Ann Thorac Surg 1997;64:1164-1166
© 1997 The Society of Thoracic Surgeons
Case Report
Atrial Replacement and Tricuspid Valve Reconstruction After Angiosarcoma Resection
P. Michael McFadden, MD,
John L. Ochsner, MD
Division of Thoracic and Cardiovascular Surgery, Department of Surgery, Ochsner Clinic and Alton Ochsner Medical Foundation, New Orleans, Louisiana
Accepted for publication May 2, 1997.
 |
Abstract
|
|---|
A cardiac angiosarcoma was resected and successfully managed by replacement of the right atrium and bileaflet reconstruction of the tricuspid valve by conserving noninvolved valvular tissue. Competency of the new valve was confirmed intraoperatively by transesophageal echocardiography and reconfirmed at discharge. Evaluation 3 months postoperatively revealed no evidence of valvular insufficiency or right heart failure. In selected patients, resection of extensive primary cardiac neoplasms may be possible without necessitating prosthetic valve replacement.
|
Introduction
|
|---|
Angiosarcoma, the most common primary malignant cardiac tumor, is essentially nonresponsive to irradiation and current chemotherapeutic regimens. Surgical resection remains the only hope for palliation or cure. Angiosarcomas are often large at clinical presentation and frequently involve vital cardiac structures, which usually indicates that they are unresectable. Radical attempts at resection may result in severe functional cardiac impairment or even death. We report a case of an extensive angiosarcoma involving the right heart. After resection, the heart defect was successfully repaired by replacement of the right atrial free wall, reconstruction of the tricuspid annulus and right ventricle, and creation of a functionally competent bileaflet atrioventricular valve by conserving noninvolved tricuspid valve tissue.
A 33-year-old man presented with fatigue, fever, and night sweats of 3 weeks' duration. Past medical history and physical examination were unremarkable. The electrocardiogram demonstrated sinus rhythm. Laboratory test results were normal except for a hematocrit of 28.1%, a hemoglobin level of 9.0 mg/dL, and a mildly elevated lactate dehydrogenase level of 331 U/L. Chest films and computed tomographic scans revealed a large 15.1 x 11.2-cm anterior mediastinal mass (Fig 1
). No other abnormalities were present. Before surgical evaluation, a computed tomography-guided needle biopsy of the mass returned necrotic material. Transesophageal echocardiography suggested a right atrial thrombus with normal valvular and left ventricular function.
View larger version (117K):
[in this window]
[in a new window]
|
Fig 1. . Computed tomogram of the chest demonstrating an angiosarcoma (A) of the heart presenting as an anterior mediastinal mass.
|
|
Surgical exploration, undertaken to diagnose and manage the mediastinal tumor, revealed an extensive cardiac angiosarcoma involving the right atrium, right ventricle, tricuspid annulus, and overlying parietal pericardium. The tumor was resected with the patient on femorofemoral cardiopulmonary bypass with superior vena caval drainage and bicaval inflow occlusion. The heart was not arrested and the left atrium was vented transseptally. Tumor resection required removal of the entire right atrial free wall and a segment of the right ventricle, a portion of the tricuspid annulus and anterior leaflet, and a majority of the posterior tricuspid leaflet. The right coronary artery was occluded by tumor and thus resected with the specimen. A portion of the uninvolved anterior tricuspid leaflet was detached from the annulus and preserved with its chordal attachments (Fig 2). The resulting cardiac defect was repaired by plication of the right ventricle, effectively reapproximating the tricuspid annulus. The preserved portion of the anterior tricuspid leaflet was reattached to the newly created tricuspid annulus, thus creating a bileaflet atrioventricular valve. The right atrial free wall was replaced with bovine pericardium (Fig 3). Competency of the reconstructed valve was confirmed by pressure monitoring and transesophageal color-flow echocardiography intraoperatively.
View larger version (47K):
[in this window]
[in a new window]
|
Fig 2. . Resection of extensive cardiac angiosarcoma including right atrial wall, right ventricle, and portions of the tricuspid valve and annulus. Uninvolved anterior and septal tricuspid valve leaflets were preserved. (ATV, PTV, STV = anterior, posterior, and septal tricuspid valve leaflet; RA = right atrium.)
|
|
View larger version (47K):
[in this window]
[in a new window]
|
Fig 3. . Reconstruction of the cardiac defect after tumor resection. The right ventricular defect was plicated and the anterior tricuspid valve leaflet was resuspended to create a bicuspid atrioventricular valve. The right free atrial wall was replaced with bovine pericardium. (ATV = anterior tricuspid valve; RV = right ventricle.)
|
|
Transthoracic echocardiography 6 days postoperatively demonstrated no valvular insufficiency (Fig 4). The peak instantaneous atrioventricular valvular gradient was 10 mm Hg, and the pulmonary artery systolic pressure was 25 mm Hg. The right atrium was akinetic as a result of the pericardial replacement. Left ventricular function was normal (ejection fraction = 0.60), whereas right ventricular contractility was mildly depressed. Patient evaluation 3 months after the operation revealed sinus rhythm and no symptoms or physical findings of right-sided cardiac dysfunction or atrioventricular valvular incompetence. A bone scan to evaluate left scapular pain demonstrated metastasis, and radiotherapy was initiated.
View larger version (60K):
[in this window]
[in a new window]
|
Fig 4. . Postoperative four-chamber echocardiogram (A) with color-flow Doppler image (B) demonstrating normal chamber size and trivial regurgitation from newly reconstructed right atrioventricular valve and atrium. (LA = left atrium; LV = left ventricle; RA = right atrium; RV = right ventricle; TV = tricuspid valve.)
|
|
|
Comment
|
|---|
Primary malignant neoplasms of the heart are rare. Angiosarcoma, the most common, comprises 37% of these tumors [1]. A 32-year retrospective review of our own experience with heart tumors reaffirmed the rarity of primary cardiac tumors. In 9,915 open heart procedures performed at our institution between 1964 and 1997, only 23 cardiac tumors were encountered: 19 (82.6%) benign and 4 (17.4%) malignant. The angiosarcoma reported here was the only primary cardiac malignancy. Angiosarcomas most commonly arise from the right atrium (68%) and involve the overlying pericardium [1], as in our case. Symptoms of malaise, fever, and fatigue often precede the clinical signs and symptoms of pericardial constriction, tamponade, and right heart obstruction [2, 3].
The prognosis for angiosarcoma is poor. Metastases occur in 66% to 89% of patients, and the reported mean survival is 3 to 6.6 months [1, 4]. Unfortunately, angiosarcomas have been essentially nonresponsive to treatment with irradiation and chemotherapeutic agents. Therefore, in the absence of metastases, early and complete resection remains the only hope for palliation or cure. Although successful cardiac transplantation for a cardiac neoplasm has been reported, the utility of this alternative appears limited [5]. Patients with extensive tumors involving vital cardiac structures such as valves, arteries, and conduction tissue are usually denied an attempt at surgical resection. Patients who undergo resection and require concomitant valve replacement are exposed to the risks of anticoagulation and thrombosis attendant with the intracardiac prostheses. In selected patients, a surgical approach that combines conservation of uninvolved cardiac structures with reconstructive techniques may preclude the necessity for prosthetic valve replacement and provide reasonable palliation or even cure. Tumors involving the right heart are best suited for this approach. Provided the right atrioventricular valve is competent, circulatory compromise or right-sided heart failure is avoided. Our experience with valvuloplasty and intraoperative transesophageal echocardiographic assessment of valvular function was invaluable in deciding whether valvular repair, rather than replacement, should be attempted in our patient.
We support an aggressive surgical approach in managing primary cardiac neoplasms. Despite limited success in the surgical resection of some malignant cardiac tumors, development of effective chemotherapeutic agents will be necessary to significantly affect long-term survival because most patients ultimately succumb to distant metastases.
|
Acknowledgments
|
|---|
We thank Barbara Siede for the artwork and Diana Cheatham for technical preparation of the manuscript.
|
Footnotes
|
|---|
Address reprint requests to Dr McFadden, Ochsner Clinic, 1514 Jefferson Highway, New Orleans, LA 70121 (e-mail: dcheatham{at}ochsner.org).
|
References
|
|---|
- Burke A, Virmani R. Primary cardiac sarcomas. In: Tumors of the heart and great vessels. Atlas of tumor pathology, third series, fascicle 16. Washington, DC: Armed Forces Institute of Pathology, 1996:127–70.
- Glancy DL, Morales JB Jr, Roberts WC. Angiosarcoma of the heart. Am J Cardiol 1968;21:413–9.[Medline]
- Herrmann MA, Shankerman RA, Edwards WD, Shub C, Schaff HV. Primary cardiac angiosarcoma: a clinicopathologic study of six cases. J Thorac Cardiovasc Surg 1992;103:655–64.[Abstract]
- Janigan DT, Husain A, Robinson NA. Cardiac angiosarcomas: a review and a case report. Cancer 1986;57:852–9.[Medline]
- 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.[Abstract]
This article has been cited by other articles:
|
|
|
|
 
K. Santo and U. Dandekar
Primary Right Atrial Angiosarcoma
Asian Cardiovasc Thorac Ann,
December 1, 2008;
16(6):
490 - 491.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. Totaro, F. Miraldi, C. Ghiribelli, and C. Biscosi
Cardiac Angiosarcoma Arising From Pulmonary Artery: Endovascular Treatment
Ann. Thorac. Surg.,
October 1, 2004;
78(4):
1468 - 1470.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
P. A. Araoz, H. E. Eklund, T. J. Welch, and J. F. Breen
CT and MR Imaging of Primary Cardiac Malignancies
RadioGraphics,
November 1, 1999;
19(6):
1421 - 1434.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
R. M. Biniwale, H. P. Pathare, N. Aggrawal, A. G. Tendolkar, J. Deshpande, and A. Sivaraman
Cardiac Sarcomas: Is Tumor Debulking Justifiable Therapy?
Asian Cardiovasc Thorac Ann,
March 1, 1999;
7(1):
52 - 55.
[Abstract]
[Full Text]
[PDF]
|
|
|
Top
Abstract
Introduction
