Intravenous Leiomyomatosis With Intracardiac Extension

CASE REPORT ZHANG ET AL INTRAVENOUS LEIOMYOMATOSIS WITH INTRACARDIAC EXTENSION

bacterial envelope in patients at high risk for CRMD infection. After this patient refused to undergo the currently recommended treatment, we decided that revision of the PG pocket, followed by local sustained delivery of combination antimicrobial agents, offered a reasonable alternative. Débridement of the pocket should include removal of the capsule, all fibrous tissue around the leads, and all unnecessary foreign material. Because the leads and PG are not removed, they should be free of residual biofilm. If complete sterilization can be achieved by means of irrigation, scrubbing, and antimicrobial delivery, the expense of PG replacement can be avoided, as well as the need for temporary pacing. In conclusion, when infection is limited to the PG pocket, local high-dose combination antimicrobial therapy and a limited surgical approach may be an attractive alternative to total system removal, with its attendant morbidity and mortality.

References 1. Hurst LN, Evans HB, Windle B, Klein GJ. The salvage of infected cardiac pacemaker pockets using a closed irrigation system. Pacing Clin Electrophysiol 1986;9:785–92. 2. Uslan DZ, Tleyjeh IM, Baddour LM, et al. Temporal trends in permanent pacemaker implantation: a population-based study. Am Heart J 2008;155:896 –903. 3. Darouiche RO. Treatment of infections associated with surgical implants. N Engl J Med 2004;350:1422–9. 4. Sohail MR, Uslan DZ, Khan AH, et al. Risk factor analysis of permanent pacemaker infection. Clin Infect Dis 2007;45: 166 –73. 5. del Río A, Anguera I, Miró JM, et al. Surgical treatment of pacemaker and defibrillator lead endocarditis: the impact of electrode lead extraction on outcome. Chest 2003;124:1451–9. 6. Baddour LM, Bettmann MA, Bolger AF, et al. Nonvalvular cardiovascular device-related infections. Circulation 2003;108: 2015–31. 7. Love CJ, Wilkoff BL, Byrd CL, et al. Recommendations for extraction of chronically implanted transvenous pacing and defibrillator leads: indications, facilities, training. North American Society of Pacing and Electrophysiology Lead Extraction Conference Faculty. Pacing Clin Electrophysiol 2000; 23:544 –51.

Intravenous Leiomyomatosis With Intracardiac Extension Chaoji Zhang, MD, Qi Miao, MD, Xingrong Liu, MD, Heng Zhang, MD, Guotao Ma, MD, Guangjun Chen, MD, and Haibo Deng, MB Department of Cardiac Surgery, Peking Union Medical College Hospital, Peking Union Medical College, Beijing, China

Intravenous leiomyomatosis with right intracardiac extension is rare. Surgical treatment of the tumor is still controversial because of the high postoperative risk of Accepted for publication Sept 16, 2009. Address correspondence to Dr Miao, Department of Cardiac Surgery, Peking Union Medical College Hospital, Dongdan, 1 Shuaifuyuan, Beijing, 100730, China; e-mail: [email protected].

© 2010 by The Society of Thoracic Surgeons Published by Elsevier Inc

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morbidity and mortality. We describe a series of 5 patients with these lesions who underwent elective operations with different strategies, including one-staged or two-staged resections and cardiopulmonary bypass with beating heart, cardioplegic arrest, or deep hypothermic circulatory arrest. We believe that this report represents one of the largest series of patients encountered in a single institution. In conclusion, radical resection is always possible and the outcomes are satisfactory with planned surgery. (Ann Thorac Surg 2010;89:1641–3) © 2010 by The Society of Thoracic Surgeons

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ntracardiac leiomyomatosis is a rare and dangerous form of intravenous leiomyomatosis (IVL) [1, 2]. Surgery is the treatment of choice, and complete removal of the IVL has favorable outcomes [2]. We believe that more than 110 cases of this tumor with intracardiac extension have been reported as individual case reports [3]. In this study, we reviewed the records of all patients with this lesion who we have been managed surgically for the prior 9 years at our institution. Between January 2001 and May 2009, 5 women were admitted to our institution with IVLs extending into the right heart. The mean age at diagnosis was 44.6 ⫾ 3.3 years (range, 41– 49 years), All 5 patients reported some cardiac symptoms, such as chest discomfort, palpitation, or syncope. Physical examination revealed severe congestive failure with signs of hepatomegaly, ascites, or lower extremity edema in 3 patients, and a pelvic mass in 2 patients. The findings of imaging showed an intracardiac mass arising from the iliac vein in 3 patients and the ovarian vein in 2 patients (Fig 1). Prior hysterectomy or salpingo-oophorectomy was performed in 4 patients. Of the 5 patients, 2 underwent a two-stage procedure with the extraction of the IVL from the right atrium under cardiopulmonary bypass with cardioplegic arrest and 3 underwent a one-stage procedure. In 2 of 3 patients who underwent the one-stage procedure, the IVLs were resected under deep hypothermic circulatory arrest, and extraction of the IVL in the other patient was made through an atriotomy under emergency cardiopulmonary bypass with a beating heart, because the mass was dropped and had obstructed the right heart after the laparotomy. All 5 patients survived with an uneventful postoperative course. The patients were followed-up for a mean of 37.8 ⫾ 31.3 months (range, 5–76 months) after surgery. Recurrence of a pelvic mass was found in 1 patient at the 3-month follow-up after a two-stage surgery, but no symptoms or intravenous mass were reported since then, even though the patient rejected further treatment. No obstruction occurred in 2 patients with inferior cava venotomy. In all 5 patients, the postoperative histology of the excised tumors confirmed the presence of IVLs and immunohistochemical studies showed that the tumor cells were positive for smooth muscle markers, including desmin and smooth muscle actin. 0003-4975/10/$36.00 doi:10.1016/j.athoracsur.2009.09.044

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CASE REPORT ZHANG ET AL INTRAVENOUS LEIOMYOMATOSIS WITH INTRACARDIAC EXTENSION

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Fig 1. Computed tomographic scan revealed an intraluminal mass (arrows) arising from the tumor extension of a left pelvic mass, which progressed through the left ovarian vein, and from the left renal vein to the inferior vena cava, reaching the right heart cavity in a patient undergoing one-stage surgical treatment.

Comment Open heart surgery was first reported by Mandelbaum and colleagues [4] in 1974. The surgical treatment choices for IVL with intracardiac extension are one-stage or two-stage procedures with cardiopulmonary bypass under the beating heart, cardioplegic arrest, or hypothermic circulatory arrest conditions. However, there are different opinions on the use of one-stage or two-stage procedures for patients with IVLs. The slow growth of the tumor allows for a safe interval between the first-stage cardiotomy and the second-stage laparotomy [2, 3]. Staged surgery can reduce the first-stage thoracic operative time, and consequently can decrease the risk of bleeding associated with systemic heparinization required for cardiopulmonary bypass. However, Filsoufi and colleagues [5] reported that avulsion injury of the renal vein leading to a large hematoma during extraction of a right atrial tumor without abdominal exposure. As a result, traction injury of the abdominal vein necessitated an emergency laparotomy, which increased the risk for operative mortality. In recent years, one-stage approaches have been

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introduced because of the increased knowledge of this disease. A one-stage operation avoids the risk for tumor embolism, tumor progression, or hemodynamic complications in the interval between the surgical stages. The one-stage operation also avoids the need for repeated anesthesia and operation. In addition, one-stage approaches can simultaneously expose the thorax and the abdominal cavity, and the approach is suitable for complete removal of the IVL, reconstruction of the vascular structures, and control of bleeding. On the basis of our experience with these cases, we recommend a singlestage approach with abdominal venous exposure at the time of removing the tumor through an atriotomy. Because single-stage surgery requires a longer operative time, which is not appropriate for critical conditions, such as severe heart failure and syncope, the two-stage procedure should be performed as early as possible in patients with urgent conditions to reduce the risk of sudden death and concomitant venous thromboembolism [6]. Different routes of extension might have a pronounced effect on the procedures available for that patient [7]. In our opinion, although the extraction of IVL can reduce bleeding and the operative time, extraction through the right atrium is not indicated in IVLs arising from the ovarian vein because of the existence of extensive attachment to the vessels at the junction of the ovarian vein into the inferior vena cava (IVC) or the renal vein [5]. Clinical findings that warrant extraction of the IVL include tumors with a small diameter, nonsignificant obstruction of blood flow in the IVC, and no markers of right heart failure, which suggests limited attachment to the cardiac and vascular structures on imaging studies. Because extensive attachment was determined using computed tomography and ultrasonography in 2 patients, circulatory arrest was necessary to avoid avulsion injury of the caval veins. However, deep hypothermic circulatory arrest is associated with increased risk for coagulopathy and neurological sequelae, which must be balanced against the advantages of working in a bloodless surgical field. In one of our patients, the IVL was extracted through an atriotomy under emergency cardiopulmonary bypass because the dropped mass obstructed the right heart. This experience suggested that the division of the intravenous tumor in the abdominal cavity should be done before establishing cardiopulmonary bypass to minimize the risk for acute pulmonary embolism and sudden death. In addition, retrograde extraction or removal using a venotomy through an isolated abdominal approach without cardiopulmonary bypass is not recommended, because the greatest diameter of the intracardiac tumor is usually 2 to 6 times larger than the diameter of the IVC inlet, as in our patients; otherwise this can cause fatal retroperitoneal hemorrhage or acute pulmonary embolism. The surgical approaches to IVL with cardiac extension must take account into the structures surrounding the IVL. A severely compromised IVC or renal vein may require repair or prosthetic replacement. A ruptured

ureter can be repaired or replaced using a ureter stent. We routinely assess unilateral renal function by isotopebased renal imaging and implant a double-J stent into the ureter before laparotomy in patients with an IVL arising from the ovarian vein. In conclusion, IVL is a rare nonmalignant smooth muscle tumor. Accurate preoperative assessment of the tumor size and different routes of extension by a multidisciplinary medical team is essential to enable successful complete tumor excision. The potential clinical applicability of the different strategies, as previously described, need further evaluation in large, randomized studies.

References 1. Gaudino M, Spatuzza P, Snider F, Luciani N, Cina G, Possati G. Surgical management of a uterine leiomyoma extending through the inferior vena cava into the right heart. Heart Vessels 2002;17:80 –2. 2. Topcuoglu MS, Yaliniz H, Poyrazoglu H, et al. Intravenous leiomyomatosis extending into the right ventricle after subtotal hysterectomy. Ann Thorac Surg 2004;78:330 –2. 3. Liu B, Changwei Liu CG, Guan H, et al. Intravenous leiomyomatosis with inferior vena cava and heart extension. J Vasc Surg 2009;50:897–902. 4. Mandelbaum I, Pauletto FJ, Nasser WK. Resection of a leiomyoma of the inferior vena cava that produced tricuspid valvular obstruction. J Thorac Cardiovasc Surg 1974;67:561–7. 5. Filsoufi F, Farivar RS, Anderson C, Santerre D, Adams DH. Renal vein injury complicating removal of intravenous leiomyoma. J Thorac Cardiovasc Surg 2002;123:820 –2. 6. Wu CK, Luo JL, Yang CY, et al. Intravenous leiomyomatosis with intracardiac extension. Intern Med 2009;48:997–1001. 7. Lam PM, Lo KW, Yu MY, et al. Intravenous leiomyomatosis: two cases with different routes of tumor extension. J Vasc Surg 2004;39:465–9.

Fatal Outcome of Recombinant Factor VIIa in Heart Transplantation With Extracorporeal Membrane Oxygenation Thomas Syburra, MD, Mario Lachat, MD, Michele Genoni, MD, and Markus J. Wilhelm, MD Clinic for Cardiovascular Surgery, University Hospital, and Clinic for Cardiac Surgery, City Hospital Triemli, Zurich, Switzerland

Recombinant activated factor VII (rFVIIa) has been approved for treatment of bleeding episodes in patients with hemophilia and in nonhemophilia patients with acquired antibodies against factor VIII or IX. The application of rFVIIa in nonapproved settings, as in cardiac surgery, has not been established. It raises concerns regarding its safety. We used rFVIIa in a patient with Accepted for publication Sept 16, 2009. Address correspondence to Dr Wilhelm, Clinic for Cardiovascular Surgery, University Hospital Zurich, Rämistrasse 100, Zurich, CH 8091, Switzerland; e-mail: [email protected].

© 2010 by The Society of Thoracic Surgeons Published by Elsevier Inc

CASE REPORT SYBURRA ET AL RECOMBINANT FACTOR VIIA IN HEART TRANSPLANTATION

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excessive nonsurgical bleeding on extracorporeal membrane oxygenation, which was established for early graft failure after heart transplantation and after 3 months of biventricular assist device support. After rFVIIa administration, cardiac thrombosis developed and caused the patient’s death. (Ann Thorac Surg 2010;89:1643–5) © 2010 by The Society of Thoracic Surgeons

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lthough only approved for application in hemophilia patients, recombinant activated factor VII (rFVIIa) has also been used successfully in nonapproved settings, such as nonsurgical bleeding situations with dilution or consumptive coagulopathies in cardiac surgery [1, 2]. Few reports exist regarding the usage of rFVIIa in patients on extracorporeal membrane oxygenation (ECMO) after heart surgery. They are case reports and small series of pediatric or adolescent patients. We want to share our experience of a fatal rFVIIa application in an adult on ECMO, implanted for early graft failure, following heart transplantation after 3 months of biventricular assist device support. A 58-year-old Caucasian man (1.86 m for 85 kg) with a history of mechanical mitral valve replacement in 1995 for severe mitral insufficiency was listed for heart transplantation in July 2007 because of rapidly progressive heart failure. After resuscitation in September 2007, emergency ECMO implantation was performed by cannulation of the femoral vessels, and the patient was put on the urgent waiting list. Because no suitable donor heart was found, the patient was switched 9 days later to a biventricular assist device (Berlin Heart Excor, Berlin, Germany). A mycotic infection at the former femoral ECMO cannulation site caused serious bleeding; an extra-anatomic iliaco femoral bypass was implanted 3 weeks later, and the groin was treated with vacuum therapy. After increasing recovery on the regular ward, a suitable donor heart was found in January 2008, and bi-ventricular assist device explantation and heart transplantation was performed. Intraoperatively, a mycotic infection caused by Candida albicans was detected underneath the sternum and subepicardially around the left pulmonary veins and pulmonary artery. After intense irrigation of the pericardium, the donor heart was implanted. Recipient aortic cross-clamp time was 73 minutes, and donor heart ischemia time was 189 minutes. Despite transfusion of 39 units of packed red blood cells, 54 units of fresh frozen plasma, 6 units of platelets (6 g fibrinogen), and 1,800 units of activated prothrombin complex concentrates, massive diffuse, nonsurgical, bleeding persisted, which lead to severe hemodynamic instability. Due to rapid deterioration of right heart function, the patient was taken on ECMO, and was transferred to the intensive care unit with an open sternum. Activated clotting time at that moment was 155 seconds, platelet count (87 ⫻ 103/␮L),. D-dimers (0.68 ␮g/mL), and fibrinogen (2.3 g/L). The bleeding persisted, impairing the ECMO function. As salvage therapy (after 0003-4975/10/$36.00 doi:10.1016/j.athoracsur.2009.09.039

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