Cardiac tumors are rarely observed. The incidence of primary cardiac tumors in autopsy series ranges from 0.0017% to 0.19%.1,2 Surgical resection is the main therapy for the majority of the cardiac tumors. Surgical treatment of these tumors carries an operative mortality rate of 3% or less.4 In this article, we present our experience with a female patient, who had a right sided atrial tumor mimicking a myxoma. Port access surgery was performed through a small right sided “key-hole” working port in the fourth intercostal space. Extracorporeal circulation was conducted by femoro-femoral bypass and a kinetic assisted venous drainage system. Although, the safety and efficacy of port access approach have been well documented for resection of left atrial tumors in some series,14,17 use of this technique for right atrial tumor resection can be detrimental.
From the *Department of Thoracic and Cardiovascular Surgery, OLV Clinic, Aalst, Belgium; †Department of Thoracic and Cardiovascular Surgery, Siyami Ersek Cardiovascular Surgery Center, Istanbul, Turkey; and ‡Department of Anesthesia and Intensive Care Medicine, OLV Clinic, Aalst, Belgium.
Accepted for publication: December 6, 2008.
Address correspondence and reprints requests to Ihsan Bakir, MD, Department of Thoracic and Cardiovascular Surgery, Siyami Ersek Cardiovascular Surgery Center, Tibbiye Caddesi Haydarpasa, 34000 Istanbul, Turkey; E-mail: firstname.lastname@example.org.
Primary cardiac tumors are uncommon. Seventy-five percent of these tumors are benign; 50% are myxomas; and most of the rest are lipomas, papillary fibroelastomas, and rhabdomyomas.1,2 Approximately 75% occur in the left atrium, 20% may be calcified, and, interestingly enough, most of these are right atrial.3–5 Less common villous or papillary myxomas are gelatinous and fragile and prone to fragmentation and embolization.6 Twenty-five percent of primary cardiac tumors are malignant, and one third of these tumors are angiosarcomas.3,7 Angiosarcomas have a predilection for the right heart. Eighty percent arise in the right atrium (RA). These tumors tend to be bulky and aggressively invade adjacent structures.8
A transthoracic echocardiogram usually provides all the necessary information for surgical resection, but transesophageal echocardiography (TEE) provides the best information concerning tumor location, size, mobility, and attachment. Transesophageal echocardiograms detect tumors as small as 1 to 3 mm in diameter.3 Although many cardiac tumors have been identified using computed tomography (CT), this technical investigation is most useful in malignant tumors of the heart because of its ability to show myocardial invasion and tumor involvement of adjacent structures. Similarly, magnetic resonance imaging (MRI) has been used in the diagnosis of cardiac neoplasms and may yield a clear picture of tumor size, shape, and surface characteristics.9
Neither CT nor MRI is needed for atrial tumors if an adequate echocardiogram is available because the information from these studies is not likely to alter surgical approach. The exception is the occasional right atrial tumors that extend into one or both caval or tricuspid orifice. CT or MRI should be reserved for the situation in which the diagnosis or characterization of the tumor is unclear after complete echocardiographic evaluation.3
Port access cardiac surgery is an option for resection of cardiac tumors as the manipulation of the heart is minimal.10 To optimize cardiopulmonary bypass (CPB), the use of a kinetic assisted venous drainage (KAVD) system and proper positioning of arterial and venous cannulas and catheters guided by TEE are essential for port access cardiac surgery.11
The following case report describes a unique event caused by the KAVD. A cardiac mass was aspirated in the venous cannula temporarily obstructing the venous return and finally lodging in the centrifugal pump. This report also illustrates that the advantages of a certain surgical technique cannot be a priori inferred to other new indications.
A 37-year-old white female was admitted to a referring hospital with dyspnea and thoracic discomfort. Pulmonary embolism (PE) was suspected. MRI revealed the presence of a mass in the RA. It was located posteriorly and basally in the RA and extended to the posterior wall of the inferior caval vein. The mass was pedunculated and mobile and was suspected to be a myxoma. TEE had also revealed a right atrial mass originating from the eustachian valve (Fig. 1). The mobile structure with sharp defined borders had a diameter of 2.2 to 2.6 cm.
Cardiac catheterization revealed normal coronary arteries and an ejection fraction of 75%. The patient was referred to our center for resection of the tumor through minimally invasive approach (port access surgery).
In the operating theater, a 17-Fr DLP cannula (Medtronic DLP, Grand Rapids, MI) was inserted percutaneously using a seldinger technique by the anesthesiologist through the internal jugular vein into the superior vena cava under TEE guidance. Before placement of the 17-Fr DLP cannula, we administered 2500 IU intravenous heparin to the patient. The right common femoral vein and artery are exposed by using a 3 to 4 cm oblique groin dissection. A double purse-string suture [4-0 polytetrafluoroethylene (Gore-Tex; registered trademark of W. L. Gore & Associates, Flagstaff, AZ)] is placed at the anterior side of the common femoral vein, and a double pledget “U” stitch is placed at the anterior side of the common femoral artery. The femoral artery and the vein were cannulated with an open seldinger technique by the surgeon also under TEE guidance. After full heparinization (300 units/kg), the femoral vein was cannulated with a 28-Fr QuickDraw venous cannula (Cardiovations, Johnson & Johnson Corporate, Somerville, NJ) first. The tip of the cannula was positioned at the junction of the atrium and the inferior caval vein. Then femoral artery was cannulated with a 21-Fr arterial endo return (Cardiovations Johnson& Johnson Corporate) cannula.
Surgery was performed through a 5 cm anterolateral thoracotomy incision in the right inframammary groove. Video-assistance was used with a 5-mm endoscope (Olympus Visera OTV-S7 Digital processor, Hamburg, Germany). A femoro-femoral extracorporeal circulation (augmented with internal jugular vein cannulation) was established and aortic cross-clamping and delivery of cold crystalloid cardioplegia were achieved with an Endo-Clamp aortic catheter (Cardiovations, Ethicon, Johnson & Johnson, Heartport, Inc.,).
CPB circuit was assembled on a Cobe Stöckert pump (Stockert Instrumente GmBH, Munich, Germany) with two centrifugal pumps. One centrifugal pump (Biomedicus BPX-80; Medtronic, Eden Prairie, MN) was used as an arterial pump and the second for augmented venous return. The rest of the extracorporeal kit consists of a closed venous reservoir (BMR 1900, Baxter Health Care Corp., Irvine, CA), a membrane oxygenator with integrated heat exchanger (Forte, Medtronic, cardiopulmonary, Anaheim, CA), filtered cardiotomy reservoir (Medtronic), arterial line filter (Medtronic), and the usual in-line monitors and safety devices. Partial CPB was started with standard gravity drainage. Once on bypass, the KAVD was started to augment the venous return.
After a short period of time on CPB, the venous return deteriorated. The femoral venous cannula was advanced and withdrawn in an attempt to enhance the venous return; however, the venous return continued to deteriorate. The negative pressure of the KAVD system was −20 to −30 mm Hg and increased further to −45 mm Hg and the venous return was normalized again. The arterial and venous parameters stayed within normal range (venous saturation was never below 70%) and there was no acidosis at this moment. The inferior vena cava was dissected from the diaphragmatic junction and both vena cava were encircled with tapes to allow isolation of the RA. After endo-clamping of the aorta and snaring of both caval veins, cardioplegia was delivered and then the RA was opened. The surgical exploration revealed that there was only pedicle of the mass found in the RA cavity. The pedicle of the mass was attached to the broad base of tissue, which was sitting on the eustachian valve and neighboring interatrial septum (IAS). The right ventricle and the pulmonary artery were investigated thoroughly with 0- and 30-degree endoscopic camera for possible tumor embolization. The rectal temperature was decreased to 23°C and total circulatory arrest was established. Retrograde cerebral perfusion is conducted through the internal jugular venous cannula. CPB time was 130 minutes; aortic cross clamp time was 67 minutes; and total circulatory arrest was 10 minutes. We placed ice on the head of the patient during the circulatory arrest phase of the procedure.
The inferior vena cava was checked and a part of the tumoral mass in close contact with the eustachian valve was removed. The base of the tumoral mass on the IAS was resected extensively as well. The defect on the IAS was repaired with a bovine pericardial patch. The patient was rewarmed before weaning from CPB. After weaning from CPB, the venous components of the CPB circuit were examined to find out the reason for the problem. The fragmented tumoral mass was discovered in the venous centrifugal pump (Fig. 2). Weaning from CPB was uneventful. Histopathologic examination of the tumor revealed a cardiac calcified amorphous tumor (CAT) (Fig. 3). There were not any neurologic sequelae immediately or at follow up. The patient was discharged in the sixth postoperative day with normal TEE result and no residual cardiac tumor. At follow-up after 2 and 4 months, the patient had no physical limitations with normal TEE examination.
Nonneoplastic cardiac masses are important because of their capacity to mimic true neoplasms. The study by Reynolds et al12 revealed that nonneoplastic lesions presenting as a primary cardiac neoplasm are composed of nodules of calcium within a background of amorphous fibrinous material. They were described as CATs. The differential diagnosis for CAT includes most benign and malignant cardiac tumors, predominantly myxomas, and nonneoplastic processes such as emboli, thrombi, and vegetations. Cardiac CATs are fragile, pedunculated, located in any of the four chambers, and diffusely calcified. In this reported case, the clinical manifestations and technical investigations were compatible with the diagnosis of a CAT. Even thrombi are easily mistaken for primary cardiac tumors because of their fragile composition, mobility, variable location, and tissue heterogeneity.10,12 The curative treatment of choice for many right sided cardiac tumors is surgical resection, as the potential embolic complications may be debilitating or lethal. Pulmonary embolism (PE) is the most feared complication of right-sided cardiac tumors. PE is also reported to occur during CPB cannulation for surgical resection of right sided tumors via standard sternotomy.13 Although the preoperative diagnosis of the tumor was not definitely stated in the present case, fragile composition of the right sided cardiac masses requires utmost caution and preventive measures during cannulation, either in port access surgery or in a conventional sternotomy approach. Guhathakurta et al1 reported that a 51-year-old man with a right atrial myxoma underwent emergency surgical resection through a median sternotomy. They used a single cannula in the superior vena cava until fibrillation, and then they inserted a cannula into the inferior vena cava. Femoral cannulation was another option in their case, but they did not used it due to such risks as injury to the vein through which it travels, lymphorrhea at the groin and deep vein thrombosis.
Right atrial tumors pose special venous cannulation problems and intraoperative echocardiography may be of benefit in allowing safe cannulation. Both vena cava may be cannulated directly. When low- or high-lying tumor pedicles preclude safe transatrial cannulation, cannulation of the jugular or femoral vein can provide venous drainage of the upper or lower body. In general, we can always cannulate the superior vena cava distal enough from the RA to allow adequate tumor resection, but occasionally femoral venous cannula drainage has been necessary for low-lying right atrial tumors encroaching on the inferior vena cava orifice. If the tumor is large or attached near both caval orifices, peripheral cannulation of both jugular and femoral vein may be used to initiate CPB and deep hypothermia.3 The tip of the femoral vein cannula should be advanced up to hepatic veins under TEE guidance to prevent dislodgement or fragmentation of the tumor in the atrium. One of the reasons of the dislogment in our case could be femoral vein cannulation far into the RA.
The surgical approach for resection of cardiac tumor has to be chosen according to its benefits and drawbacks. As a referral center for video-assisted port access surgery since 1997, mitral valve procedures and tumor resections have been performed at our institution with very low morbidity and mortality.14–16 Port access approach can offer many favorable points. The surgical actions are mostly limited to the thorax and due to the small opening, there is less blood loss, no local cooling, or possible aspiration of fluids, fat, and bone.11 However, surgical manipulations during cannulations should not be underestimated as well. Potential complications of peripheral cannulation could be bleeding, local lacerations, dissection, lymphorrhea at the groin, perforation, and tumor dislodgement or fragmentation as in our case. Port access surgery can provide minimal discomfort, less postoperative pain, fast postoperative recovery, excellent cosmetic healing, and a shortened hospital stay.10 Tumor manipulation should be minimal during this kind of operation to prevent intraoperative tumor-dislodgement and embolization. Another important advantage of port access surgery technique for tumor resection is the minimal manipulation of the heart in comparison with conventional sternotomy approach.
Our hypothesis about this event was that femoral cannulation was the reason of fragmentation of the tumoral mass and KAVD system was the cause of embolization of the tumor in the femoral cannula. To our knowledge, this is the first case of femoral vein cannulation and/or KAVD system in port access surgery that caused dislodgement of an uncommon fragile right atrial tumor and suction into the centrifugal pump.
Minimally invasive approaches constitute an expanding field for the treatment of many cardiac diseases and may be an alternative for the treatment of tumor resections because of less surgical trauma and cosmetic superiority.17 However, the following recommendations may help to reduce the risk of dislodgement and potential embolic events of fragile RA masses in port access surgery: (1) tip of the femoral vein cannula should be advanced up to hepatic veins under TEE guidance and (2) KAVD application should be commenced in case the gravity drainage is not adequate. If it is possible the negative pressure must be kept at a minimal level to achieve sufficient perfusion.
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This is an interesting case report from Dr. Bakir and his colleagues in Aalst, Belgium. Their group have one of the world’s largest experiences with port access surgery. They report a unique complication in which right atrial tumor fragmentation into the venous cannulae resulted in tumor dislodgement into the centrifugal pump and poor venous drainage. The authors suggest ways to avoid this problem, including echocardiographic placement of the femoral venous cannulae up to but not beyond the hepatic veins and the use of gravity drainage or only minimal negative pressure to achieve sufficient perfusion.
Keywords:© 2009 Lippincott Williams & Wilkins, Inc.
Port access; Surgery; Myxoma; Peripheral cannulation