Ann Thorac Surg 2009;87:1589-1592. doi:10.1016/j.athoracsur.2008.09.077
© 2009 The Society of Thoracic Surgeons
Case Reports
Diagnostic and Surgical Issues in Emergency Presentation of a Pelvic Leiomyoma in the Right Heart
Nicola Luciani, MDa,
Amedeo Anselmi, MDa,*,
Franco Glieca, MDa,
Lorenzo Martinelli, MDb,
Gianfederico Possati, MDa
a Division of Cardiac Surgery, Catholic University, Rome, Italy
b Division of Cardiac Anesthesia, Catholic University, Rome, Italy
Accepted for publication September 30, 2008.
* Address correspondence to Dr Anselmi, Division of Cardiac Surgery, Catholic University, Largo A. Gemelli 8, Rome, 00168, Italy (Email: amedeo.anselmi{at}alice.it).
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Abstract
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A computed tomography scan in a 43-year-old woman with a nonsignificant previous medical history demonstrated an inferior caval mass prolapsing through the right atrium and the tricuspid valve. The mass was misdiagnosed as a thrombus-in-transit, and heparin was started. The clinical picture suddenly evolved into cardiogenic shock, and the patient underwent an emergency resection of the intracardiac portion of the mass. Macroscopic and microscopic features were consistent with leiomyoma. In the presence of an inferior caval mass, historical elements and computed tomography imaging that do not corroborate the hypothesis of caval thrombosis should raise the suspicion of intracaval tumor originating from an abdominal or pelvic organ. The preferred treatment strategy is an elective combined thoracic and abdominal resection.
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Introduction
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The surgical strategy to treat gynecologic neoplasms extending into the inferior vena cava up to the right atrium is well described in the elective setting [1–3]. Single-stage cardiac and abdominal approach by a sternolaparotomy is usually preferred to obtain radical resection. However, these rare entities may sporadically present as emergencies and cause sudden hemodynamic instability and shock. The optimal strategy in such cases is not clarified. We discuss a patient who received emergency resection of the sole intracardiac portion of a misdiagnosed uterine leiomyoma extending in the inferior vena cava.
A 43-year-old woman was admitted to another hospital after complaining of sudden-onset dyspnea, orthopnea, and dizziness during the previous 10 hours. Her medical history was negative for known neoplasms, pelvic operations, hip replacement, or previous deep venous thrombosis/pulmonary embolism. She had bilateral lower limb edema.
A computed tomography (CT) scan of the thorax and the abdomen revealed an intravenous mass within the right common iliac vein and the inferior vena cava. The mass was extended in the right atrium and prolapsed through the tricuspid valve. The inferior vena cava was distended, and the femoral veins were free from intraluminal thrombus. The mass appeared not to have close relationship with the vessel wall; rather, it had a floating appearance within the inferior vena cava (Fig 1). The patient received a diagnosis of thrombosis, and treatment with intravenous heparin was initiated.
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Fig 1. Preoperative computed tomography scan of the thorax and the abdomen shows (A) a mass within the right atrium and prolapsing in the right ventricle through the tricuspid orifice (white arrowhead). (B, C, D) The intracaval mass has an irregular, cord-shaped, floating appearance (white arrows).
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Within a few hours the patient suddenly presented with severe heart failure. Endotracheal intubation was required to maintain blood oxygen saturation. The patient was emergently transferred to our cardiac surgery department in a condition of cardiogenic shock refractory to vasoconstrictors. Her blood pressure was 60/40 and the pulse rate was 120 beats/min. She was receiving intravenous noradrenalin. Transthoracic echocardiography revealed a mass within the right atrium passing through the tricuspid orifice (Fig 2).
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Fig 2. Preoperative transthoracic echocardiography section shows an elongated mass within the right cardiac chambers (white arrowheads) and passing through the tricuspid orifice (white star).
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The patient was immediately transferred to the operating room. The superior vena cava was cannulated percutaneously through the right internal jugular vein, and the right femoral artery and vein were cannulated according to the Seldinger technique. After median sternotomy and before establishment of extracorporeal circulation, the right cardiac chambers appeared collapsed because of inadequate venous return from the inferior vena cava. Hypothermic cardiopulmonary bypass (CPB) was instituted, and a third venous cannula was placed in the right atrium to achieve adequate drainage.
On circulatory arrest at 28°C rectal temperature, a right atriotomy was performed; a mass was inspected coming out from the inferior vena cava and prolapsing through the tricuspid orifice. At macroscopic examination, the mass appeared nonfriable and of fatty, irregular, and rubbery consistency; no adhesions were noted with the internal wall of the vena cava. The mass could be easily removed from the right chambers by simple traction; mild adhesions with the septal tricuspid leaflet were separated uneventfully. A portion of the mass was adherent to 2 chordae tendineae pertaining to the septal leaflet; these had to be excised (Fig 3). The proximal end of the mass was gently pulled upwards by about 10 cm, and the subhepatic veins appeared to be not involved. As resistance to traction was noticed, the mass was transversely sectioned at the level of the subhepatic veins because sudden retroperitoneal hemorrhage is possible in the instance of attempt to avulse the tumor from the cardiac end [4]. The tricuspid valve was carefully inspected for tears. A hemodynamic test disclosed no significant regurgitation; hence, no valve repair procedure was performed. After full rewarming and weaning from CPB, intraoperative transesophageal echocardiography showed mild tricuspid insufficiency.
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Fig 3. Gross appearance of the excised specimen shows the portion of the mass within the right atrium (white arrow), the portion of the mass that had a relationship with the septal leaflet of the tricuspid valve and with the chordae tendineae (black arrowheads), and the level of proximal transection (white arrowhead).
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Circulatory arrest time was 14 minutes. The patient was transferred to the intensive care unit in good hemodynamic condition. Her postoperative course was complicated by acute renal insufficiency requiring temporary hemodialysis.
Histologic examination confirmed the leiomyomatosis nature of the mass excised, and a postoperative CT scan indicated the mass was arising from the uterine wall. After general recovery, the patient ultimately underwent excision of the residual tumor mass. A hysterectomy was performed and the caudal end of the mass was isolated from the distal vena cava. No adhesions were present between the mass and the vein wall. With the patient in the anti-Trendelenburg position, the mass was pulled down without encountering resistance and the infrarenal vena cava was cross-clamped as soon as possible. The vein wall was sutured, and the patient recovered uneventfully.
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Comment
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Intravenous leiomyomatosis is defined as an extension into the venous channel of a histologically benign smooth-muscle tumor arising from the uterus or other organs [5]. This is a rare condition occurring more commonly in middle-aged women, and intracardiac localization is seen in approximately 10%. Surgical intervention is generally indicated on the basis of right-sided heart failure or risk of embolism, or both.
Spreading within the right-sided heart chambers can be easily misdiagnosed as either a primary cardiac tumor or a venous thrombus-in-transit. Misdiagnosis may lead to inappropriate treatment until severe heart failure occurs due to obstruction to flow across the tricuspid valve. At CT scan, the absence of adhesion between the intravenous mass and the caval wall and the cord-shaped appearance as far as the absence of thrombus within the femoral veins may suggest the neoplastic nature of the mass. Elements such as the absence in the clinical history of recent hip or knee operations, lower limb/hip traumas or bone fractures, previous pulmonary embolism, or deep vein thrombosis may also help to differentiate between vena caval thrombosis and metastatic tumor. A history of previous operations for pelvic myomas, which have been associated with intraoperative seeding of neoplastic cells to the pelvic vessels [6], is an additional useful element.
In the most recent series and in our experience, the combined single-staged approach under sternal laparotomy is preferred for the management of caval tumors originating from the uterus, the ovaries, or the kidneys because it allows complete removal of the mass from both the large veins and the abdomen or the pelvis (combined hysterectomy, oophorectomy, and nephrectomy) [1–3]. In the patient we have described, the hemodynamic instability prompted treatment solely of the intracardiac portion of the tumor. Additional determinants of such strategy were the incomplete data on the proximal origin of the tumor and the compromised general condition of the patient, who had scarce possibilities to tolerate a prolonged thoracoabdominal procedure.
Elective management in our previous experience of 5 patients with intracardiac uterine leiomyomatosis or renal malignancy extending to the right atrium through the inferior vena cava included a single-staged operation involving two surgical teams and complete resection of the tumor. The postoperative course was uneventful in all patients. Surgical strategy incorporated CPB with hypothermic circulatory arrest, as in the present patient.
Our experience has shown this approach guarantees several advantages, including (1) the possibility to perform extensive cavotomy if required, (2) the ability to evaluate the subhepatic veins for possible involvement, and (3) a bloodless operative field. These advantages counterbalance, in most cases, the known pitfalls of circulatory arrest including coagulopathy and neurologic risk. Ongoing CPB during the entire procedure should be probably reserved for rare instances of limited intravenous caval thrombosis or tumor without extension into the lower portions of the vena cava. Endoscopic resection of intracardiac leiomyomatosis has also been reported, and in experienced hands it is an effective and reliable method to remove the intracardiac portion of the mass and repair or replace the tricuspid valve whenever needed [7].
The encroachment of the tumor mass within the right-sided chambers and the tricuspid annulus has the potential to injure the valve leaflets; and in this regard, a wide spectrum of lesions are possible and their management may vary from no surgical procedure on the tricuspid valve to valvuloplasty and valve replacement [4]. Intraoperative transesophageal echocardiography should be always performed in these patients before and after CPB.
The emergency presentation with hemodynamic instability in the present patient obligated us to a salvage procedure and to only partial removal of the overall tumor mass. A second operation was then needed to obtain radical excision. Correct diagnosis at the first presentation is therefore crucial to plan the treatment strategy under elective conditions. A high degree of suspicion should be probably kept when, in the presence of an intravenous inferior caval mass, historical elements and CT imaging features do not corroborate the hypothesis of caval thrombosis.
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References
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- Thukkani N, Ravichandran PS, Das A, Slater MS. Leiomyomatosis metastatic to the tricuspid valve complicated by pelvic hemorrhage Ann Thorac Surg 2005;79:707-709.[Abstract/Free Full Text]
- Clement PB. Intravenous leiomyomatosis of the uterus Pathol Annu 1988;23:153-183.[Medline]
- Katsumata T, Shinfeld A, Houel R, Westaby S. Pelvic leiomyoma in the right atrium Ann Thorac Surg 1998;66:2095-2096.[Abstract/Free Full Text]
- Tsuji Y, Yamashita C, Wakiyama H, et al. Surgical treatment for transvenous tumor extension into the heart: four cases J Vasc Surg 1998;27:740-744.[Medline]
- Wakiyama H, Sugimoto T, Ataka K, et al. Intravenous leiomyomatosis extending into the right ventricular cavity: one stage radical operation using cardiopulmonary bypass Angiology 2000;51:505-509.[Medline]
- Okamoto H, Itoh T, Morita S, Matsuura A, Yasuura K. Intravenous leiomyomatosis extending into the right ventricle: one-stage radical excision during hypothermic circulatory arrest Thorac Cardiovasc Surg 1994;42:361-363.[Medline]
- Jeanmart H, Lecompte P, Casselman F, Coddens J, Van Vaerenberg G, Vanermen H. Endoscopic tumor resection of the inferior vena cava J Thorac Cardiovasc Surg 2006;132:687-688.[Free Full Text]
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Abstract
Introduction
