A 52-year-old woman presented to our institution with progressive dyspnea and palpitations. Her medical history was limited to a hysterectomy for uterine fibroids 3 years before admission. A computed tomographic scan revealed a large mass arising from the left pelvis extending up the inferior vena cava (IVC) to the right atrium (RA). The patient was scheduled for surgical extraction of this mass. She gave consent for publication of this report.
After induction of anesthesia, a transesophageal echocardiogram (TEE) with emphasis on the entire potential course of the tumor was performed. Imaging of the intrahepatic IVC revealed a large, smooth walled, polycystic mass. In the midesophageal (ME) bicaval view, the freely mobile intracardiac portion of the mass nearly filled the RA. In this view, color flow Doppler at a low Nyquist limit suggested no patent foramen ovale, lessening concern for systemic embolism. In the 4-chamber view, the tumor protruded into the right ventricle (RV) during diastole (Video 1, see Supplemental Digital Content 1, http://links.lww.com/AA/A179; see Appendix for video legends). The ME RV inflow-outflow view showed the bulky mass traversing the tricuspid annulus (Video 2, see Supplemental Digital Content 2, http://links.lww.com/AA/A180; see Appendix for video legends). The view was obtained as described by the American Society of Echocardiography/Society of Cardiovascular Anesthesiologists guidelines, by turning the probe right from the ME 4-chamber view to center the tricuspid valve (TV) and then rotating the multiplane angle to 60 to 90 degrees while keeping the TV visible. During this rotation, the RV outflow tract opened and the pulmonic valve and pulmonary artery (PA) came into view, somehow distorted by the bulk and mobility of the mass. In this view, the tricuspid subvalvular apparatus appeared hyperechoic but was obscured by the mass. Tumor involvement of the TV could not be excluded. Finally, in the ME ascending aortic short-axis view, a linear echodensity was noted in the right PA, but was believed to be more consistent with near field artifact. The left ventricular ejection fraction was estimated at 65%. There were no wall motion abnormalities noted.
The initial surgical approach for excision of the tumor involved a midline sternotomy and bilateral subcostal incisions. After cannulation of the aorta and RA appendage and the institution of cardiopulmonary bypass (CPB), deep hypothermic circulatory arrest was initiated and the intraabdominal vena cava opened. A long rubbery rope of tumor was easily extracted and removed from its point of attachment to the left iliac vein. The tumor appeared grossly intact and was sent to pathology (Fig. 1).
After closure of the IVC, during the rewarming phase before weaning from CPB, ongoing TEE of the RV inflow-outflow view revealed a thin sliver of residual tumor entangled in the chordae of the septal leaflet of the TV and fluttering in the RV outflow tract (Video 3, see Supplemental Digital Content 3, http://links.lww.com/AA/A181; see Appendix for video legends). The surgeons were notified of these findings. They elected to recool the patient and perform an atriotomy to extract the residual sliver of tumor from the RV (Fig. 1). No further evidence of tumor was noted during the subsequent termination of CPB. The patient had an uneventful postoperative course. Pathologic examination of the specimens confirmed the diagnosis of intravascular leiomyomatosis.
This case adds to the body of literature advocating complete TEE evaluation of the entire right-sided circulation throughout surgical extraction of IVC-occupying tumors. Complete intraoperative TEE imaging depends on adequate and timely views. The entire potential course of the tumor should be imaged, from the IVC through the atrium (ME bicaval view with examination of intrahepatic IVC and evaluation for interatrial shunt), RV (4-chamber and RV inflow-outflow view), and PAs (ME ascending aortic short-axis view). These images should be obtained throughout surgery: before, during, and after resection. Initial examination reveals the extent of the mass, its points of attachment, and the presence of an intracardiac shunt. Ongoing examination during surgical manipulation of the tumor can detect embolism.1,2 Postexcisional examination is critical for surveillance for residual tumor.3
In our case, the RV inflow-outflow images obtained pre-excision raised the suspicion of tumor involvement of the TV and RV. By obtaining the RV inflow-outflow view again after transcaval tumor excision but while the patient was still on CPB, we were able to confirm the presence of residual tumor and guide the surgeons to complete resection of the tumor. To obtain this view while the patient was still on CPB, we used the previously described approach, but this time found the optimal image at a greater degree of rotation. We also requested that the surgeon and perfusionist allow some ventricular filling. The blood volume filling the heart helped to elucidate the intracardiac structures. Communication between the anesthesia and surgical teams allowed optimization of the TEE examination and the diagnosis of residual tumor.
Our case involves IV leiomyomatosis, a tumor that shares occupancy of the IVC with the more familiar renal cell carcinoma, but has unique characteristics important to the anesthesiologist and echocardiographer. It is a rare type of tumor that, although benign, can be fatal because of mechanical interference with cardiac function. These tumors occur primarily in middle-aged women with prior hysterectomy. They frequently arise from uterine, ovarian, or hypogastric veins, and extend via the IVC to the RA, remaining in the intravascular space. These tumors can have multiple endovascular points of attachment. Rubbery and smooth, they have little thrombogenic potential, in contrast with renal cell carcinomas. They can, however, give rise to similar satellite lesions. Treatment depends on complete resection of the tumor. The surgical approach is variable and can involve staged abdominal and thoracic procedures or 1-step procedures with or without CPB and circulatory arrest.4 Intraoperative TEE is a powerful tool to guide the surgical approach by defining the extent of the tumor and its points of attachment.5
1. Chen H, Ng V, Kane CJ, Russell IA. The role of transesophageal echocardiography in rapid diagnosis and treatment of migratory tumor embolus. Anesth Analg 2004;99:357–9
2. Komanapalli CB, Tripathy U, Sokoloff M, Daneshmand S, Das A, Slater M. Intraoperative renal cell carcinoma tumor embolization to the right atrium: incidental diagnosis by transesophageal echocardiography. Anesth Analg 2006;102:378–9
3. Martinelli SM, Mitchell JD, McCann RL, Podgoreanu MV, Mathew JP, Swaminathan M. Intraoperative transesophageal echocardiographic diagnosis of residual tumor fragment after surgical removal of renal cell carcinoma. Anesth Analg 2008;106:1633–5
4. Harris LM, Karakousis CP. Intravenous leiomyomatosis with cardiac extension: tumor thrombectomy through an abdominal approach. J Vasc Surg 2000;31:1046–51
5. Subramaniam B, Pawlowski J, Gross BA, Kim YB, LoGerfo FW. TEE-guided one-stage excision of intravenous leiomyomatosis with cardiac extension through an abdominal approach. J Cardiothorac Vasc Anesth 2006;20:94–5
APPENDIX: ONLINE ECHOCARDIOGRAPHIC VIDEO IMAGES LEGENDS
Video 1: The 4-chamber view demonstrates a large polycystic mass in the right atrium (RA), protruding through the tricuspid valve (TV) into the right ventricle (RV) during diastole. LA = left atrium; MV = mitral valve; LV = left ventricle.
Video 2: In this prebypass right ventricular inflow-outflow view, the aortic valve (Ao) is central with the right heart inflow on the right of the screen and the outflow tract on the left. The mass is seen protruding through the tricuspid valve (TV). A hyperechoic linear density arising from the subvalvular apparatus and traversing the right ventricle (RV) toward the pulmonic valve (PV) is suggested. Tumor involvement of the RV and pulmonary artery cannot be excluded.
Video 3: This right ventricular inflow-outflow image was obtained during intravascular volume loading in preparation for discontinuing cardiopulmonary bypass. Although the view is somewhat distorted, the aortic valve (Ao) is still central. Clearly visible is a tumor fragment tangled in the tricuspid subvalvular apparatus and fluttering in the right ventricular outflow tract (RVOT). RA = right atrium; TV = tricuspid valve; RV = right ventricle.
Clinician's Key Teaching Points By Kent H. Rehfeldt, MD, Nikolaos J. Skubas, MD, and Martin J. London, MD
- In a patient with an inferior vena cava (IVC) mass, a systematic intraoperative transesophageal echocardiographic (TEE) examination is important for patient management (mass effect or potential for paradoxical embolization via interatrial communications) and surgical planning (need for cardiopulmonary bypass). This should include imaging of the intrahepatic IVC, the right cardiac chambers (midesophageal [ME] 4-chamber, right ventricular [RV] inflow/outflow and bicaval views), the pulmonary artery (ME ascending aorta short-axis view), tricuspid valve, and interatrial septum.
- The IVC can be imaged by advancing the probe slightly from the ME 4-chamber view while turning it rightward; the IVC appears in short axis at its confluence with the right atrium. Increasing the multiplane angle gradually to between 30 and 90 degrees facilitates long-axis imaging of the IVC, while progressive advancement of the probe displays the intrahepatic portion of the IVC and its confluence with hepatic veins.
- In this case, TEE displayed extension of the tumor from the IVC into the RV through the tricuspid annulus in diastole. A repeat TEE examination after initial resection demonstrated the presence of residual tumor within the RV and prompted further surgical exploration.
- Leiomyomatosis is a large IVC tumor with cavitary lesions, which may give rise to satellite lesions; complete resection is critical. A methodical intraoperative TEE examination of all intra- and extracardiac structures along the potential route of tumor extension, before and after surgical manipulation, helps document tumor extent and complete removal.