- Email: [email protected]
Intracardiac extension of an intracaval sarcoma of endometrial origin
742
CASEREPORT PHILLIPSET AL INTRACARDIACEXTENSIONOF UTERINESARCOMA
tricuspid valve replacement, because the geometry and function of the right ventricle are better preserved and complications inherent in prosthetic heart valves are avoided. Also, a shorter duration between injury and operation m a y improve long-term results of operative treatment, not only because right ventricular function is then better preserved, but also because of a higher probability of feasibility of repair because of decreased likelihood that the involved valvular structures are found in a contracted and atrophied state, findings that often preclude repair [4]. Additionally, early repair limits right atrial fibrillation, which increases the likelihood that these patients can be maintained in sinus rhythm. Although tricuspid valve repair had been performed sporadically as early as the late 1950s and 1960s [6, 7], during the last decade more patients have benefited from repair due to increased awareness that valve repair, w h e n feasible, is superior to valve replacement, improved techniques in reparative valve surgery, and widespread use of intraoperative echocardiography. The operative technique generally will be dictated by the specific injury discovered at the time of operation. Repair usually can be e~ected if the injury is limited to rupture of chordae tendineae or papillary muscle or laceration of a leaflet and if the involved structures are not severely contracted or atrophied. Previously reported techniques of repair of the traumatically injured tricuspid valve consist of plication or partial resection of redundant leaflets, "mitralization" of the tricuspid valve by suturing the anterior leaflet to the septal leaflet with creation of a single anteroseptal leaflet, reinsertion of detached leaflets, plication of elongated chordae, and use of autogenous pericardial strips to replace ruptured chordae [1-4]. Limited information is available about long-term results of chordal replacement for the tricuspid valve. In children we have obtained excellent results with replacement of ruptured or elongated tricuspid chordae by polytetrafluoroethylene artificial chordae (Gore-Tex; W.L. Gore & Assoc, Newark, DE) (Van Son JAM, Hanley FL; unpublished data). Encouraging late results have been reported for chordal replacement in the mitral position, using glutaraldehyde-tanned xenograft pericardium or polytetrafluoroethylene [8]. The technique of tricuspid valve repair as reported here has the advantage that autogenous trabecular muscle bundles are used, which are abundantly present in the right ventricle, thus avoiding the use of foreign material. This technique is applicable in situations where, due to a long duration between injury and tricuspid valve repair, the anterior papillary muscle is contracted and atrophied. References
1. Katz NM, Pallas RS. Traumatic rupture of the tricuspid valve: repair by chordal replacements and annuloplasty. J Thorac Cardiovasc Surg 1986;91:310-4. 2. Noera G, Sanguinetti M, Pensa P, et al. Tricuspid valve incompetence caused by nonpenetrating thoracic trauma. Ann Thorac Surg 1991;51:320-2. 3. Dontigny L, Baillot R, Panneton J, Page P, Cossette R. Surgical repair of traumatic tricuspid insufficiency: report of three cases. J Trauma 1992;33:266-9. © 1995 by The Society of Thoracic Surgeons
Ann Thorac Surg 1995;59:742-4
4. Van Son JAM, Danielson GK, Schat~ HV, Miller FA Jr. Traumatic tricuspid insufficiency: experience in 13 patients. J Thorac Cardiovasc Surg 1994;108:893-8. 5. Miller FA Jr, Seward JB, Gersh BJ, Tajik AJ, Mucha P Jr. Two-dimensional echocardiographic findings in cardiac trauma. Am J Cardiol 1982;50:1022-7. 6. Parmley LF, Manion WC, Mattingly TW. Nonpenetraling traumatic injury of the heart. Circulation 1958;18:371-96. 7. Osborn JR, Jones RC, Jahnke EJ Jr. Traumatic tricuspid insufficiency. Hemodynamic data and surgical management. Circulation 1964;30:317-22. 8. Frater RWM, Vetter HO, Zussa C, Dahm M. Chordal replacement in mitral valve repair. Circulation 1990;82(Suppl 4):125--30.
Intracardiac Extension of an Intracaval Sarcoma of Endometrial Origin Michael R. Phillips, MD, Thomas C. Bower, MD, Thomas A. Orszulak, MD, and Lynn C. Hartmann, MD Division of Vascular Surgery, Section of Cardiovascular Surgery, and Division of Medical Oncology, Mayo Clinic and Mayo Foundation, Rochester, Minnesota A 52-year-old w o m a n was diagnosed with a cardiac tumor by transesophageal echocardiography and magnetic resonance imaging. An intraoperative biopsy indicated the mass was low-grade endometrial stromal sarcoma. Successful tumor removal from the heart and inferior vena cava was accomplished with the use of hypothermic circulatory arrest. The chronically occluded infrarenal portion of the inferior vena cava was transected. The patient has no evidence of recurrent disease after a follow-up of 18 months and has no significant restriction in her daily activities.
(Ann Thorac Surg 1995;59:742-4) ntracardiac masses are found in less than 0.5% of the general population [1]. We report a patient who had successful removal of a low-grade endometrial stromal cell sarcoma that extended from the iliac veins through the vena cava and into the right ventricle, with prolapse across the tricuspid valve. A 52-year-old woman, gravida IV, para IV, presented with a 5-day history of progressive left leg and thigh swelling, pain, and tenderness. The patient's past medical history was significant for a diagnosis of a low-grade endometrial stromal cell sarcoma found incidentally after a total abdominal hysterectomy for menometrorrhagia and, 2 years later, subsequent operations for local tumor recurrence. Pulmonary wedge resection was performed for metastatic disease 2 years before the present admission. At admission to our hospital, she was normotensive, had a regular heart rate and rhythm, and was in no acute
I
Accepted for publication June 30, 1994. Address reprint requests to Dr Bower, Mayo Clinic, 200 First St SW, Rochester, MN 55905. 0003-4975/95/$9.50 0003-4975(94)00580-Z
Ann Thorac Surg 1995;59:742-4
distress. Pulses were intact with no jugular v e n o u s distention. Cardiac examination r e v e a l e d a g r a d e 2/6 systolic ejection m u r m u r a n d an S 3 heart sound. N u m e r o u s v e n o u s collaterals were n o t e d along the right a b d o m i n a l w a l l a n d the left thigh a n d leg were e d e m a t o u s b u t not tender. Varicosities were evident in both lower extremities. Electrocardiography s h o w e d a n o r m a l sinus rhythm. Laboratory studies were u n r e m a r k a b l e except for increased concentrations of s e r u m alkaline p h o s p h a t a s e , 436 U/L (normal, 90 to 234 U/L), a n d s e r u m aspartate aminotransferase, 294 U/L (normal, 12 to 31 U/L). Transthoracic e c h o c a r d i o g r a p h y r e v e a l e d h y p e r d y n a m i c left ventricular function with an ejection fraction of 0.75, b u t there was no evidence of an intracardiac mass. However, t r a n s e s o p h a g e a l e c h o c a r d i o g r a p h y d i d show a m a s s ext e n d i n g from the inferior vena cava (IVC) t h r o u g h the right atrium a n d tricuspid valve into the apex of the right ventricle (Figs 1, 2). The m a s s was not a d h e r e n t to the c h a m b e r s on the right side of the h e a r t b u t d i d obstruct the tricuspid valve intermittently. O n magnetic resonance images, the m a s s e x t e n d e d from the level of the right external iliac vein t h r o u g h the IVC into the right side of the heart. N o flow could b e seen in the infrarenal IVC. V e n o g r a p h y confirmed occlusion of the iliac v e n o u s system with v e n o u s d r a i n a g e t h r o u g h the a b d o m i n a l wall collaterals into the intercostal veins a n d azygos system. C o m p u t e d t o m o g r a p h y - g u i d e d n e e d l e b i o p s y r e v e a l e d multiple spindle cells consistent with a sarcoma. The intracaval a n d intracardiac mass was resected in continuity d u r i n g a single operation with two surgical teams. Initially, control of the retrohepatic, suprarenal, a n d infrarenal portions of the IVC was o b t a i n e d t h r o u g h a midline incision b y using m e d i a l viscera rotation. After the a b d o m i n a l dissection was completed, the patient was p l a c e d on c a r d i o p u l m o n a r y b y p a s s with h y p o t h e r m i c circulatory arrest b y using a single v e n o u s cannula in the s u p e r i o r vena cava. The right atrium a n d s u p r a r e n a l IVC were o p e n e d simultaneously. The t u m o r was r e m o v e d
CASE REPORT PHILLIPSET AL INTRACARDIACEXTENSIONOF UTERINE SARCOMA
743
iiiiiiiiiiiill iiJi~i~ii!i;ii ~ i!!i!ii~i!iiill
Fig 2. Schematic diagram representing the tumor as it extends into the right atrium with prolapse across the triscupid valve. (By permission of Mayo Foundation.)
from the right side of the heart, 1VC, a n d c o m m o n iliac vein b y b l u n t dissection. Because the infrarenal portion of the IVC h a d b e e n occluded chronically a n d to p r e v e n t further growth of t u m o r into the heart t h r o u g h the vena cava, the vena cava was transected just b e l o w the renal veins a n d oversewn proximal a n d distally. Final histopathologic results were consistent with metastatic lowgrade e n d o m e t r i a l stromal cell sarcoma. The patient tolerated the operation well a n d h a d an uneventful recovery. At 18-month follow-up, the patient r e m a i n s a s y m p t o m a t i c a n d free of disease.
Comment
Fig 1. Transesophageal echocardiogram of intracardiac extension of malignant endometrial stromal sarcoma (T). (IVC = inferior vena cava; R A = right atrium; RV = right ventricle; T = tumor; T V = tricuspid valve.)
Uterine t u m o r s rarely extend into the h e a r t t h r o u g h the major veins. E n d o m e t r i a l stromal cell sarcoma r e p r e s e n t s only 3% of all uterine t u m o r s a n d has a p r o p e n s i t y for recurrence, often m a n y years after complete surgical r e m o v a l [2]. Despite its a p p a r e n t t e n d e n c y for lymphatic a n d v e n o u s extension, e n d o m e t r i a l stromal sarcoma has b e e n o b s e r v e d rarely with metastases to the larger vessels [2]. To the b e s t of our knowledge, this patient r e p r e s e n t s the fourth r e p o r t e d case of intracardiac extension of e n d o m e t r i a l sarcoma a n d is only the second patient to survive the initial p r o c e d u r e [3]. Intracavitary cardiac malignancies that have e x t e n d e d into the heart t h r o u g h the major veins cause few specific cardiac s y m p t o m s or signs. Fatigue, palpitations, dizzi-
744
CASEREPORT BLANCHEET AL WOLFF-PARKINSON-WHITEIN ALLOGRAFT
ness, conduction defects, arrhythmias, a n d symptoms from emboli are the most c o m m o n clinical presentations [1]. S u d d e n cardiac d e c o m p e n s a t i o n or death from obstruction of the heart valves can occur a n d is a major reason to consider resection of the t u m o r in addition to control of the malignancy. Various studies are necessary to d o c u m e n t the extent of the tumor, within the vena cava a n d the heart, a n d to exclude metastatic disease in other locations. Transsternal or transesophageal echocardiography provides useful d e t e r m i n a t i o n s of cardiac function a n d the location a n d intracardiac extent of the tumor. Magnetic resonance i m a g i n g has b e e n f o u n d by several authors to define accurately the u p p e r extent of malignancies i n v a d i n g or growing in the vena cava, especially those that involve the retrohepatic vena cava a n d the hepatic veins [4-6]. C o m p u t e d t o m o g r a p h i c scan, u l t r a s o n o g r a p h y , a n d cavography can also be used in selected patients a n d m a y be i m p o r t a n t if concomitant reconstruction of the vena cava is necessary. Operative resection of these tumors is based on the overall medical condition of the patient a n d the longterm prognosis from the cancer. Those patients with a good performance status a n d a low-grade m a l i g n a n c y with no other evidence of metastatic disease a n d those in w h o m no other a d j u v a n t therapy is useful are candidates for resection. C a r d i o p u l m o n a r y bypass with or without hypothermic circulatory arrest has b e e n used successfully a n d safely by several centers in the treatment of tumors with caval a n d intracardiac extension [7-10]. Hypothermic circulatory arrest was indicated in our patient to facilitate a wide exposure with s i m u l t a n e o u s t u m o r extraction from the right side of the heart a n d IVC without significant blood loss. Concomitant partial or complete vena caval resection a n d replacement m a y be necessary if there is a direct invasion of the wall of the vena cava. In most patients, however, because of the t u m o r encapsulation, the mass can be r e m o v e d from within the l u m e n of the vena cava a n d the heart by b l u n t dissection. In select patients with intracaval a n d intracardiac extension of malignancies, surgical resection may offer the only chance of t u m o r control.
References 1. Panidis IP, Kofler MN, Mintz GS, Ross J. Clinical and echocardiographic features of fight atrial masses. Am Heart J 1984;107:745-58. 2. Berchuck A, Rubin SC, Hoskins WJ, Saigo PE, Pierce VK, Lewis JL Jr. Treatment of endometrial stromal tumors. Gynecol Oncol 1990;36:60-5. 3. Vargas-Barron J, Keirns C, Barragan-Garcia R, et al. Intracardiac extension of malignant uterine tumors: echocardiographic detection and successful surgical resection. J Thorac Cardiovasc Surg 1990;99:1099-103. 4. Horan JJ, Robertson CN, Choyke PL, et al. The detection of renal carcinoma extension into the renal vein and inferior vena cava: a prospective comparison of venacavography and magnetic resonance imaging. J Urol 1989;142:943-7. 5. Bretan PN Jr, Williams RD, Hricak HH. Preoperative assessment of retroperitoneal pathology by magnetic resonance imaging: primary leiomyosarcoma of inferior vena cava. Urology 1986;28:251-5. © 1995 by The Society of Thoracic Surgeons
Ann Thorac Surg 1995;59:744-6
6. Myneni L, Hricak H, Carroll PR. Magnetic resonance imaging of renal carcinoma with extension into the vena cava: staging accuracy and recent advances. Br J Urol 1991;68: 571-8. 7. Lansing AM, Witten FR, Masri ZH, Hubbard JG. Resection of renal cell carcinoma with vena cava extension using circulatory arrest. J Ky Med Assoc 1991;89:369-72. 8. Stewart JR, Carey JA, McDougal WS, Merrill WH, Koch MO, Bender HW Jr. Cavoatrial tumor thrombectomy using cardiopulmonary bypass without circulatory arrest. Ann Thorac Surg 1991;51:717-21. 9. Shida T, Yoshimura M, Chihara H, Nakamura K. Intravenous leiomyomatosis of the pelvis with reextension into the heart. Ann Thorac Surg 1986;42:104-6. 10. Stegmann T, Garcia-Gallont R, D/Sring W. Intravascular leiomyomatosis: report of a case and review of the literature. Thorac Cardiovasc Surg 1987;35:157-60.
Wolff-Parkinson-White Syndrome in a Cardiac Allograft Carlos Blanche, MD, C h u n Hwang, MD, Mario Valenza, MD, Robert M. Kass, MD, Lawrence S. C. Czer, MD, William J. Mandel, MD, a n d Alfredo Trento, MD Department of Cardiothoracic Surgery and Division of Cardiology, Cedars-Sinai Medical Center, Los Angeles, California A 61-year-old man underwent orthotopic heart transplantation for end-stage ischemic cardiomyopathy. The donor presented with Wolff-Parkinson-White syndrome and the allograft was successfully transplanted. The acces-
sory pathway was interrupted postoperatively by radiofrequency current catheter ablation, and the patient is clinically well and free of preexcitation 24 months later. (Ann Thorac Surg 1995;59:744-6) lthough heart transplantation has evolved into an accepted treatment modality for patients with endstage heart disease, the shortage of donor hearts has become the most critical factor in limiting the n u m b e r of procedures performed. The use of " b o r d e r l i n e " high-risk donors as a potential source of cardiac aUografts has resulted in excellent graft function a n d patient survival [1]. We report the case of a donor who presented with Wolff-Parkinson-White s y n d r o m e with supraventricular tachycardia. The allograft was t r a n s p l a n t e d successfully, a n d the accessory pathway was ablated postoperatively.
A
A 61-year-old m a n presented with end-stage ischemic cardiomyopathy. He had u n d e r g o n e implantation of a cardioverter-defibrillator for an episode of s u d d e n carAccepted for publicationJune 30, 1994. Address reprint requests to Dr Blanche, Heart Transplant Program, Cardiothoracic Surgery, Cedars-SinaiMedicalCenter, 8700BeverlyBlvd, #6215, Los Angeles,CA 90048. 0003-4975/95/$9.50 0003-4975(94)00570-W
CASEREPORT PHILLIPSET AL INTRACARDIACEXTENSIONOF UTERINESARCOMA
tricuspid valve replacement, because the geometry and function of the right ventricle are better preserved and complications inherent in prosthetic heart valves are avoided. Also, a shorter duration between injury and operation m a y improve long-term results of operative treatment, not only because right ventricular function is then better preserved, but also because of a higher probability of feasibility of repair because of decreased likelihood that the involved valvular structures are found in a contracted and atrophied state, findings that often preclude repair [4]. Additionally, early repair limits right atrial fibrillation, which increases the likelihood that these patients can be maintained in sinus rhythm. Although tricuspid valve repair had been performed sporadically as early as the late 1950s and 1960s [6, 7], during the last decade more patients have benefited from repair due to increased awareness that valve repair, w h e n feasible, is superior to valve replacement, improved techniques in reparative valve surgery, and widespread use of intraoperative echocardiography. The operative technique generally will be dictated by the specific injury discovered at the time of operation. Repair usually can be e~ected if the injury is limited to rupture of chordae tendineae or papillary muscle or laceration of a leaflet and if the involved structures are not severely contracted or atrophied. Previously reported techniques of repair of the traumatically injured tricuspid valve consist of plication or partial resection of redundant leaflets, "mitralization" of the tricuspid valve by suturing the anterior leaflet to the septal leaflet with creation of a single anteroseptal leaflet, reinsertion of detached leaflets, plication of elongated chordae, and use of autogenous pericardial strips to replace ruptured chordae [1-4]. Limited information is available about long-term results of chordal replacement for the tricuspid valve. In children we have obtained excellent results with replacement of ruptured or elongated tricuspid chordae by polytetrafluoroethylene artificial chordae (Gore-Tex; W.L. Gore & Assoc, Newark, DE) (Van Son JAM, Hanley FL; unpublished data). Encouraging late results have been reported for chordal replacement in the mitral position, using glutaraldehyde-tanned xenograft pericardium or polytetrafluoroethylene [8]. The technique of tricuspid valve repair as reported here has the advantage that autogenous trabecular muscle bundles are used, which are abundantly present in the right ventricle, thus avoiding the use of foreign material. This technique is applicable in situations where, due to a long duration between injury and tricuspid valve repair, the anterior papillary muscle is contracted and atrophied. References
1. Katz NM, Pallas RS. Traumatic rupture of the tricuspid valve: repair by chordal replacements and annuloplasty. J Thorac Cardiovasc Surg 1986;91:310-4. 2. Noera G, Sanguinetti M, Pensa P, et al. Tricuspid valve incompetence caused by nonpenetrating thoracic trauma. Ann Thorac Surg 1991;51:320-2. 3. Dontigny L, Baillot R, Panneton J, Page P, Cossette R. Surgical repair of traumatic tricuspid insufficiency: report of three cases. J Trauma 1992;33:266-9. © 1995 by The Society of Thoracic Surgeons
Ann Thorac Surg 1995;59:742-4
4. Van Son JAM, Danielson GK, Schat~ HV, Miller FA Jr. Traumatic tricuspid insufficiency: experience in 13 patients. J Thorac Cardiovasc Surg 1994;108:893-8. 5. Miller FA Jr, Seward JB, Gersh BJ, Tajik AJ, Mucha P Jr. Two-dimensional echocardiographic findings in cardiac trauma. Am J Cardiol 1982;50:1022-7. 6. Parmley LF, Manion WC, Mattingly TW. Nonpenetraling traumatic injury of the heart. Circulation 1958;18:371-96. 7. Osborn JR, Jones RC, Jahnke EJ Jr. Traumatic tricuspid insufficiency. Hemodynamic data and surgical management. Circulation 1964;30:317-22. 8. Frater RWM, Vetter HO, Zussa C, Dahm M. Chordal replacement in mitral valve repair. Circulation 1990;82(Suppl 4):125--30.
Intracardiac Extension of an Intracaval Sarcoma of Endometrial Origin Michael R. Phillips, MD, Thomas C. Bower, MD, Thomas A. Orszulak, MD, and Lynn C. Hartmann, MD Division of Vascular Surgery, Section of Cardiovascular Surgery, and Division of Medical Oncology, Mayo Clinic and Mayo Foundation, Rochester, Minnesota A 52-year-old w o m a n was diagnosed with a cardiac tumor by transesophageal echocardiography and magnetic resonance imaging. An intraoperative biopsy indicated the mass was low-grade endometrial stromal sarcoma. Successful tumor removal from the heart and inferior vena cava was accomplished with the use of hypothermic circulatory arrest. The chronically occluded infrarenal portion of the inferior vena cava was transected. The patient has no evidence of recurrent disease after a follow-up of 18 months and has no significant restriction in her daily activities.
(Ann Thorac Surg 1995;59:742-4) ntracardiac masses are found in less than 0.5% of the general population [1]. We report a patient who had successful removal of a low-grade endometrial stromal cell sarcoma that extended from the iliac veins through the vena cava and into the right ventricle, with prolapse across the tricuspid valve. A 52-year-old woman, gravida IV, para IV, presented with a 5-day history of progressive left leg and thigh swelling, pain, and tenderness. The patient's past medical history was significant for a diagnosis of a low-grade endometrial stromal cell sarcoma found incidentally after a total abdominal hysterectomy for menometrorrhagia and, 2 years later, subsequent operations for local tumor recurrence. Pulmonary wedge resection was performed for metastatic disease 2 years before the present admission. At admission to our hospital, she was normotensive, had a regular heart rate and rhythm, and was in no acute
I
Accepted for publication June 30, 1994. Address reprint requests to Dr Bower, Mayo Clinic, 200 First St SW, Rochester, MN 55905. 0003-4975/95/$9.50 0003-4975(94)00580-Z
Ann Thorac Surg 1995;59:742-4
distress. Pulses were intact with no jugular v e n o u s distention. Cardiac examination r e v e a l e d a g r a d e 2/6 systolic ejection m u r m u r a n d an S 3 heart sound. N u m e r o u s v e n o u s collaterals were n o t e d along the right a b d o m i n a l w a l l a n d the left thigh a n d leg were e d e m a t o u s b u t not tender. Varicosities were evident in both lower extremities. Electrocardiography s h o w e d a n o r m a l sinus rhythm. Laboratory studies were u n r e m a r k a b l e except for increased concentrations of s e r u m alkaline p h o s p h a t a s e , 436 U/L (normal, 90 to 234 U/L), a n d s e r u m aspartate aminotransferase, 294 U/L (normal, 12 to 31 U/L). Transthoracic e c h o c a r d i o g r a p h y r e v e a l e d h y p e r d y n a m i c left ventricular function with an ejection fraction of 0.75, b u t there was no evidence of an intracardiac mass. However, t r a n s e s o p h a g e a l e c h o c a r d i o g r a p h y d i d show a m a s s ext e n d i n g from the inferior vena cava (IVC) t h r o u g h the right atrium a n d tricuspid valve into the apex of the right ventricle (Figs 1, 2). The m a s s was not a d h e r e n t to the c h a m b e r s on the right side of the h e a r t b u t d i d obstruct the tricuspid valve intermittently. O n magnetic resonance images, the m a s s e x t e n d e d from the level of the right external iliac vein t h r o u g h the IVC into the right side of the heart. N o flow could b e seen in the infrarenal IVC. V e n o g r a p h y confirmed occlusion of the iliac v e n o u s system with v e n o u s d r a i n a g e t h r o u g h the a b d o m i n a l wall collaterals into the intercostal veins a n d azygos system. C o m p u t e d t o m o g r a p h y - g u i d e d n e e d l e b i o p s y r e v e a l e d multiple spindle cells consistent with a sarcoma. The intracaval a n d intracardiac mass was resected in continuity d u r i n g a single operation with two surgical teams. Initially, control of the retrohepatic, suprarenal, a n d infrarenal portions of the IVC was o b t a i n e d t h r o u g h a midline incision b y using m e d i a l viscera rotation. After the a b d o m i n a l dissection was completed, the patient was p l a c e d on c a r d i o p u l m o n a r y b y p a s s with h y p o t h e r m i c circulatory arrest b y using a single v e n o u s cannula in the s u p e r i o r vena cava. The right atrium a n d s u p r a r e n a l IVC were o p e n e d simultaneously. The t u m o r was r e m o v e d
CASE REPORT PHILLIPSET AL INTRACARDIACEXTENSIONOF UTERINE SARCOMA
743
iiiiiiiiiiiill iiJi~i~ii!i;ii ~ i!!i!ii~i!iiill
Fig 2. Schematic diagram representing the tumor as it extends into the right atrium with prolapse across the triscupid valve. (By permission of Mayo Foundation.)
from the right side of the heart, 1VC, a n d c o m m o n iliac vein b y b l u n t dissection. Because the infrarenal portion of the IVC h a d b e e n occluded chronically a n d to p r e v e n t further growth of t u m o r into the heart t h r o u g h the vena cava, the vena cava was transected just b e l o w the renal veins a n d oversewn proximal a n d distally. Final histopathologic results were consistent with metastatic lowgrade e n d o m e t r i a l stromal cell sarcoma. The patient tolerated the operation well a n d h a d an uneventful recovery. At 18-month follow-up, the patient r e m a i n s a s y m p t o m a t i c a n d free of disease.
Comment
Fig 1. Transesophageal echocardiogram of intracardiac extension of malignant endometrial stromal sarcoma (T). (IVC = inferior vena cava; R A = right atrium; RV = right ventricle; T = tumor; T V = tricuspid valve.)
Uterine t u m o r s rarely extend into the h e a r t t h r o u g h the major veins. E n d o m e t r i a l stromal cell sarcoma r e p r e s e n t s only 3% of all uterine t u m o r s a n d has a p r o p e n s i t y for recurrence, often m a n y years after complete surgical r e m o v a l [2]. Despite its a p p a r e n t t e n d e n c y for lymphatic a n d v e n o u s extension, e n d o m e t r i a l stromal sarcoma has b e e n o b s e r v e d rarely with metastases to the larger vessels [2]. To the b e s t of our knowledge, this patient r e p r e s e n t s the fourth r e p o r t e d case of intracardiac extension of e n d o m e t r i a l sarcoma a n d is only the second patient to survive the initial p r o c e d u r e [3]. Intracavitary cardiac malignancies that have e x t e n d e d into the heart t h r o u g h the major veins cause few specific cardiac s y m p t o m s or signs. Fatigue, palpitations, dizzi-
744
CASEREPORT BLANCHEET AL WOLFF-PARKINSON-WHITEIN ALLOGRAFT
ness, conduction defects, arrhythmias, a n d symptoms from emboli are the most c o m m o n clinical presentations [1]. S u d d e n cardiac d e c o m p e n s a t i o n or death from obstruction of the heart valves can occur a n d is a major reason to consider resection of the t u m o r in addition to control of the malignancy. Various studies are necessary to d o c u m e n t the extent of the tumor, within the vena cava a n d the heart, a n d to exclude metastatic disease in other locations. Transsternal or transesophageal echocardiography provides useful d e t e r m i n a t i o n s of cardiac function a n d the location a n d intracardiac extent of the tumor. Magnetic resonance i m a g i n g has b e e n f o u n d by several authors to define accurately the u p p e r extent of malignancies i n v a d i n g or growing in the vena cava, especially those that involve the retrohepatic vena cava a n d the hepatic veins [4-6]. C o m p u t e d t o m o g r a p h i c scan, u l t r a s o n o g r a p h y , a n d cavography can also be used in selected patients a n d m a y be i m p o r t a n t if concomitant reconstruction of the vena cava is necessary. Operative resection of these tumors is based on the overall medical condition of the patient a n d the longterm prognosis from the cancer. Those patients with a good performance status a n d a low-grade m a l i g n a n c y with no other evidence of metastatic disease a n d those in w h o m no other a d j u v a n t therapy is useful are candidates for resection. C a r d i o p u l m o n a r y bypass with or without hypothermic circulatory arrest has b e e n used successfully a n d safely by several centers in the treatment of tumors with caval a n d intracardiac extension [7-10]. Hypothermic circulatory arrest was indicated in our patient to facilitate a wide exposure with s i m u l t a n e o u s t u m o r extraction from the right side of the heart a n d IVC without significant blood loss. Concomitant partial or complete vena caval resection a n d replacement m a y be necessary if there is a direct invasion of the wall of the vena cava. In most patients, however, because of the t u m o r encapsulation, the mass can be r e m o v e d from within the l u m e n of the vena cava a n d the heart by b l u n t dissection. In select patients with intracaval a n d intracardiac extension of malignancies, surgical resection may offer the only chance of t u m o r control.
References 1. Panidis IP, Kofler MN, Mintz GS, Ross J. Clinical and echocardiographic features of fight atrial masses. Am Heart J 1984;107:745-58. 2. Berchuck A, Rubin SC, Hoskins WJ, Saigo PE, Pierce VK, Lewis JL Jr. Treatment of endometrial stromal tumors. Gynecol Oncol 1990;36:60-5. 3. Vargas-Barron J, Keirns C, Barragan-Garcia R, et al. Intracardiac extension of malignant uterine tumors: echocardiographic detection and successful surgical resection. J Thorac Cardiovasc Surg 1990;99:1099-103. 4. Horan JJ, Robertson CN, Choyke PL, et al. The detection of renal carcinoma extension into the renal vein and inferior vena cava: a prospective comparison of venacavography and magnetic resonance imaging. J Urol 1989;142:943-7. 5. Bretan PN Jr, Williams RD, Hricak HH. Preoperative assessment of retroperitoneal pathology by magnetic resonance imaging: primary leiomyosarcoma of inferior vena cava. Urology 1986;28:251-5. © 1995 by The Society of Thoracic Surgeons
Ann Thorac Surg 1995;59:744-6
6. Myneni L, Hricak H, Carroll PR. Magnetic resonance imaging of renal carcinoma with extension into the vena cava: staging accuracy and recent advances. Br J Urol 1991;68: 571-8. 7. Lansing AM, Witten FR, Masri ZH, Hubbard JG. Resection of renal cell carcinoma with vena cava extension using circulatory arrest. J Ky Med Assoc 1991;89:369-72. 8. Stewart JR, Carey JA, McDougal WS, Merrill WH, Koch MO, Bender HW Jr. Cavoatrial tumor thrombectomy using cardiopulmonary bypass without circulatory arrest. Ann Thorac Surg 1991;51:717-21. 9. Shida T, Yoshimura M, Chihara H, Nakamura K. Intravenous leiomyomatosis of the pelvis with reextension into the heart. Ann Thorac Surg 1986;42:104-6. 10. Stegmann T, Garcia-Gallont R, D/Sring W. Intravascular leiomyomatosis: report of a case and review of the literature. Thorac Cardiovasc Surg 1987;35:157-60.
Wolff-Parkinson-White Syndrome in a Cardiac Allograft Carlos Blanche, MD, C h u n Hwang, MD, Mario Valenza, MD, Robert M. Kass, MD, Lawrence S. C. Czer, MD, William J. Mandel, MD, a n d Alfredo Trento, MD Department of Cardiothoracic Surgery and Division of Cardiology, Cedars-Sinai Medical Center, Los Angeles, California A 61-year-old man underwent orthotopic heart transplantation for end-stage ischemic cardiomyopathy. The donor presented with Wolff-Parkinson-White syndrome and the allograft was successfully transplanted. The acces-
sory pathway was interrupted postoperatively by radiofrequency current catheter ablation, and the patient is clinically well and free of preexcitation 24 months later. (Ann Thorac Surg 1995;59:744-6) lthough heart transplantation has evolved into an accepted treatment modality for patients with endstage heart disease, the shortage of donor hearts has become the most critical factor in limiting the n u m b e r of procedures performed. The use of " b o r d e r l i n e " high-risk donors as a potential source of cardiac aUografts has resulted in excellent graft function a n d patient survival [1]. We report the case of a donor who presented with Wolff-Parkinson-White s y n d r o m e with supraventricular tachycardia. The allograft was t r a n s p l a n t e d successfully, a n d the accessory pathway was ablated postoperatively.
A
A 61-year-old m a n presented with end-stage ischemic cardiomyopathy. He had u n d e r g o n e implantation of a cardioverter-defibrillator for an episode of s u d d e n carAccepted for publicationJune 30, 1994. Address reprint requests to Dr Blanche, Heart Transplant Program, Cardiothoracic Surgery, Cedars-SinaiMedicalCenter, 8700BeverlyBlvd, #6215, Los Angeles,CA 90048. 0003-4975/95/$9.50 0003-4975(94)00570-W