizhong Sun, MDa,
Ming Du, MDa,
Yingmao Ruan, MDb,
Hongyue Wang, MDb
a Department of Cardiovascular Surgery, Cardiovascular Institute, Fu Wai Hospital, Chinese Academy of Medical Science, Peking Union Medical College, Beijing, China
b Department of Pathology, Cardiovascular Institute, Fu Wai Hospital, Chinese Academy of Medical Science, Peking Union Medical College, Beijing, China
Accepted for publication May 28, 2003.
* Address reprint requests to Dr Huang, Department of Cardiovascular Surgery, Cardiovascular Institute, Fu Wai Hospital, A 167 Beilishi Rd, Fuchengmenwai, Beijing 100037, China.
e-mail: huangzhixiong{at}fwxw.org
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Abstract
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BACKGROUND: Our goal was to study the clinical characteristics of primary valve tumors and the late surgical results of their resection.
METHODS: We reviewed our clinical experience with the surgical treatment of ten primary valve tumors at Fu Wai Hospital over the past 19 years. During that time, cardiac valve tumors accounted for 2.65% of all primary cardiac tumors at our hospital, and the incidence of primary valve tumors was roughly one in 4,000 cardiac operations. There were 5 male and 5 female patients aged 2 to 66 years (mean age, 30.1 years). The clinical presentation included exertional dyspnea in 7 patients, neurological symptoms in 2, and cyanosis at rest in 1 patient. The diagnosis was established by preoperative echocardiography in 8 patients, and in the other 2, it was confirmed by the findings at operation. All of the tumors were resected. Eight of the ten tumors were benign, and two were malignant.
RESULTS: All patients survived the operation and recovered uneventfully. Late outcomes were known for all patients. There were three late deaths. One patient with a benign tricuspid valve tumor died 2 months postoperatively of an electrolyte disorder. The other 2 patients with a malignant mitral valve tumor died within 1 year postoperatively. The 7 survivors, all with a benign valve tumor, were followed for an average of 5.7 years (range, 8 months to 19 years), and all were in functional class I. Exercise tolerance improved to normal levels. The latest follow-up echocardiograms showed no evidence of local recurrence in any patient.
CONCLUSIONS: Excellent early and late surgical results can be obtained in patients with benign valve tumors. The prognosis for patients with a malignant valve tumor is poor.
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Introduction
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Cardiac valve tumors are rare, accounting for less than 10% of all primary cardiac tumors [1]. At our hospital, 378 patients underwent surgical resection of a primary cardiac tumor between November 1983 and November 2002. Among them, only 10 (2.65%) had a diagnosis of primary valve tumor. During the same period, 42,023 cardiac operations were performed. Therefore, the incidence of primary valve tumors was roughly one in 4,000 cardiac operations. Most reports about valve tumors are based on scattered cases. Edwards and colleagues [1] reported the largest group with only six cases of tumor resection, but long-term follow-up was not available. Therefore, clinical characteristics of primary valve tumors and late surgical results are still unclear. This report reviews our clinical experience with and the late results of surgical treatment of primary valve tumors at Fu Wai Hospital over the past 19 years.
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Material and methods
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From November 1983 to November 2002, 10 patients underwent surgical resection of primary valve tumors at Fu Wai Hospital. We excluded tumors that were metastatic or infectious. Data were obtained from medical records and recent examination of the slides and tissue blocks. Survivors were contacted by letter to assess functional status, and the results of late echocardiography were reviewed.
There were 5 male and 5 female patients aged 2 to 66 years (mean age, 30.1 years). Eight of the patients had a benign tumor and 2, a malignant tumor. Whether the tumor was benign or malignant, there were no major extracardiac symptoms in this series, such as weight loss, fever, arthralgia, and myalgia. All laboratory results were normal including routine blood study, erythrocyte sedimentation rate, and level of C-reactive protein.
All operations were done with the patient under general anesthesia, with total cardiopulmonary bypass using aortic and bicaval cannulation, and with systemic hypothermia (28°C). There were no hospital deaths.
Follow-up of all patients ranged from 2 months to 19 years, (mean follow-up, 4.3 years). There were three late deaths. The 7 survivors, all of whom had had a benign valve tumor, were in New York Heart Association (NYHA) functional class I. Exercise tolerance had improved to normal levels. At the latest follow-up echocardiographic study, no evidence of local recurrence was found in any patient.
The tumors were grouped into three categories according to location: mitral valve tumors, tricuspid valve tumors, and pulmonary valve tumors. There were no aortic valve tumors in our series.
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Results
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Primary mitral valve tumors
Six primary mitral valve tumors were detected. Four were benign and two, malignant.
Benign primary mitral valve tumors
There were 4 patients, 2 male and 2 female, aged 13 to 66 years (mean age, 43 years) in this subgroup. The presenting symptoms included exertional dyspnea from mitral stenosis in 3 patients who were in NYHA functional class III and hemiplegia from a cerebral embolism in 1 patient whose mitral valve functioned well. The duration of symptoms averaged 58.5 days (range, 10 days to 4 months). An apical diastolic rumble was heard in 3 patients. The electrocardiograms were normal in all patients. The preoperative chest roentgenograms showed cardiothoracic ratios ranging from 0.43 to 0.50, (mean ratio, 0.47); mild pulmonary venous congestion was present in 3 patients. The diagnosis was established by preoperative echocardiography in all patients.
The detailed pathological findings and surgical procedures are shown in Table 1 (see Figure 1).
In patients 2 and 4, the area of involved anterior leaflet was too large to reconstruct the mitral valve after complete resection of the neoplasm. Therefore, mitral valve replacement with complete resection of the subvalvular apparatus was performed.
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Fig 1. (Patient 4, benign mitral valve tumors.) Histology of lymphangioma. The tumor tissue is composed of different sizes and contains cysts filled with gelatinous liquid and some lymphocytes. There are no red blood cells in the cysts. (Hematoxylin and eosin; x200 before % reduction.)
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There were no operative deaths. Follow-up ranged from 4 years to 19 years (mean follow-up, 8.8 years). All patients were in NYHA functional class I. The latest follow-up echocardiograms showed no evidence of local recurrence in any patient. The prosthetic valves were functioning well, and there was only mild mitral regurgitation in the 2 patients without mitral valve replacement.
Malignant primary mitral valve tumors
Both patients in this subgroup were men. One was 22 years old and had hemiplegia after a cerebral embolism for 4 months. Heart function, the electrocardiogram, and the chest roentgenogram were normal. The other patient was 33 years old. He had exertional dyspnea as a result of mitral stenosis for 3 months and was in NYHA functional class III. An apical diastolic rumble was audible, the electrocardiogram indicated right ventricular hypertrophy, and mild pulmonary venous congestion was seen on the preoperative chest roentgenogram. The diagnosis of mitral valve tumor was established by preoperative echocardiography in both patients, but the malignant nature of the tumor was not known. The detailed pathological findings and procedures are provided in Table 2 (see Figure 2).
Adjuvant therapy such as radiotherapy or chemotherapy was not given in accordance with the wishes of the patients.
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Fig 2. (Patient 1, malignant mitral valve tumors.) Histology of mesenchymal sarcoma. Tumor cells of different sizes and shapes have pleomorphic nuclei and much secretion of fluid matrix. Karyokinesis is visible. (Hematoxylin and eosin; x200 before % reduction.)
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Both patients died during the follow-up period, 1 of congestive heart failure related to local recurrence 11 months after operation and the other of cerebral metastasis 12 months postoperatively. In the former, the tumor was considered benign because of its cauliflower like appearance with a short pedicle arising from the anterior leaflet. On the basis of this misdiagnosis, mitral valvuloplasty rather than mitral valve replacement was performed. Six months after the patient was discharged, local recurrence and moderate mitral stenosis were detected by echocardiography. The patient refused to undergo a redo operation. The tumor in patient 2 looked like coral with a broad attachment to the posterior leaflet. Symptoms indicative of intracranial hypertension including severe headache, cerebral vomiting, and metamorphopsia were noted before death. A computed tomographic scan confirmed cerebral metastasis, but there was no evidence of local recurrence on echocardiography at the local hospital. Neither patient had received adjuvant therapy postoperatively.
Benign primary tricuspid valve tumors
Three female patients were in this group.
A 9-year-old girl had complained of exertional dyspnea resulting from an associated ventricular septal defect for 2 years. She was in NYHA functional class III. Physical examination revealed mild cyanosis occasionally during exercise, a grade II systolic murmur at the left sternal edge, and an accentuated second heart sound. The oxygen saturation on femoral artery blood gas analysis was 94.5%. The electrocardiogram revealed combined ventricular hypertrophy. The chest roertgenogram showed generalized cardiac enlargement, increased pulmonary vascular markings, and prominence of the main pulmonary artery. Transthoracic echocardiography indicated a mass on the septal leaflet and a large perimembranous ventricular septal defect 20 mm in diameter with a bidirectional shunt. Cardiac catheterization revealed moderate pulmonary hypertension and high pulmonary vascular resistance, but both decreased remarkably when she inhaled 100% oxygen (mean pulmonary artery pressure, 49.5 mm Hg versus 39.75 mm Hg; pulmonary vascular resistance, 18.4 Wood units m2 versus 9.6 Wood units m2).
A 22-year-old woman had complained of exertional dyspnea related to associated congenital cardiac defects for 1 month. She was in NYHA functional class II. At physical examination, a grade III systolic murmur was heard in the second to fifth left intercostal space, and the second heart sound was accentuated. The electrocardiogram revealed left ventricular hypertrophy. The alterations on the chest roentgenograms were similar to those in patient 1 in this group. Transthoracic echocardiography revealed a large perimembranous ventricular septal defect 22 mm in diameter, an atrial septal defect 15 mm in diameter, and a patent ductus arteriosus 5 mm in diameter.
A 42-year-old woman had experienced exertional dyspnea caused by tricuspid incompetence for 3 years. She was in NYHA functional class III. Jugular venous distention, peripheral edema, hepatomegaly, and a grade III subxiphoid systolic murmur were noted. The electrocardiogram indicated right ventricular hypertrophy. The preoperative chest roentgenogram showed a cardiothoracic ratio of 0.69. Echocardiography established the diagnosis of a tricuspid valve tumor complicated by severe tricuspid regurgitation.
The detailed pathological findings in these patients and the procedures undertaken are shown in Table 3.
Postoperatively, patients 2 and 3 were in NYHA functional class I, and patient 1 was in class III. Follow-up ranged from 2 to 10 months (mean follow-up, 5.3 months). Patient 1 died 2 months postoperatively of an electrolyte disorder resulting from acute gastroenteritis. The last follow-up echocardiogram from each of the other 2 patients showed no evidence of local recurrence or tricuspid incompetence, and both were still in NYHA class I.
Benign primary pulmonary valve tumor
There was only 1 patient in this group. A 2-year-old boy had had cyanosis at rest for 10 months. A grade IV systolic murmur was audible along the left sternal edge. The electrocardiogram, the chest roentgenogram, and the echocardiogram all suggested a typical form of tetralogy of Fallot; the tumor was not found on the preoperative echocardiogram. The diagnosis of tetralogy of Fallot was confirmed at operation, and the abnormalities were corrected successfully. In addition, a red granular neoplasm arising from the right pulmonary cusp on the pulmonary side was found. It measured 0.3 x 0.5 x 0.4 cm and had a smooth surface. The neoplasm was resected, and the cut edges of the leaflet were directly sutured. Histopathologically, the tumor was composed of many big-branched sinusoids, which were filled with erythrocytes. The pathological diagnosis was angioma.
During a 5-year follow-up, the patient was in NYHA functional class I. No evidence of local recurrence was found on the latest echocardiogram.
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Comment
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Primary cardiac valve tumors are quite rare, and most of the available information was obtained at postmortem examinations. To date, the clinical manifestations and surgical guidelines have not been well described.
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Clinical features
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According to previous reports, most valve tumors are asymptomatic [1, 2]. However, the symptoms were definitely due to the tumors in 7 of our 10 patients, which implies the presence of different clinical characteristics of primary valve tumors in the Chinese population. There were three major patterns in the clinical presentation: congestive heart failure, embolism, and systemic symptoms. In our series, congestive heart failure resulting from tumor-induced valvular dysfunction was present in 5 of the 10 patients and valve tumor–related cerebral embolism, in 2. Congestive heart failure could be elicited by tumor-induced regurgitation, as in our series, as well as tumor-related serious obstruction of blood flow across the valve. Cerebral, coronary, pulmonary, and retinal artery embolism related to primary valve tumors has been reported [2]. The emboli came from either tumor fragments or thrombus around the tumor. Mitral valve tumor is most frequently associated with embolism; both patients with a cerebral embolism in our series proved to have a mitral valve tumor. Pathologically, papillary fibroelastoma is the most likely to be related to embolism [2]. Patients with primary valve papillary fibroelastoma, myxoma, or malignant valve tumor can have systemic symptoms such as weight loss, fever, and arthralgia, whereas in our series, there were no major systemic symptoms whether the tumor was benign or malignant.
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Diagnosis
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The advances in diagnostic technology, especially the frequent use of echocardiography, have contributed to the diagnosis of valve tumors in living patients. In the present study, the diagnosis of a valvular mass was established by preoperative echocardiography in 8 patients. In the case of papillary fibroelastoma, the most common form of primary valve tumor, echocardiography alone provided satisfactory information for operation. Sun and colleagues [2] summarized the typical echocardiographic features of these particular tumors: round, oval, or irregular in shape with clear borders and homogeneous texture; small in size in most instances; pedunculate and mobile in nearly half of the cases; and arising from the aortic valve in the majority of cases and from the mitral valve secondly. Of the 2 patients in our series whose tumor was not diagnosed by preoperative echocardiography, associated lesions masked the tumors. In 1, a tricuspid valve tumor was associated with a large ventricular septal defect, an atrial septal defect, and a patent ductus arteriosies, and in the other, a pulmonary valve tumor was associated with tetralogy of Fallot. The following reasons could explain why echocardiography did not detect the tumors preoperatively: the tumor was masked by associated lesions; the tumor was too small to be seen at routine echocardiography; and the examination was not done with a sufficient index of suspicion.
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Operative findings
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The pathological nature of primary valve tumors is difficult to determine on the basis of preoperative clinical presentation and echocardiography. The characteristic appearance of some tumors could suggest the probable diagnosis. For instance, papillary fibroelastoma typically resembles a sea anemone with multiple frondlike projections. Myxoma, the second most common primary valve tumor, is round or oval, polypoid, pedunculate, and mobile. In our study, the mitral valve myxoma was light yellow and gelatinous, and looked like fish eggs. Lipoma is easily differentiated from other forms by its appearance and contents. A red granular neoplasm with a smooth surface is most likely an angioma. All cystic tumors were benign in our patients.
In some circumstances, it is still difficult to determine whether the tumor is benign or malignant by only its gross appearance. In our series, two malignant primary mitral valve tumors were misdiagnosed as benign tumors. Therefore, frozen sections of the tumor must be obtained for differentiation in any questionable case.
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Surgical guidelines
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It is generally agreed that all valve tumors must be resected [1, 2]. If the valve tumor is malignant, complete resection of the tumor and replacement of the involved valve is the best choice. For a malignant atrioventricular valve tumor, complete resection of the tumor and replacement of the involved valve with complete resection of the subvalvular apparatus is advised. In this series, 1 patient had a malignant mitral valve tumor that was thought to be benign, and only tumor resection was performed. The patient died of local recurrence within a year after operation.
In the case of benign primary valve tumors, the surgical strategy is to excise the tumor completely, and every effort should be made to preserve the original valve. The performance of valve reconstruction or replacement depends largely on the area of attachment of the tumor to the leaflet. If the involved area is too large, it is impossible to reconstruct the valve after complete tumor resection, and valve replacement with complete resection of the subvalvular apparatus should be done. For cystic valve tumors, removal of the anterior wall and the contents along with cauterization of the posterior wall is a good choice.
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Prognosis
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Excellent early and late results have been achieved in patients with benign valve tumors. Our 7 survivors with benign valve tumors were followed for an average of 5.7 years, and all were in NYHA functional class I. The latest follow-up echocardiograms show no evidence of local recurrence in any patient.
The prognosis for patients with a malignant valve tumor is poor. The 2 patients with a malignant mitral valve tumor died within a year after operation. One died of local recurrence and the other, of distant metastasis. Both patients refused adjuvant therapy. It is reasonable to presume that the surgical results for primary malignant valve tumors would improve if complete tumor resection and affected valve replacement were combined with adjuvant therapy.
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References
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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]
- Sun J.P., Asher C.R., Yang X.S.H., et al. Clinical and echocardiographic characteristics of papillary fibroelastomas: a retrospective and prospective study in 162 patients. Circulation 2001;103:2687-2693.[Abstract/Free Full Text]
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Abstract
Introduction
