- Email: [email protected]
Infected Atrial Myxoma Presenting With Septic Shock
Infected Atrial Myxoma Presenting With Septic Shock Maggy G. Riad, MD, MSEd, Jeffrey D. Parks, MD, Patrick B. Murphy, RN, JD, and Duraiyah Thangathurai, MD, JD
I
NFECTED RIGHT ATRIAL MYXOMA presenting with sepsis as an emergency is very rare. The hospital course and the case management of a patient with an infected large right atrial myxoma are presented. This case was unique because the initial presentation was septic shock, and the infected tumor was a diagnosis of exclusion during workup for sepsis. CASE REPORT
A 36-year-old man presented to the hospital’s emergency room (ER) complaining of worsening dyspnea on minimal exertion accompanied by fever, fatigue, malaise, nausea, and abdominal pain. He also reported an episode of hemoptysis of a small amount of blood 1 week before admission. The patient denied having chest pain, weight loss, night sweats, syncope, or any focal neurologic symptoms. He had a known history of hepatitis C associated with intravenous heroin abuse. Family and social history were noncontributory. There were no known drug allergies, and he denied taking any medications. The patient appeared ill, jaundiced, cachectic, and tachypneic with a respiratory rate of 22 breaths/min. His blood pressure on presentation was 95/55 mmHg, heart rate was 117 beats/min, and tympanic temperature was 38.5°C. Relevant findings on physical examination included jugular venous distention (8 cm) with positive hepatojugular reflux. Examination of the lungs revealed coarse bilateral breath sounds with inspiratory basal crackles. His heart rhythm was regular. There was III/VI systolic murmur heard loudest at the lower right sternal border with a diastolic rumble heard loudest at the left lower sternal border, and a right ventricular heave could be felt. Abdominal examination revealed a soft and distended abdomen with no organomegaly. Extremities had mild bilateral pitting edema, and a neurologic examination was negative. Electrocardiogram showed sinus tachycardia with an RSR= pattern in V1 and QRS duration of 100 ms suggestive of an incomplete right bundle-branch block. A radiograph of the chest obtained in the supine position showed bilateral atelectasis and pleural effusions. Hematologic laboratory values revealed anemia (hemoglobin, 9 g/dL), leukocytosis (white blood cell count, 22,000/mm3), and a platelet count of 65,000/mm3. The prothrombin level was prolonged (18 seconds) with an international normalized ratio of 1.7, and the partial thromboplastin level was also increased (36 seconds). Other laboratory workup was significant for elevated total bilirubin (5.4 mg/dL), low serum albumin (2g/dL), and elevated serum lactate dehydrogenase level (1,556 U/L). Ultrasound examination of the abdomen revealed mild ascites with no organomegaly. Transthoracic echocardiography was performed in the ER to rule out the presence of infective endocarditis. It revealed an enlarged right atrium occupied by a large, mobile mass, which appeared to be attached to the interatrial septum. The mass measured about 7.5 cm in length.
From the Department of Anesthesiology, University of Southern California, Los Angeles, CA. Address reprint requests to Maggy G. Riad, MD, Department of Anesthesiology, University of Southern California, 1441 Eastlake Avenue, Room 4341, Los Angeles, CA 90033. E-mail: [email protected] © 2005 Elsevier Inc. All rights reserved. 1053-0770/05/1904-0018$30.00/0 doi:10.1053/j.jvca.2005.05.010 Key words: atrial myxoma, infection, septic shock, intraoperative, anesthesia management, TEE examination, cardiac surgery, complications 508
The right ventricle was dilated, and the mass prolapsed into the ventricle with each cardiac cycle, interfering with tricuspid valve closure and causing moderate-to-severe tricuspid regurgitation (TR). There was no evidence of compromise of the right ventricular outflow tract; however, the estimated pulmonary artery pressures were elevated (59/38 mmHg). The aortic valve was normal, and the mitral and pulmonic valves showed a trace of regurgitation. The left atrium was normal in size, and the left ventricle was hyperkinetic with an estimated ejection fraction of 55% and preserved function. A bubble study was negative, and there was no evidence of other vegetation-like lesions. The superior (SVC) and inferior (IVC) vena cavae were examined and were found to be free of thrombus or tumor extension. The patient was admitted with a diagnosis of sepsis, and empiric antibiotic treatment was initiated. Soon after admission and while still in the ER, the patient’s condition rapidly deteriorated and he required intubation, mechanical ventilation, and inotropic support. In the intensive care unit (ICU), he had a brief period of acute renal failure that resolved with fluid administration. Mechanical ventilation was weaned, and the patient was successfully extubated on the sixth day of hospitalization. He remained febrile (37.7°-38.2°C) and continued to require large volumes of fluid for resuscitation, as well as vasoactive drugs (dopamine and norepinephrine) to support his blood pressure. Multiple blood cultures were persistently positive for methicillin-resistant Staphylococcus aureus despite therapeutic blood levels of vancomycin. Other sources of infection could not be found; therefore, the atrial mass was suspected to be the cause of sepsis, and urgent surgical removal was scheduled on the tenth day of hospitalization. In the operating room, standard ASA monitors were placed and a radial arterial catheter was inserted under local anesthesia and intravenous sedation. The patient had an existing femoral venous catheter (triple-lumen catheter) through which dopamine (3 g/kg/min) and norepinephrine (4 g/min) continued to be infused. His baseline blood pressure was 102/60 mmHg, and his heart rate ranged between 94 and 105 beats/min. The induction of anesthesia proceeded with cricoid pressure using fentanyl (1.6 g/kg), ketamine (1.6 mg/kg), and succinylcholine (2 mg/kg). The trachea was successfully intubated, and the patient’s vital signs remained within 10% of starting values. Additional central venous access through the internal jugular vein was placed under direct echocardiographic visualization of the atrial mass to avoid tumor dislodgement or embolization. On the bicaval view, the junction between the SVC and the right atrium was visualized (Fig 1). During the central catheter placement, the guidewire could be seen close to the mass (Fig 2A and B). An 8F catheter was placed without incident with the tip located well above the tumor. Jugular central venous pressure (CVP) tracing showed a pattern consistent with TR, and pressures ranged between 8 to 12 mmHg. Transesophageal echocardiography (TEE) examination showed flow dynamics of the mass; during early diastole, the mass floated freely inside the enlarged right atrium limiting venous return (Fig 3). With atrial contraction, the mass moved through the tricuspid valve extending into the right ventricle as the atrium contracted around it. During ventricular contraction, the mass moved back toward the atrium, preventing tricuspid valve closure and causing severe TR (Fig 4). The mass could be seen tethered to the interatrial septum. The IVC and hepatic veins were examined and were free of tumor extensions; however, they were found to be distended, likely contributing to the patient’s liver dysfunction. Surgical approach was through a median sternotomy. During surgical placement of the venous cannulae and throughout the entire case (except for the bypass phase), the mass was monitored by TEE for signs of disruption or embolization. One of the venous cannulae was placed
Journal of Cardiothoracic and Vascular Anesthesia, Vol 19, No 4 (August), 2005: pp 508-511
INFECTED ATRIAL MYXOMA
Fig 1.
Junction of SVC with right atrium.
in the IVC through the femoral route and was used for initiation of cardiopulmonary bypass (CPB). The second cannula was placed in the SVC after the initiation of CPB. The aortic cross-clamp was applied, and in addition a tourniquet was applied to the pulmonary artery to minimize embolization to the pulmonary circulation during surgical resection. After cardiac standstill, the right atrium was opened and the mass was removed in its entirety (Fig 5). It was originating from the interatrial septum at the area of the fossa ovalis. The left atrium was examined and found free of tumor, and the septal defect that was created by the removal of the mass was closed. The tricuspid valve was also inspected and was found to be intact. A complete TEE examination was performed to check for any new valvular lesions, shunts, air, clots in the pulmonary vessels, or residual tumor in the right cardiac chambers. The patient was successfully weaned from CPB and a flow-directed pulmonary artery catheter was floated. Hemodynamic measurements
509
Fig 3.
At the beginning of diastole, the ventricle fills.
showed a CVP between 7 to 9 mmHg; pulmonary artery pressures were in the range of 37 to 42 mmHg systolic and 23 to 30 mmHg diastolic. Systemic blood pressure and the CVP waveform were consistent with persistent TR (Fig 6), which was also confirmed by TEE. At the conclusion of surgery, the patient remained intubated and was transferred to the ICU on phenylephrine (4 g/min) and dopamine (3 g/kg/min). The patient was extubated on the third postoperative day but continued to require vasopressors for blood pressure support, which were gradually decreased over the next 3 days, and weaned on postoperative day 6. He was discharged from ICU the following day and was observed on the ward for another 27 days, where he continued to receive antibiotic treatment. His international normalized ratio remained elevated in the postoperative period, and no anticoagulation was given. His ascites improved, and his neurologic examination remained negative. The patient was discharged home on postoperative day 34. The final pathology report described an 8 ⫻ 5 cm atrial myxoma, infected with gram-positive cocci (S aureus) in clusters. DISCUSSION
Myxomas represent the most common benign cardiac tumors and are thought to occur in 0.2% to 0.3% of the population; however, the exact incidence is unknown. Men are more frequently affected than women. The majority (75%-80%) arise from the left atrium. Right-sided tumors are less frequently encountered and constitute only 18% of all cardiac myxomas. Renal cell carcinoma invading the renal vein can extend into the hepatic vein, the IVC, and the right atrium depending on the stage or level. Ten percent of all renal cell cancers grow into the IVC, and 1% invade the right atrium1 and can be erroneously
Fig 2. (A) Guidewire at closest point during systole. (B) Guidewire at closest point during diastole.
Fig 4.
Systole results in a disturbed valve and severe TR.
510
Fig 5. Gross photography of the mass. (Color version of figure is available online.)
diagnosed as tumors originating from the right atrium. In patients presenting with right atrial masses, it is necessary to exclude renal and IVC pathology before proceeding with cardiac surgery to avoid performing an unnecessary sternotomy.2 Infected cardiac myxomas are very rare. According to Revankar and Clark’s review of the world’s literature,3 there are only a total of 40 reported cases and only 4 of these were right-sided tumors. Diagnostic criteria of definite infected cardiac myxomas are either documented myxoma on pathology and microorganisms seen by pathology or positive blood cultures and inflammation seen by pathology. Right-sided intracavitary masses can present with obstructive, constitutional, or embolic symptoms. Obstruction of the right ventricular outflow tract results in signs of right-sided congestion with elevated JVD, ascites, and lower-extremity edema. Constitutional symptoms, although more predominant with left-sided tumors, include fever, fatigue, malaise, weight loss, rash, myalgia, abdominal pain, nausea, and clubbing.4 Elevated erythrocyte sedimentation rate, hemolytic anemia, thrombocytopenia, leucocytosis, and elevated creatine kinase levels are frequently encountered on laboratory analysis. Elevated lactate dehydrogenase levels may be explained by pulmonary events like an infarction or an embolus or may be secondary to liver congestion. Embolization of thrombus or tumor fragments affects the cerebral and systemic circulation in left-sided lesions, whereas right-sided myxomas embolize to the lungs and the pulmonary circulation.5 This can result in pleuritic symptoms, chronic pulmonary hypertension, and cor pulmonale and may be fatal. The patient presented in this case report had obstructive and constitutional symptoms at the time of admission to the authors’ facility. He had an episode of hemoptysis before admission that could have represented an embolic event or could have been the result of his chronic ongoing liver disease. The anesthesia management of this case presented the authors with the following challenges: (1) ongoing sepsis requiring blood pressure support with large volumes of fluid and vasoactive agents, (2) limited venous return because of the presence of a large mass occupying most of the right atrium and ventricle, and (3) high risk of showering septic emboli or tumor fragments with right atrial manipulation. This patient presented with sepsis confirmed with blood
RIAD ET AL
cultures and hemodynamic collapse. Sepsis is a syndrome of an overwhelming systemic response to infection. It is associated with widespread inflammation, coagulation abnormalities, and tissue hypoperfusion that can rapidly lead to shock, organ failure, and death. Early fluid resuscitation is important for restoring mean circulatory pressure, improving tissue oxygenation, and maintaining organ perfusion. This was difficult to accomplish in this patient given the size and position of the atrial mass and its interference with venous return. The lack of reliable measures of the mean circulatory filling pressures added to the challenge in the assessment and guidance of resuscitation efforts. Countering the effects of mechanical ventilation and the restricting effects of increased pleural pressures on ventricular filling made the maintenance of adequate preload more difficult. Mean arterial blood pressure was maintained with fluids and vasopressors that helped the patient recover from an episode of acute renal failure that developed soon after the initiation of mechanical ventilation during the early hospitalization course. After induction of general anesthesia, it was necessary to obtain additional venous access to continue with aggressive fluid resuscitation and blood pressure control. The patient had an existing femoral catheter on one side, and the other groin was planned to be used for surgical venous cannulation. Venous access in the neck is desirable because it provides easy access for the infusion of vasoactive substances and serves as an introducer site for floating a pulmonary artery catheter, which is useful for measurement of hemodynamic parameters in the postoperative period. Central venous catheterization in the head and neck was particularly challenging in this case given the high risk of disturbing the atrial mass and showering septic emboli into the pulmonary circulation. This required the placement of the jugular catheter under direct echocardiographic visualization of the mass. Extra precautions were taken during the TEE probe placement. Given the patient’s long standing history of hepatitis, the presence of ascites, and the history of hemoptysis, the authors wanted to rule out the presence of esophageal varices first before inserting the TEE probe. An 18F Foley catheter was inserted into the esophagus and lubricant was injected through it into the esophageal lumen and the absence of blood was noted on its removal. Subse-
Fig 6. Electrocardiogram and CVP juxtaposed, showing the A, C, and V waves of severe TR.
INFECTED ATRIAL MYXOMA
511
quently, the TEE probe was inserted without difficulty. The lubrication technique described is routinely used by the authors before the insertion of the large (60F) bougie required during Nissen fundoplication surgery. Septic embolization from an infected right atrial mass may cause chronic pulmonary hypertension from recurrent pulmonary thromboemboli or may result in sudden death by a fatal acute event. This patient presented with signs of right ventricular strain that were confirmed on the first echocardiographic examination. Worsening respiratory status necessitating mechanical ventilation may have been the result of an embolic event or a consequence of septic shock. All possible measures were taken to monitor and prevent intraoperative embolization. The authors used the TEE to guide the placement of the venous access catheter and directly visualize the guidewire and the catheter tip, thereby avoiding unnecessary tumor mobilization. TEE also helped to ascertain the exact position of the IVC venous cannula at the junction of IVC with the atrium. In addition, a tourniquet was placed around the pulmonary artery before surgical manipulation of the mass in an attempt to isolate the pulmonary system and prevent intraoperative embolization.
The availability of intraoperative TEE examination played other pivotal roles in the management of this patient. The presence of a large atrial mass caused severe tricuspid regurgitation, rendering the CVP measurement unreliable for evaluation of the volume status. TEE was therefore helpful in evaluating the volume status and guiding the fluid administration. A comprehensive TEE examination was valuable before initiation of surgical intervention to confirm the location of the tumor and to rule out extension of thrombus into or from the IVC. TEE monitoring during surgical manipulation of the heart also helped in early detection and diagnosis of tumor fragmentation, dislodgement, or embolization, which can have fatal consequences and may hinder weaning from CPB. TEE monitoring during surgical removal of the mass also helped to ensure completeness of excision and rule out the existence of residual atrial septal defects after surgical closure. In summary, this patient presented the authors with many challenges. The institution of early and aggressive resuscitation measures, coordination of the intraoperative approach with the surgical team, and the use of intraoperative TEE helped contribute to the good outcome in this case.
REFERENCES 1. Chiappini B, Savini C, Marinelli G, et al: Cavoatrial tumor thrombus: Single-stage surgical approach with profound hypothermia and circulatory arrest, including a review of the literature. J Thorac Cardiovasc Surg 124:684-688, 2002 2. Mellor A, Clark J, Morgan-Hughes NJ, et al: Transesophageal echocardiography during surgery for intra-atrial masses. Anesthesia 58:283-285, 2003
3. Revankar SG, Clark RA: Infected cardiac myxoma: Case report and literature review. Medicine 77:337-344, 1998 4. Attar S, Lee Y-C, Singelton R, et al: Cardiac myxoma. Ann Thorac Surg 29:397-405, 1980 5. Peters MN, Hall RJ, Cooley DA, et al: The clinical syndrome of atrial myxoma. JAMA 230:695-701, 1974
I
NFECTED RIGHT ATRIAL MYXOMA presenting with sepsis as an emergency is very rare. The hospital course and the case management of a patient with an infected large right atrial myxoma are presented. This case was unique because the initial presentation was septic shock, and the infected tumor was a diagnosis of exclusion during workup for sepsis. CASE REPORT
A 36-year-old man presented to the hospital’s emergency room (ER) complaining of worsening dyspnea on minimal exertion accompanied by fever, fatigue, malaise, nausea, and abdominal pain. He also reported an episode of hemoptysis of a small amount of blood 1 week before admission. The patient denied having chest pain, weight loss, night sweats, syncope, or any focal neurologic symptoms. He had a known history of hepatitis C associated with intravenous heroin abuse. Family and social history were noncontributory. There were no known drug allergies, and he denied taking any medications. The patient appeared ill, jaundiced, cachectic, and tachypneic with a respiratory rate of 22 breaths/min. His blood pressure on presentation was 95/55 mmHg, heart rate was 117 beats/min, and tympanic temperature was 38.5°C. Relevant findings on physical examination included jugular venous distention (8 cm) with positive hepatojugular reflux. Examination of the lungs revealed coarse bilateral breath sounds with inspiratory basal crackles. His heart rhythm was regular. There was III/VI systolic murmur heard loudest at the lower right sternal border with a diastolic rumble heard loudest at the left lower sternal border, and a right ventricular heave could be felt. Abdominal examination revealed a soft and distended abdomen with no organomegaly. Extremities had mild bilateral pitting edema, and a neurologic examination was negative. Electrocardiogram showed sinus tachycardia with an RSR= pattern in V1 and QRS duration of 100 ms suggestive of an incomplete right bundle-branch block. A radiograph of the chest obtained in the supine position showed bilateral atelectasis and pleural effusions. Hematologic laboratory values revealed anemia (hemoglobin, 9 g/dL), leukocytosis (white blood cell count, 22,000/mm3), and a platelet count of 65,000/mm3. The prothrombin level was prolonged (18 seconds) with an international normalized ratio of 1.7, and the partial thromboplastin level was also increased (36 seconds). Other laboratory workup was significant for elevated total bilirubin (5.4 mg/dL), low serum albumin (2g/dL), and elevated serum lactate dehydrogenase level (1,556 U/L). Ultrasound examination of the abdomen revealed mild ascites with no organomegaly. Transthoracic echocardiography was performed in the ER to rule out the presence of infective endocarditis. It revealed an enlarged right atrium occupied by a large, mobile mass, which appeared to be attached to the interatrial septum. The mass measured about 7.5 cm in length.
From the Department of Anesthesiology, University of Southern California, Los Angeles, CA. Address reprint requests to Maggy G. Riad, MD, Department of Anesthesiology, University of Southern California, 1441 Eastlake Avenue, Room 4341, Los Angeles, CA 90033. E-mail: [email protected] © 2005 Elsevier Inc. All rights reserved. 1053-0770/05/1904-0018$30.00/0 doi:10.1053/j.jvca.2005.05.010 Key words: atrial myxoma, infection, septic shock, intraoperative, anesthesia management, TEE examination, cardiac surgery, complications 508
The right ventricle was dilated, and the mass prolapsed into the ventricle with each cardiac cycle, interfering with tricuspid valve closure and causing moderate-to-severe tricuspid regurgitation (TR). There was no evidence of compromise of the right ventricular outflow tract; however, the estimated pulmonary artery pressures were elevated (59/38 mmHg). The aortic valve was normal, and the mitral and pulmonic valves showed a trace of regurgitation. The left atrium was normal in size, and the left ventricle was hyperkinetic with an estimated ejection fraction of 55% and preserved function. A bubble study was negative, and there was no evidence of other vegetation-like lesions. The superior (SVC) and inferior (IVC) vena cavae were examined and were found to be free of thrombus or tumor extension. The patient was admitted with a diagnosis of sepsis, and empiric antibiotic treatment was initiated. Soon after admission and while still in the ER, the patient’s condition rapidly deteriorated and he required intubation, mechanical ventilation, and inotropic support. In the intensive care unit (ICU), he had a brief period of acute renal failure that resolved with fluid administration. Mechanical ventilation was weaned, and the patient was successfully extubated on the sixth day of hospitalization. He remained febrile (37.7°-38.2°C) and continued to require large volumes of fluid for resuscitation, as well as vasoactive drugs (dopamine and norepinephrine) to support his blood pressure. Multiple blood cultures were persistently positive for methicillin-resistant Staphylococcus aureus despite therapeutic blood levels of vancomycin. Other sources of infection could not be found; therefore, the atrial mass was suspected to be the cause of sepsis, and urgent surgical removal was scheduled on the tenth day of hospitalization. In the operating room, standard ASA monitors were placed and a radial arterial catheter was inserted under local anesthesia and intravenous sedation. The patient had an existing femoral venous catheter (triple-lumen catheter) through which dopamine (3 g/kg/min) and norepinephrine (4 g/min) continued to be infused. His baseline blood pressure was 102/60 mmHg, and his heart rate ranged between 94 and 105 beats/min. The induction of anesthesia proceeded with cricoid pressure using fentanyl (1.6 g/kg), ketamine (1.6 mg/kg), and succinylcholine (2 mg/kg). The trachea was successfully intubated, and the patient’s vital signs remained within 10% of starting values. Additional central venous access through the internal jugular vein was placed under direct echocardiographic visualization of the atrial mass to avoid tumor dislodgement or embolization. On the bicaval view, the junction between the SVC and the right atrium was visualized (Fig 1). During the central catheter placement, the guidewire could be seen close to the mass (Fig 2A and B). An 8F catheter was placed without incident with the tip located well above the tumor. Jugular central venous pressure (CVP) tracing showed a pattern consistent with TR, and pressures ranged between 8 to 12 mmHg. Transesophageal echocardiography (TEE) examination showed flow dynamics of the mass; during early diastole, the mass floated freely inside the enlarged right atrium limiting venous return (Fig 3). With atrial contraction, the mass moved through the tricuspid valve extending into the right ventricle as the atrium contracted around it. During ventricular contraction, the mass moved back toward the atrium, preventing tricuspid valve closure and causing severe TR (Fig 4). The mass could be seen tethered to the interatrial septum. The IVC and hepatic veins were examined and were free of tumor extensions; however, they were found to be distended, likely contributing to the patient’s liver dysfunction. Surgical approach was through a median sternotomy. During surgical placement of the venous cannulae and throughout the entire case (except for the bypass phase), the mass was monitored by TEE for signs of disruption or embolization. One of the venous cannulae was placed
Journal of Cardiothoracic and Vascular Anesthesia, Vol 19, No 4 (August), 2005: pp 508-511
INFECTED ATRIAL MYXOMA
Fig 1.
Junction of SVC with right atrium.
in the IVC through the femoral route and was used for initiation of cardiopulmonary bypass (CPB). The second cannula was placed in the SVC after the initiation of CPB. The aortic cross-clamp was applied, and in addition a tourniquet was applied to the pulmonary artery to minimize embolization to the pulmonary circulation during surgical resection. After cardiac standstill, the right atrium was opened and the mass was removed in its entirety (Fig 5). It was originating from the interatrial septum at the area of the fossa ovalis. The left atrium was examined and found free of tumor, and the septal defect that was created by the removal of the mass was closed. The tricuspid valve was also inspected and was found to be intact. A complete TEE examination was performed to check for any new valvular lesions, shunts, air, clots in the pulmonary vessels, or residual tumor in the right cardiac chambers. The patient was successfully weaned from CPB and a flow-directed pulmonary artery catheter was floated. Hemodynamic measurements
509
Fig 3.
At the beginning of diastole, the ventricle fills.
showed a CVP between 7 to 9 mmHg; pulmonary artery pressures were in the range of 37 to 42 mmHg systolic and 23 to 30 mmHg diastolic. Systemic blood pressure and the CVP waveform were consistent with persistent TR (Fig 6), which was also confirmed by TEE. At the conclusion of surgery, the patient remained intubated and was transferred to the ICU on phenylephrine (4 g/min) and dopamine (3 g/kg/min). The patient was extubated on the third postoperative day but continued to require vasopressors for blood pressure support, which were gradually decreased over the next 3 days, and weaned on postoperative day 6. He was discharged from ICU the following day and was observed on the ward for another 27 days, where he continued to receive antibiotic treatment. His international normalized ratio remained elevated in the postoperative period, and no anticoagulation was given. His ascites improved, and his neurologic examination remained negative. The patient was discharged home on postoperative day 34. The final pathology report described an 8 ⫻ 5 cm atrial myxoma, infected with gram-positive cocci (S aureus) in clusters. DISCUSSION
Myxomas represent the most common benign cardiac tumors and are thought to occur in 0.2% to 0.3% of the population; however, the exact incidence is unknown. Men are more frequently affected than women. The majority (75%-80%) arise from the left atrium. Right-sided tumors are less frequently encountered and constitute only 18% of all cardiac myxomas. Renal cell carcinoma invading the renal vein can extend into the hepatic vein, the IVC, and the right atrium depending on the stage or level. Ten percent of all renal cell cancers grow into the IVC, and 1% invade the right atrium1 and can be erroneously
Fig 2. (A) Guidewire at closest point during systole. (B) Guidewire at closest point during diastole.
Fig 4.
Systole results in a disturbed valve and severe TR.
510
Fig 5. Gross photography of the mass. (Color version of figure is available online.)
diagnosed as tumors originating from the right atrium. In patients presenting with right atrial masses, it is necessary to exclude renal and IVC pathology before proceeding with cardiac surgery to avoid performing an unnecessary sternotomy.2 Infected cardiac myxomas are very rare. According to Revankar and Clark’s review of the world’s literature,3 there are only a total of 40 reported cases and only 4 of these were right-sided tumors. Diagnostic criteria of definite infected cardiac myxomas are either documented myxoma on pathology and microorganisms seen by pathology or positive blood cultures and inflammation seen by pathology. Right-sided intracavitary masses can present with obstructive, constitutional, or embolic symptoms. Obstruction of the right ventricular outflow tract results in signs of right-sided congestion with elevated JVD, ascites, and lower-extremity edema. Constitutional symptoms, although more predominant with left-sided tumors, include fever, fatigue, malaise, weight loss, rash, myalgia, abdominal pain, nausea, and clubbing.4 Elevated erythrocyte sedimentation rate, hemolytic anemia, thrombocytopenia, leucocytosis, and elevated creatine kinase levels are frequently encountered on laboratory analysis. Elevated lactate dehydrogenase levels may be explained by pulmonary events like an infarction or an embolus or may be secondary to liver congestion. Embolization of thrombus or tumor fragments affects the cerebral and systemic circulation in left-sided lesions, whereas right-sided myxomas embolize to the lungs and the pulmonary circulation.5 This can result in pleuritic symptoms, chronic pulmonary hypertension, and cor pulmonale and may be fatal. The patient presented in this case report had obstructive and constitutional symptoms at the time of admission to the authors’ facility. He had an episode of hemoptysis before admission that could have represented an embolic event or could have been the result of his chronic ongoing liver disease. The anesthesia management of this case presented the authors with the following challenges: (1) ongoing sepsis requiring blood pressure support with large volumes of fluid and vasoactive agents, (2) limited venous return because of the presence of a large mass occupying most of the right atrium and ventricle, and (3) high risk of showering septic emboli or tumor fragments with right atrial manipulation. This patient presented with sepsis confirmed with blood
RIAD ET AL
cultures and hemodynamic collapse. Sepsis is a syndrome of an overwhelming systemic response to infection. It is associated with widespread inflammation, coagulation abnormalities, and tissue hypoperfusion that can rapidly lead to shock, organ failure, and death. Early fluid resuscitation is important for restoring mean circulatory pressure, improving tissue oxygenation, and maintaining organ perfusion. This was difficult to accomplish in this patient given the size and position of the atrial mass and its interference with venous return. The lack of reliable measures of the mean circulatory filling pressures added to the challenge in the assessment and guidance of resuscitation efforts. Countering the effects of mechanical ventilation and the restricting effects of increased pleural pressures on ventricular filling made the maintenance of adequate preload more difficult. Mean arterial blood pressure was maintained with fluids and vasopressors that helped the patient recover from an episode of acute renal failure that developed soon after the initiation of mechanical ventilation during the early hospitalization course. After induction of general anesthesia, it was necessary to obtain additional venous access to continue with aggressive fluid resuscitation and blood pressure control. The patient had an existing femoral catheter on one side, and the other groin was planned to be used for surgical venous cannulation. Venous access in the neck is desirable because it provides easy access for the infusion of vasoactive substances and serves as an introducer site for floating a pulmonary artery catheter, which is useful for measurement of hemodynamic parameters in the postoperative period. Central venous catheterization in the head and neck was particularly challenging in this case given the high risk of disturbing the atrial mass and showering septic emboli into the pulmonary circulation. This required the placement of the jugular catheter under direct echocardiographic visualization of the mass. Extra precautions were taken during the TEE probe placement. Given the patient’s long standing history of hepatitis, the presence of ascites, and the history of hemoptysis, the authors wanted to rule out the presence of esophageal varices first before inserting the TEE probe. An 18F Foley catheter was inserted into the esophagus and lubricant was injected through it into the esophageal lumen and the absence of blood was noted on its removal. Subse-
Fig 6. Electrocardiogram and CVP juxtaposed, showing the A, C, and V waves of severe TR.
INFECTED ATRIAL MYXOMA
511
quently, the TEE probe was inserted without difficulty. The lubrication technique described is routinely used by the authors before the insertion of the large (60F) bougie required during Nissen fundoplication surgery. Septic embolization from an infected right atrial mass may cause chronic pulmonary hypertension from recurrent pulmonary thromboemboli or may result in sudden death by a fatal acute event. This patient presented with signs of right ventricular strain that were confirmed on the first echocardiographic examination. Worsening respiratory status necessitating mechanical ventilation may have been the result of an embolic event or a consequence of septic shock. All possible measures were taken to monitor and prevent intraoperative embolization. The authors used the TEE to guide the placement of the venous access catheter and directly visualize the guidewire and the catheter tip, thereby avoiding unnecessary tumor mobilization. TEE also helped to ascertain the exact position of the IVC venous cannula at the junction of IVC with the atrium. In addition, a tourniquet was placed around the pulmonary artery before surgical manipulation of the mass in an attempt to isolate the pulmonary system and prevent intraoperative embolization.
The availability of intraoperative TEE examination played other pivotal roles in the management of this patient. The presence of a large atrial mass caused severe tricuspid regurgitation, rendering the CVP measurement unreliable for evaluation of the volume status. TEE was therefore helpful in evaluating the volume status and guiding the fluid administration. A comprehensive TEE examination was valuable before initiation of surgical intervention to confirm the location of the tumor and to rule out extension of thrombus into or from the IVC. TEE monitoring during surgical manipulation of the heart also helped in early detection and diagnosis of tumor fragmentation, dislodgement, or embolization, which can have fatal consequences and may hinder weaning from CPB. TEE monitoring during surgical removal of the mass also helped to ensure completeness of excision and rule out the existence of residual atrial septal defects after surgical closure. In summary, this patient presented the authors with many challenges. The institution of early and aggressive resuscitation measures, coordination of the intraoperative approach with the surgical team, and the use of intraoperative TEE helped contribute to the good outcome in this case.
REFERENCES 1. Chiappini B, Savini C, Marinelli G, et al: Cavoatrial tumor thrombus: Single-stage surgical approach with profound hypothermia and circulatory arrest, including a review of the literature. J Thorac Cardiovasc Surg 124:684-688, 2002 2. Mellor A, Clark J, Morgan-Hughes NJ, et al: Transesophageal echocardiography during surgery for intra-atrial masses. Anesthesia 58:283-285, 2003
3. Revankar SG, Clark RA: Infected cardiac myxoma: Case report and literature review. Medicine 77:337-344, 1998 4. Attar S, Lee Y-C, Singelton R, et al: Cardiac myxoma. Ann Thorac Surg 29:397-405, 1980 5. Peters MN, Hall RJ, Cooley DA, et al: The clinical syndrome of atrial myxoma. JAMA 230:695-701, 1974