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Bilateral acute lower limb ischemia secondary to complete embolization of cardiac myxoma
Bilateral acute lower limb ischemia secondary to complete embolization of cardiac myxoma Kelvin Kam Fai Ho, MBBS/BSc,a Ramez Barsoum, MBBS,a Benjamin Shepherd, MBBS,b and Timothy McGahan, MBBS, FRACS,a Woollongabba, Queensland, Australia
ABSTRACT Cardiac myxomas are the most common benign cardiac tumors in adults that can present with peripheral embolization. Complete detachments of myxomas are rare and tend to cause aortoiliac embolism. We report a case of a middle-aged woman with bilateral popliteal artery and segmental renal artery embolisms secondary to a completely detached cardiac myxoma. This case highlights cardiac myxomas as an important cause of acute limb ischemia and that it is not excluded by a normal echocardiogram result. (J Vasc Surg 2019;-:1-3.) Keywords: Cardiac myxoma; Atrial myxoma; Limb ischemia; Peripheral embolization
A 50-year-old woman presented to the emergency department with sudden right leg pain and paresthesia during her morning run. This pain was associated with subtle discomfort in the right popliteal fossa for several weeks prior. She is otherwise healthy, without history of cardiovascular or thromboembolic diseases, or malignancy. She did not have any recent trauma. She is a nonsmoker and takes levonorgestrel as her oral contraceptive. On examination, she had normal femoral and popliteal pulses bilaterally, without palpable pedal pulses. Her legs were cold and painful bilaterally. Her right leg was tense and tender in the anterior compartment, associated with an insensate right foot. Her cardiac examination was normal, with no additional heart sounds or murmurs. Her abdominal examination was also unremarkable. An emergent computed tomography angiogram (CTA) confirmed bilateral popliteal occlusions, as well as bilateral segmental renal infarcts (Fig 1). It did not demonstrate any atheroma or thrombus in the distal thoracic aorta and abdominal aorta that could be the embolic source. It did not show features of anatomic popliteal entrapment. The patient was diagnosed with acute limb ischemia and compartment syndrome. She was started on an intravenous heparin infusion and was taken to the operating room urgently. Bilateral popliteal embolectomies were performed through medial infrageniculate approaches to the From the Department of Vascular Surgery,a and the Pathology Queensland,b Princess Alexandra Hospital. Author conflict of interest: none. Correspondence: Kelvin Kam Fai Ho, MBBS/BSc, Department of Vascular Surgery, Princess Alexandra Hospital, PO Box 8101, Woolloongabba, QLD 4102, Australia (e-mail: [email protected]). The editors and reviewers of this article have no relevant financial relationships to disclose per the JVS policy that requires reviewers to decline review of any manuscript for which they may have a conflict of interest. 0741-5214 Copyright Ó 2019 by the Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved. https://doi.org/10.1016/j.jvs.2019.08.264
popliteal arteries. A single compartment fasciotomy was performed for the anterior compartment as well. The embolus consisting of an abundance of firm paleyellow gelatinous material was removed from popliteal and tibial arteries bilaterally using Fogarty catheters (Fig 2). The arteries were closed and the fasciotomy wound was left open for delayed closure. On completion of her embolectomy, the patient had palpable pedal pulses bilaterally. Her postoperative recovery was unremarkable. Delayed closure of her fasciotomy wound was performed as planned. Given the findings on CTA, a cardioembolic source was thought to be likely. Therefore, a transesophageal echocardiogram (TEE) was performed during the operation, which showed normal size and function of her left and right ventricles, no valvular abnormalities, and no intracardiac defects or intracardiac masses. Histopathology of the emboli from both popliteal embolectomies showed features consistent with cardiac myxoma with lepidic cells seen in corded arrangement (Fig 3). Further investigation of the cause of the thromboembolic disease was performed in the subsequent days. Her thrombophilia serology and genetic sequencing was negative. Likewise, a second CTA did not show any disease in her thoracic aorta. Further investigation with cardiac magnetic resonance imaging (MRI) did not identify any focal mass lesion or any definite filling defect, suggesting complete embolization of a cardiac myxoma. The patient was started on rivaroxaban because the original site of attachment of the cardiac myxoma was thought to predispose to further thromboembolic disease. The patient was discharged on postoperative day 7 and returned 10 days later for a planned repeat TEE, which was normal again. She continues to attend follow-up clinic appointments with a vascular surgeon and her anticoagulation will be continued until her next surveillance echocardiogram in 12 months. Written informed consent has been given by the patient for the publication of the case details and images. 1
2
Ho et al
Journal of Vascular Surgery ---
2019
Fig 2. Fragments of pale yellow emboli from bilateral popliteal embolectomies, later shown to be cardiac myxomas histologically.
Fig 1. Computed tomography angiography (CTA) scan with three-dimensional reconstruction showing abrupt cutoff in the above-knee popliteal artery and below-knee popliteal artery of the right and left legs, respectively.
DISCUSSION Cardiac myxomas are the most common benign intracardiac tumors in adults, accounting for one-half of all cases.1 Myxomas can originate from any chamber within the heart. However, the attachment to the left atrium, typically along the interatrial septum, account for the vast majority of cases.1,2 They are most common in the third to sixth decades of life, with a slight female predominance.1 Surgical removal is the treatment of choice. Although this is usually curative, rare recurrence after excision have been reported.1,2 Clinical presentations of cardiac myxomas are highly variable. Roughly 20% of myxomas remain asymptomatic and are found incidentally on imaging.2 Typical presentations of cardiac myxomas include cardiac obstruction, constitutional symptoms such as fever and fatigue, and embolization.1,2 Myxomatous embolism affects the cerebral circulation in most cases; however, any vascular bed can be affected. Embolism causing stroke, limb ischemia, visceral ischemia, and even myocardial infarcts have been reported.1-3 Complete detachments of cardiac myxomas are rare and typically result in aortoiliac embolism.4-10 Early literature has described fatal saddle embolisms of the aortic bifurcation from complete dislodgement of cardiac
myxomas. In these instances, examination of the left atrium during autopsy demonstrated a pedicle, residual thrombus or a rough surface on the endocardium which are thought to be the original attachment of the myxomas.4-7 In the rare survivable cases of complete myxoma embolization, cardiac angiography, echocardiogram, or MRI have been used to exclude residual intracardiac tumour.8-10 Among early reports of myxoma embolization, Carter et al8 described a case of bilateral acute limb ischemia that was treated with aortic embolectomy. Myxomatous tissue was seen in the retrieved embolus. Postoperative angiocardiography was performed and demonstrated no intra-atrial tumor mass. No further investigations were performed given that the patient was in “good health.” Fang et al9 reported a case of infrarenal aortic occlusion with total dislodgement of a cardiac myxoma. A transthoracic echocardiogram and TEE performed preoperatively and repeated examination up to 1 year postoperatively could not identify any residual intracardiac tumor. Coley et al10 reported a case of acute limb ischemia caused by complete detachment of an atrial myxoma. Myxomatous tissue was retrieved from the bilateral iliac and femoropopliteal arteries. Postoperative TEE and MRI demonstrated a residual irregularity on the interatrial septum, which was thought to be the original site of attachment of the embolized myxoma. In our current case, the cardiac myxoma was completely embolized and did not leave any identifiable impression of its original attachment despite multimodality imaging. However, we have commenced anticoagulation with rivaroxaban for our patient given previous autopsy studies have demonstrated residual abnormalities to the endothelium after complete detachment of myxomas. Among patients who underwent surgical removal of myxomas,
Journal of Vascular Surgery Volume
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Number
Ho et al
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Fig 3. Histopathology of a popliteal embolus with hematoxylin and eosin stain showing typical features of cardiac myxoma with papillary projections and an abundant loose myxoid stroma under 50 magnification (A) with cells arranged in strands under 200 magnification (B).
recurrence has been noted even several years later.11 Although there are no reported cases of recurrence after total embolization of myxomas, we have elected to continue long-term surveillance with repeat imaging to monitor for this possibility. Our case highlights the importance of cardiac myxomas as a cause of acute limb ischemia. Although completely embolized myxomas tend to cause aortoiliac occlusion, the present case demonstrates that fragmentation of the tumor and small vessel occlusion can occur as well. Cardiac myxomas should be suspected in young and previously healthy patients who present with acute limb ischemia. A normal echocardiogram does not exclude the diagnosis and histologic examination of the embolus is mandatory in such cases. In cases where embolism from a cardiac myxoma is suspected as the cause of acute limb ischemia, a high index of suspicion for embolism into other vascular beds is warranted.
REFERENCES 1. Reynen K. Cardiac myxomas. N Engl J Med 1995;333:1610-7. 2. Wang JG, Li YJ, Liu H, Li NN, Zhao J, Xing X. Clinicopathologic analysis of cardiac myxomas: Seven years’ experience with 61 patients. J Thorac Dis 2012;4:272-83. 3. Vogel B, Thomas D, Mereles D, Rottbauer W, Katus HA. Systemic embolization and myocardial infarction due to
4.
5.
6.
7.
8. 9.
10.
11.
clinically unrecognized left atrial myxoma. Case Rep Med 2011;2011:159024. Silverman J, Olwin JS, Graettinger JS. Cardiac myxomas with systemic embolization. Review of the literature and report of a case. Circulation 1962;26:99-103. Ohgo T, Yamamoto K, Furuno T. Complete detachment of cardiac myxoma causing aortic saddle embolization and cerebral infarction. Int J Cardiol 2008;127:e48-9. Kulkarni M, Jessiman IM, French S. Entire left atrial myxoma presenting as a saddle embolus. Thorax 1969;24: 629-31. Eriksen UH, Baandrup U, Jensen BS. Total disruption of left atrial myxoma causing a cerebral attack and a saddle embolus in the iliac bifurcation. Int J Cardiol 1992;35:127-9. Carter AB, Lowe KG, Hill IG. Cardiac myxomata and aortic saddle embolism. Br Heart J 1960;22:502-4. Fang BR, Chang CP, Cheng CW, Yang NI, Shieh MC, Lee N. Total detachment of cardiac myxoma causing saddle embolization and mimicking aortic dissection. Jpn Heart J 2004;45:359-63. Coley C, Lee KR, Steiner M, Thompson CS. Complete embolization of a left atrial myxoma resulting in acute lower extremity ischemia. Tex Heart Inst J 2005;32:238-40. Pinede L, Duhaut P, Loire R. Clinical presentation of left atrial cardiac myxoma. A series of 112 consecutive cases. Medicine (Baltimore) 2001;80:159-72.
Submitted May 1, 2019; accepted Aug 6, 2019.
ABSTRACT Cardiac myxomas are the most common benign cardiac tumors in adults that can present with peripheral embolization. Complete detachments of myxomas are rare and tend to cause aortoiliac embolism. We report a case of a middle-aged woman with bilateral popliteal artery and segmental renal artery embolisms secondary to a completely detached cardiac myxoma. This case highlights cardiac myxomas as an important cause of acute limb ischemia and that it is not excluded by a normal echocardiogram result. (J Vasc Surg 2019;-:1-3.) Keywords: Cardiac myxoma; Atrial myxoma; Limb ischemia; Peripheral embolization
A 50-year-old woman presented to the emergency department with sudden right leg pain and paresthesia during her morning run. This pain was associated with subtle discomfort in the right popliteal fossa for several weeks prior. She is otherwise healthy, without history of cardiovascular or thromboembolic diseases, or malignancy. She did not have any recent trauma. She is a nonsmoker and takes levonorgestrel as her oral contraceptive. On examination, she had normal femoral and popliteal pulses bilaterally, without palpable pedal pulses. Her legs were cold and painful bilaterally. Her right leg was tense and tender in the anterior compartment, associated with an insensate right foot. Her cardiac examination was normal, with no additional heart sounds or murmurs. Her abdominal examination was also unremarkable. An emergent computed tomography angiogram (CTA) confirmed bilateral popliteal occlusions, as well as bilateral segmental renal infarcts (Fig 1). It did not demonstrate any atheroma or thrombus in the distal thoracic aorta and abdominal aorta that could be the embolic source. It did not show features of anatomic popliteal entrapment. The patient was diagnosed with acute limb ischemia and compartment syndrome. She was started on an intravenous heparin infusion and was taken to the operating room urgently. Bilateral popliteal embolectomies were performed through medial infrageniculate approaches to the From the Department of Vascular Surgery,a and the Pathology Queensland,b Princess Alexandra Hospital. Author conflict of interest: none. Correspondence: Kelvin Kam Fai Ho, MBBS/BSc, Department of Vascular Surgery, Princess Alexandra Hospital, PO Box 8101, Woolloongabba, QLD 4102, Australia (e-mail: [email protected]). The editors and reviewers of this article have no relevant financial relationships to disclose per the JVS policy that requires reviewers to decline review of any manuscript for which they may have a conflict of interest. 0741-5214 Copyright Ó 2019 by the Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved. https://doi.org/10.1016/j.jvs.2019.08.264
popliteal arteries. A single compartment fasciotomy was performed for the anterior compartment as well. The embolus consisting of an abundance of firm paleyellow gelatinous material was removed from popliteal and tibial arteries bilaterally using Fogarty catheters (Fig 2). The arteries were closed and the fasciotomy wound was left open for delayed closure. On completion of her embolectomy, the patient had palpable pedal pulses bilaterally. Her postoperative recovery was unremarkable. Delayed closure of her fasciotomy wound was performed as planned. Given the findings on CTA, a cardioembolic source was thought to be likely. Therefore, a transesophageal echocardiogram (TEE) was performed during the operation, which showed normal size and function of her left and right ventricles, no valvular abnormalities, and no intracardiac defects or intracardiac masses. Histopathology of the emboli from both popliteal embolectomies showed features consistent with cardiac myxoma with lepidic cells seen in corded arrangement (Fig 3). Further investigation of the cause of the thromboembolic disease was performed in the subsequent days. Her thrombophilia serology and genetic sequencing was negative. Likewise, a second CTA did not show any disease in her thoracic aorta. Further investigation with cardiac magnetic resonance imaging (MRI) did not identify any focal mass lesion or any definite filling defect, suggesting complete embolization of a cardiac myxoma. The patient was started on rivaroxaban because the original site of attachment of the cardiac myxoma was thought to predispose to further thromboembolic disease. The patient was discharged on postoperative day 7 and returned 10 days later for a planned repeat TEE, which was normal again. She continues to attend follow-up clinic appointments with a vascular surgeon and her anticoagulation will be continued until her next surveillance echocardiogram in 12 months. Written informed consent has been given by the patient for the publication of the case details and images. 1
2
Ho et al
Journal of Vascular Surgery ---
2019
Fig 2. Fragments of pale yellow emboli from bilateral popliteal embolectomies, later shown to be cardiac myxomas histologically.
Fig 1. Computed tomography angiography (CTA) scan with three-dimensional reconstruction showing abrupt cutoff in the above-knee popliteal artery and below-knee popliteal artery of the right and left legs, respectively.
DISCUSSION Cardiac myxomas are the most common benign intracardiac tumors in adults, accounting for one-half of all cases.1 Myxomas can originate from any chamber within the heart. However, the attachment to the left atrium, typically along the interatrial septum, account for the vast majority of cases.1,2 They are most common in the third to sixth decades of life, with a slight female predominance.1 Surgical removal is the treatment of choice. Although this is usually curative, rare recurrence after excision have been reported.1,2 Clinical presentations of cardiac myxomas are highly variable. Roughly 20% of myxomas remain asymptomatic and are found incidentally on imaging.2 Typical presentations of cardiac myxomas include cardiac obstruction, constitutional symptoms such as fever and fatigue, and embolization.1,2 Myxomatous embolism affects the cerebral circulation in most cases; however, any vascular bed can be affected. Embolism causing stroke, limb ischemia, visceral ischemia, and even myocardial infarcts have been reported.1-3 Complete detachments of cardiac myxomas are rare and typically result in aortoiliac embolism.4-10 Early literature has described fatal saddle embolisms of the aortic bifurcation from complete dislodgement of cardiac
myxomas. In these instances, examination of the left atrium during autopsy demonstrated a pedicle, residual thrombus or a rough surface on the endocardium which are thought to be the original attachment of the myxomas.4-7 In the rare survivable cases of complete myxoma embolization, cardiac angiography, echocardiogram, or MRI have been used to exclude residual intracardiac tumour.8-10 Among early reports of myxoma embolization, Carter et al8 described a case of bilateral acute limb ischemia that was treated with aortic embolectomy. Myxomatous tissue was seen in the retrieved embolus. Postoperative angiocardiography was performed and demonstrated no intra-atrial tumor mass. No further investigations were performed given that the patient was in “good health.” Fang et al9 reported a case of infrarenal aortic occlusion with total dislodgement of a cardiac myxoma. A transthoracic echocardiogram and TEE performed preoperatively and repeated examination up to 1 year postoperatively could not identify any residual intracardiac tumor. Coley et al10 reported a case of acute limb ischemia caused by complete detachment of an atrial myxoma. Myxomatous tissue was retrieved from the bilateral iliac and femoropopliteal arteries. Postoperative TEE and MRI demonstrated a residual irregularity on the interatrial septum, which was thought to be the original site of attachment of the embolized myxoma. In our current case, the cardiac myxoma was completely embolized and did not leave any identifiable impression of its original attachment despite multimodality imaging. However, we have commenced anticoagulation with rivaroxaban for our patient given previous autopsy studies have demonstrated residual abnormalities to the endothelium after complete detachment of myxomas. Among patients who underwent surgical removal of myxomas,
Journal of Vascular Surgery Volume
-,
Number
Ho et al
3
-
Fig 3. Histopathology of a popliteal embolus with hematoxylin and eosin stain showing typical features of cardiac myxoma with papillary projections and an abundant loose myxoid stroma under 50 magnification (A) with cells arranged in strands under 200 magnification (B).
recurrence has been noted even several years later.11 Although there are no reported cases of recurrence after total embolization of myxomas, we have elected to continue long-term surveillance with repeat imaging to monitor for this possibility. Our case highlights the importance of cardiac myxomas as a cause of acute limb ischemia. Although completely embolized myxomas tend to cause aortoiliac occlusion, the present case demonstrates that fragmentation of the tumor and small vessel occlusion can occur as well. Cardiac myxomas should be suspected in young and previously healthy patients who present with acute limb ischemia. A normal echocardiogram does not exclude the diagnosis and histologic examination of the embolus is mandatory in such cases. In cases where embolism from a cardiac myxoma is suspected as the cause of acute limb ischemia, a high index of suspicion for embolism into other vascular beds is warranted.
REFERENCES 1. Reynen K. Cardiac myxomas. N Engl J Med 1995;333:1610-7. 2. Wang JG, Li YJ, Liu H, Li NN, Zhao J, Xing X. Clinicopathologic analysis of cardiac myxomas: Seven years’ experience with 61 patients. J Thorac Dis 2012;4:272-83. 3. Vogel B, Thomas D, Mereles D, Rottbauer W, Katus HA. Systemic embolization and myocardial infarction due to
4.
5.
6.
7.
8. 9.
10.
11.
clinically unrecognized left atrial myxoma. Case Rep Med 2011;2011:159024. Silverman J, Olwin JS, Graettinger JS. Cardiac myxomas with systemic embolization. Review of the literature and report of a case. Circulation 1962;26:99-103. Ohgo T, Yamamoto K, Furuno T. Complete detachment of cardiac myxoma causing aortic saddle embolization and cerebral infarction. Int J Cardiol 2008;127:e48-9. Kulkarni M, Jessiman IM, French S. Entire left atrial myxoma presenting as a saddle embolus. Thorax 1969;24: 629-31. Eriksen UH, Baandrup U, Jensen BS. Total disruption of left atrial myxoma causing a cerebral attack and a saddle embolus in the iliac bifurcation. Int J Cardiol 1992;35:127-9. Carter AB, Lowe KG, Hill IG. Cardiac myxomata and aortic saddle embolism. Br Heart J 1960;22:502-4. Fang BR, Chang CP, Cheng CW, Yang NI, Shieh MC, Lee N. Total detachment of cardiac myxoma causing saddle embolization and mimicking aortic dissection. Jpn Heart J 2004;45:359-63. Coley C, Lee KR, Steiner M, Thompson CS. Complete embolization of a left atrial myxoma resulting in acute lower extremity ischemia. Tex Heart Inst J 2005;32:238-40. Pinede L, Duhaut P, Loire R. Clinical presentation of left atrial cardiac myxoma. A series of 112 consecutive cases. Medicine (Baltimore) 2001;80:159-72.
Submitted May 1, 2019; accepted Aug 6, 2019.