Downloaded from heart.bmj.com on June 9, 2014 - Published by group.bmj.com
Heart Online First, published on May 22, 2014 as 10.1136/heartjnl-2014-305865 Image challenge
An unusual cause of a non-ST segment elevation myocardial infarction CLINICAL INTRODUCTION A 66-year-old man presented with dull, left-sided chest pain for 1 week occurring mainly at rest. The symptoms had been present for 1 month but had increased in frequency over the preceding 3 days. He became acutely short of breath with substernal chest pain while climbing a flight of stairs the day of admission. The pain was pressure-like, did not radiate and was associated with presyncope, but not syncope, diaphoresis, nausea nor vomiting. He had no past medical history of cardiac disease but did have risk factors for coronary artery disease (CAD) including hypertension, history of smoking and the metabolic syndrome. His past medical history was also significant for non-Hodgkin’s stage IV marginal zone B cell lymphoma treated with rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone (R-CHOP) 4 years earlier. Vital signs and physical exam were normal without murmurs, rubs or gallops. The lung fields were clear, there was no jugular venous distention nor lower extremity oedema. A 12-lead ECG showed normal sinus rhythm without ST segment elevations and his serum troponin I was elevated to 1.69 ng/mL. He was treated medically for a non-ST segment elevation myocardial infarction and underwent non-emergent coronary angiography for recurrent chest pain. Figure 1 shows injection of right coronary artery (RCA) in left anterior oblique orientation (LAO) at the time of coronary angiography (figure 1).
Figure 1 Still frame of injection of right coronary artery in left anterior oblique orientation at the time of coronary angiography.
QUESTION Cardiac angiographic image is most consistent with the following: A. Pulmonary arterial-venous malformation with fistula to the RCA. B. Significant stenosis of the mid portion of the RCA. C. Tumour blush due to vascular tumour with blood supply derived from the RCA. D. Fistula between the RCA and right atrium (RA).
Levit RD, et al. Heart Month 2014 Vol 0 employer) 2014. Produced by BMJ Publishing Group Ltd (& BCS) under licence. 1 Copyright Article author (or0 No their
Downloaded from heart.bmj.com on June 9, 2014 - Published by group.bmj.com
Image challenge 2
ANSWER: C Angiogram of the RCA showed tumour blush in the RA (figure 2A), not the pulmonary artery nor lung, as expected in a pulmonary artery fistula. RCA-RA fistula would opacify the entire atrium instead of a discrete area. There is no RCA stenosis and the left coronary artery had non-obstructive disease. Cardiac MRI and transesophageal echo confirmed a soft tissue mass within the RA (figure 2B,C). The mass was surgically resected (figure 2D) and showed high-grade angiosarcoma (figure 2E). The patient underwent chemotherapy, surviving 8 months. Rarely, tumours are identified by coronary angiography when contrast highlights tumour vasculature. Angiographers should maintain a high index of suspicion for non-coronary pathology at the time of cardiac catheterisation. Large or obstructing tumours can cause heart failure from flow congestion through cardiac chambers and valves. Infiltration of the myocardium can cause necrosis, arrhythmias or steal flow from the coronary arteries. In this patient, chest pain and shortness of breath was related to necrosis and reduced flow in the RCA causing RV dysfunction and troponin elevation. Primary cardiac malignancies are rare with a prevalence of 0.001–0.03%.1 Fifty per cent of patients present with metastasis.2 3 Angiosarcomas are the most common primary cardiac malignancy.2 With surgical resection and chemotherapy, 5-year survival is less than 10%.2 4 1
2
2
Division of Cardiology, Department of Medicine, Emory University School of Medicine, Atlanta, Georgia, USA
Correspondence to Dr Gautam Kumar, Cardiology Division (111C/D), Atlanta Veterans Affairs Medical Center, 1670 Clairmont Road, Decatur, GA 30033, USA; [email protected] Contributors All authors had a highly significant contribution to this work. RDL drafted the article and collected images. MP, JDV and DRM collected additional surgical and pathological images and contributed significantly to manuscript revisions. GK conceptualised the format, contributed significantly to revisions and obtained clinical images. Competing interests None. Provenance and peer review Not commissioned; internally peer reviewed. To cite Levit RD, Polomsky M, Vega JD, et al. Heart Published Online First: [ please include Day Month Year] doi:10.1136/heartjnl-2014-305865 Received 22 March 2014 Accepted 21 April 2014 Heart 2014;0:1–2. doi:10.1136/heartjnl-2014-305865
REFERENCES 1 2
3
Rebecca D Levit, Marek Polomsky, J David Vega, David R Martin, Gautam Kumar1,4 1
Division of Cardiothoracic Surgery, Department of Surgery, Emory University School of Medicine, Atlanta, Georgia, USA 3 Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Atlanta, Georgia, USA 4 Division of Cardiology, Atlanta Veterans Affairs Medical Center, Decatur, Georgia, USA
3 4
Grebenc ML, Rosado de Christenson ML, Burke AP, et al. Primary cardiac and pericardial neoplasms: radiologic-pathologic correlation. Radiographics 2000;20:1073–103. Fatima J, Duncan AA, Maleszewski JJ, et al. Primary angiosarcoma of the aorta, great vessels, and the heart. J Vasc Surg 2013;57:756–64. Orlandi A, Ferlosio A, Roselli M, et al. Cardiac sarcomas: an update. J Thorac Oncol 2010;5:1483–9. Blackmon SH, Patel A, Reardon MJ. Management of primary cardiac sarcomas. Expert Rev Cardiovasc Ther 2008;6:1217–22.
Figure 2 Multimodality imaging, surgical view and histopathological appearance of an intracardiac angiosarcoma. Left anterior oblique view of right coronary artery at time of cardiac catheterisation revealed tumour blush (arrow) in vicinity of right atrium (A). Tumour was localised to the floor of the right atrium and appendage by T2-weighted, precontrast cardiac MRI (B, arrow) and by contrast-enhanced transesophageal echocardiography (C, arrow). Following median sternotomy and cannulation, a 3.0×2.5×1.5 cm angiosarcoma was seen arising from the right atrial appendage (D) and resected with bovine pericardium atrial reconstruction. H&E stain of the surgical specimen (E) showed high-grade angiosarcoma with spindle cell morphology, focal vasoformative architecture and mitotically active nuclei intermixed with background cardiomyocytes (20×). 2
Levit RD, et al. Heart Month 2014 Vol 0 No 0
Downloaded from heart.bmj.com on June 9, 2014 - Published by group.bmj.com
An unusual cause of a non-ST segment elevation myocardial infarction Rebecca D Levit, Marek Polomsky, J David Vega, et al. Heart published online May 22, 2014
doi: 10.1136/heartjnl-2014-305865
Updated information and services can be found at: http://heart.bmj.com/content/early/2014/05/22/heartjnl-2014-305865.full.html
These include:
References
This article cites 4 articles http://heart.bmj.com/content/early/2014/05/22/heartjnl-2014-305865.full.html#ref-list-1
P
Heart Online First, published on May 22, 2014 as 10.1136/heartjnl-2014-305865 Image challenge
An unusual cause of a non-ST segment elevation myocardial infarction CLINICAL INTRODUCTION A 66-year-old man presented with dull, left-sided chest pain for 1 week occurring mainly at rest. The symptoms had been present for 1 month but had increased in frequency over the preceding 3 days. He became acutely short of breath with substernal chest pain while climbing a flight of stairs the day of admission. The pain was pressure-like, did not radiate and was associated with presyncope, but not syncope, diaphoresis, nausea nor vomiting. He had no past medical history of cardiac disease but did have risk factors for coronary artery disease (CAD) including hypertension, history of smoking and the metabolic syndrome. His past medical history was also significant for non-Hodgkin’s stage IV marginal zone B cell lymphoma treated with rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone (R-CHOP) 4 years earlier. Vital signs and physical exam were normal without murmurs, rubs or gallops. The lung fields were clear, there was no jugular venous distention nor lower extremity oedema. A 12-lead ECG showed normal sinus rhythm without ST segment elevations and his serum troponin I was elevated to 1.69 ng/mL. He was treated medically for a non-ST segment elevation myocardial infarction and underwent non-emergent coronary angiography for recurrent chest pain. Figure 1 shows injection of right coronary artery (RCA) in left anterior oblique orientation (LAO) at the time of coronary angiography (figure 1).
Figure 1 Still frame of injection of right coronary artery in left anterior oblique orientation at the time of coronary angiography.
QUESTION Cardiac angiographic image is most consistent with the following: A. Pulmonary arterial-venous malformation with fistula to the RCA. B. Significant stenosis of the mid portion of the RCA. C. Tumour blush due to vascular tumour with blood supply derived from the RCA. D. Fistula between the RCA and right atrium (RA).
Levit RD, et al. Heart Month 2014 Vol 0 employer) 2014. Produced by BMJ Publishing Group Ltd (& BCS) under licence. 1 Copyright Article author (or0 No their
Downloaded from heart.bmj.com on June 9, 2014 - Published by group.bmj.com
Image challenge 2
ANSWER: C Angiogram of the RCA showed tumour blush in the RA (figure 2A), not the pulmonary artery nor lung, as expected in a pulmonary artery fistula. RCA-RA fistula would opacify the entire atrium instead of a discrete area. There is no RCA stenosis and the left coronary artery had non-obstructive disease. Cardiac MRI and transesophageal echo confirmed a soft tissue mass within the RA (figure 2B,C). The mass was surgically resected (figure 2D) and showed high-grade angiosarcoma (figure 2E). The patient underwent chemotherapy, surviving 8 months. Rarely, tumours are identified by coronary angiography when contrast highlights tumour vasculature. Angiographers should maintain a high index of suspicion for non-coronary pathology at the time of cardiac catheterisation. Large or obstructing tumours can cause heart failure from flow congestion through cardiac chambers and valves. Infiltration of the myocardium can cause necrosis, arrhythmias or steal flow from the coronary arteries. In this patient, chest pain and shortness of breath was related to necrosis and reduced flow in the RCA causing RV dysfunction and troponin elevation. Primary cardiac malignancies are rare with a prevalence of 0.001–0.03%.1 Fifty per cent of patients present with metastasis.2 3 Angiosarcomas are the most common primary cardiac malignancy.2 With surgical resection and chemotherapy, 5-year survival is less than 10%.2 4 1
2
2
Division of Cardiology, Department of Medicine, Emory University School of Medicine, Atlanta, Georgia, USA
Correspondence to Dr Gautam Kumar, Cardiology Division (111C/D), Atlanta Veterans Affairs Medical Center, 1670 Clairmont Road, Decatur, GA 30033, USA; [email protected] Contributors All authors had a highly significant contribution to this work. RDL drafted the article and collected images. MP, JDV and DRM collected additional surgical and pathological images and contributed significantly to manuscript revisions. GK conceptualised the format, contributed significantly to revisions and obtained clinical images. Competing interests None. Provenance and peer review Not commissioned; internally peer reviewed. To cite Levit RD, Polomsky M, Vega JD, et al. Heart Published Online First: [ please include Day Month Year] doi:10.1136/heartjnl-2014-305865 Received 22 March 2014 Accepted 21 April 2014 Heart 2014;0:1–2. doi:10.1136/heartjnl-2014-305865
REFERENCES 1 2
3
Rebecca D Levit, Marek Polomsky, J David Vega, David R Martin, Gautam Kumar1,4 1
Division of Cardiothoracic Surgery, Department of Surgery, Emory University School of Medicine, Atlanta, Georgia, USA 3 Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Atlanta, Georgia, USA 4 Division of Cardiology, Atlanta Veterans Affairs Medical Center, Decatur, Georgia, USA
3 4
Grebenc ML, Rosado de Christenson ML, Burke AP, et al. Primary cardiac and pericardial neoplasms: radiologic-pathologic correlation. Radiographics 2000;20:1073–103. Fatima J, Duncan AA, Maleszewski JJ, et al. Primary angiosarcoma of the aorta, great vessels, and the heart. J Vasc Surg 2013;57:756–64. Orlandi A, Ferlosio A, Roselli M, et al. Cardiac sarcomas: an update. J Thorac Oncol 2010;5:1483–9. Blackmon SH, Patel A, Reardon MJ. Management of primary cardiac sarcomas. Expert Rev Cardiovasc Ther 2008;6:1217–22.
Figure 2 Multimodality imaging, surgical view and histopathological appearance of an intracardiac angiosarcoma. Left anterior oblique view of right coronary artery at time of cardiac catheterisation revealed tumour blush (arrow) in vicinity of right atrium (A). Tumour was localised to the floor of the right atrium and appendage by T2-weighted, precontrast cardiac MRI (B, arrow) and by contrast-enhanced transesophageal echocardiography (C, arrow). Following median sternotomy and cannulation, a 3.0×2.5×1.5 cm angiosarcoma was seen arising from the right atrial appendage (D) and resected with bovine pericardium atrial reconstruction. H&E stain of the surgical specimen (E) showed high-grade angiosarcoma with spindle cell morphology, focal vasoformative architecture and mitotically active nuclei intermixed with background cardiomyocytes (20×). 2
Levit RD, et al. Heart Month 2014 Vol 0 No 0
Downloaded from heart.bmj.com on June 9, 2014 - Published by group.bmj.com
An unusual cause of a non-ST segment elevation myocardial infarction Rebecca D Levit, Marek Polomsky, J David Vega, et al. Heart published online May 22, 2014
doi: 10.1136/heartjnl-2014-305865
Updated information and services can be found at: http://heart.bmj.com/content/early/2014/05/22/heartjnl-2014-305865.full.html
These include:
References
This article cites 4 articles http://heart.bmj.com/content/early/2014/05/22/heartjnl-2014-305865.full.html#ref-list-1
P
