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Pulmonary venous drainage through a highly vascularized left atrial tumor
International Journal of Cardiology 116 (2007) e76 – e77 www.elsevier.com/locate/ijcard
Letter to the Editor
Pulmonary venous drainage through a highly vascularized left atrial tumor Ronen Rubinshtein a,⁎, Dan Aravot b , Moshe Y. Flugelman a , David A. Halon a , Boris Orlov b , Inna Naroditsky c , Basil S. Lewis a a
Department of Cardiovascular Medicine, Lady Davis Carmel Medical Center and the Bruce Rappaport Faculty of Medicine, Technion-IIT, Haifa, Israel Department of Cardiothoracic Surgery, Lady Davis Carmel Medical Center and the Bruce Rappaport Faculty of Medicine, Technion-IIT, Haifa, Israel c Department of Pathology, Lady Davis Carmel Medical Center and the Bruce Rappaport Faculty of Medicine, Technion-IIT, Haifa, Israel
b
Received 6 July 2006; accepted 31 July 2006 Available online 7 November 2006
Abstract We present a patient with symptomatic congestive heart failure due to a left atrial sarcoma infiltrating and apparently occluding the left pulmonary veins. The tumor was highly vascularized and enabled attenuated blood drainage from the left upper and lower pulmonary veins despite intra-operative appearance as completely obliterative, thus avoiding persistent left lung pulmonary edema. The tumor was partially removed and the pathologic findings showed advanced angiogenesis. Malignant tumors may occlude large blood vessels and thus may result in the development of a collateral flow. However, we suggest that highly vascularized tumors (macroscopic totally occlusive) may serve as a sponge and enable attenuated blood flow through the tumor and hence may avoid a complete cut off in blood supply or drainage. © 2006 Elsevier Ireland Ltd. All rights reserved. Keywords: Left atrial tumor; Sarcoma; Angiogenesis; Congestive heart failure
1. Patient description An 81-year old hypertensive woman presented with recurrent episodes of pulmonary edema. On admission the patient appeared pale and cachectic. She was dyspneaic, the pulse rate was 76/min and blood pressure was 112/75 mm Hg. Jugular venous pressure was 11 cm above sternal angle. Heart auscultation revealed a grade 2 apical diastolic murmur. Examination of the lungs revealed bilateral, diffuse crackles over the lung bases. Complete blood count showed normochromic normocytic anemia (Hemoglobin level was 10.6 g/ dL). ECG showed normal sinus rhythm. The chest X-ray showed a normal cardiac silhouette and bilateral pulmonary congestion. The patient was treated successfully with diuretics and underwent transthoracic and transesophageal echocardiography demonstrating a large left atrial mass interfering with the opening of the anterior mitral valve leaflet without ⁎ Corresponding author. Department of Cardiovascular Medicine, Lady Davis Carmel Medical Center, 7 Michal Street, Haifa 34362, Israel. Tel.: +972 4 8250288; fax: +972 4 8250119. E-mail address: [email protected] (R. Rubinshtein). 0167-5273/$ - see front matter © 2006 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ijcard.2006.07.232
definite significant infiltration into the left upper and lower pulmonary veins. Doppler ultrasonography of the left pulmonary veins suggested residual blood flow. Coronary angiography showed normal coronary arteries, an A-V fistula arising from the left main coronary artery and draining to the left atrium and a vascularized mass in the left atrium (Fig. 1). CT angiography showed reduced left pulmonary venous drainage due to the presence of large tumor at the left atrium. Due to severe symptomatic heart failure and lack of clear-cut demonstration of pulmonary venous obstruction, surgery was performed. However, the solid tumor completely obliterated the left pulmonary veins and only partial debulcking was possible. Pathological examination identified the tumor as undifferentiated sarcoma and anti-CD 31 immunohistochemistry (specific marker for vascular endothelium) demonstrated highly angiogenic tumor (Fig. 2). Recovery from surgery was uneventful. Post-operative left and right heart catheterization revealed residual pressure gradient (mean = 15 mm Hg) between the left pulmonary capillary wedge and left ventricular diastolic pressures (LVDP), due to residual growth obstructing the left pulmonary veins and a mean gradient of 9 mm Hg between the right pulmonary capillary wedge and
R. Rubinshtein et al. / International Journal of Cardiology 116 (2007) e76–e77
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LVDP due to partial obstruction of the mitral valve by residual growth in the left atrium. One month post-operatively local recurrence and tumor proliferation within the left atrium were identified by echocardiography. The patient died of heart failure three months following surgery. 2. Discussion Cardiac tumors may interfere with blood flow or valve function, and cause distal embolization, metastatic involvement of adjacent organs or cardiac tamponade. Constitutional syndromes are common [1]. The sarcomas are the most common primary malignant tumors of the heart and are usually associated with poor prognosis [1]. Left atrial sarcomas frequently interfere with mitral valve opening and may cause functional mitral stenosis [2]. It has been suggested that tumor histology may identify those patients in whom intervention may improve prognosis but only few patients may benefit from an aggressive surgical approach [3,4]. Despite the operative findings in our patient of obstruction of the left pulmonary venous drainage and probable invasion of the left upper and lower pulmonary veins, the absence of single lung pulmonary edema and the CT (and invasive) angiographic findings as well as of the Doppler echocardiography suggested residual pulmonary venous drainage from the left lung into the left atrium. We considered possible explanations for this unusual phenomenon of complete obliteration of pulmonary veins accompanied by (partially) effective drainage of the left lung. Although increased collateral flow through the left bronchial vessels may
Fig. 2. Highly angiogenic undifferentiated sarcoma of the left atrium as demonstrated by anti-CD31 staining for vascular endothelium.
have contributed to left lung blood drainage, we suggest, based on CT and coronary angiography and tumor histology, a mechanism of drainage via highly vascularized tumor that serves as sponge which transfers blood from the higher-pressure compartment (pulmonary veins) to the lower-pressure compartment (left atrium). Highly vascular tumors in which vascular endothelial growth factor is the angiogenic trigger are typical of some cardiac tumors such as myxoma [5]. In summary, this patient presentation and unusual findings suggest a novel mechanism of pulmonary drainage in an obliterating tumor. Based on the vascularity of the tumor itself acting as a drainage sponge this possibility should be kept in mind and accounted for the absence of unilateral pulmonary edema that usually characterizes such patients. References [1] Roberts WC. Cardiac neoplasms. In: Topol EJ, editor. Textbook of Cardiovascular Medicine. 2nd edition. Philadelphia USA: Lippincot Williams & Wilkins publishers; 2002. p. 917–33. [2] Domanski MJ, Delaney TF, Kleiner Jr DE, et al. Primary sarcoma of the heart causing mitral stenosis. Am J Cardiol 1990;66:893–5. [3] Burke AP, Cowan D, Virmani R. Primary sarcomas of the heart. Cancer 1992;69:387–95. [4] Talbot SM, Taub RN, Keohan ML, Edwards N, Galantowicz ME, Schulman LL. Combined heart and lung transplantation for unresectable primary cardiac sarcoma. J Thorac Cardiovasc Surg 2002;124:1145–8. [5] Kono T, Koide N, Hama Y, et al. Expression of vascular endothelial growth factor and angiogenesis in cardiac myxoma: a study of fifteen patients. J Thorac Cardiovasc Surg 2000;119:101–7.
Fig. 1. Highly vascularized left atrial tumor (white arrows) and attenuated drainage of an AV fistula from the left main coronary artery into left atrium (yellow arrows) as shown by coronary angiography. (For interpretation of the references to color in this figure legend, the reader is referred to the web version of this article.)
Letter to the Editor
Pulmonary venous drainage through a highly vascularized left atrial tumor Ronen Rubinshtein a,⁎, Dan Aravot b , Moshe Y. Flugelman a , David A. Halon a , Boris Orlov b , Inna Naroditsky c , Basil S. Lewis a a
Department of Cardiovascular Medicine, Lady Davis Carmel Medical Center and the Bruce Rappaport Faculty of Medicine, Technion-IIT, Haifa, Israel Department of Cardiothoracic Surgery, Lady Davis Carmel Medical Center and the Bruce Rappaport Faculty of Medicine, Technion-IIT, Haifa, Israel c Department of Pathology, Lady Davis Carmel Medical Center and the Bruce Rappaport Faculty of Medicine, Technion-IIT, Haifa, Israel
b
Received 6 July 2006; accepted 31 July 2006 Available online 7 November 2006
Abstract We present a patient with symptomatic congestive heart failure due to a left atrial sarcoma infiltrating and apparently occluding the left pulmonary veins. The tumor was highly vascularized and enabled attenuated blood drainage from the left upper and lower pulmonary veins despite intra-operative appearance as completely obliterative, thus avoiding persistent left lung pulmonary edema. The tumor was partially removed and the pathologic findings showed advanced angiogenesis. Malignant tumors may occlude large blood vessels and thus may result in the development of a collateral flow. However, we suggest that highly vascularized tumors (macroscopic totally occlusive) may serve as a sponge and enable attenuated blood flow through the tumor and hence may avoid a complete cut off in blood supply or drainage. © 2006 Elsevier Ireland Ltd. All rights reserved. Keywords: Left atrial tumor; Sarcoma; Angiogenesis; Congestive heart failure
1. Patient description An 81-year old hypertensive woman presented with recurrent episodes of pulmonary edema. On admission the patient appeared pale and cachectic. She was dyspneaic, the pulse rate was 76/min and blood pressure was 112/75 mm Hg. Jugular venous pressure was 11 cm above sternal angle. Heart auscultation revealed a grade 2 apical diastolic murmur. Examination of the lungs revealed bilateral, diffuse crackles over the lung bases. Complete blood count showed normochromic normocytic anemia (Hemoglobin level was 10.6 g/ dL). ECG showed normal sinus rhythm. The chest X-ray showed a normal cardiac silhouette and bilateral pulmonary congestion. The patient was treated successfully with diuretics and underwent transthoracic and transesophageal echocardiography demonstrating a large left atrial mass interfering with the opening of the anterior mitral valve leaflet without ⁎ Corresponding author. Department of Cardiovascular Medicine, Lady Davis Carmel Medical Center, 7 Michal Street, Haifa 34362, Israel. Tel.: +972 4 8250288; fax: +972 4 8250119. E-mail address: [email protected] (R. Rubinshtein). 0167-5273/$ - see front matter © 2006 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ijcard.2006.07.232
definite significant infiltration into the left upper and lower pulmonary veins. Doppler ultrasonography of the left pulmonary veins suggested residual blood flow. Coronary angiography showed normal coronary arteries, an A-V fistula arising from the left main coronary artery and draining to the left atrium and a vascularized mass in the left atrium (Fig. 1). CT angiography showed reduced left pulmonary venous drainage due to the presence of large tumor at the left atrium. Due to severe symptomatic heart failure and lack of clear-cut demonstration of pulmonary venous obstruction, surgery was performed. However, the solid tumor completely obliterated the left pulmonary veins and only partial debulcking was possible. Pathological examination identified the tumor as undifferentiated sarcoma and anti-CD 31 immunohistochemistry (specific marker for vascular endothelium) demonstrated highly angiogenic tumor (Fig. 2). Recovery from surgery was uneventful. Post-operative left and right heart catheterization revealed residual pressure gradient (mean = 15 mm Hg) between the left pulmonary capillary wedge and left ventricular diastolic pressures (LVDP), due to residual growth obstructing the left pulmonary veins and a mean gradient of 9 mm Hg between the right pulmonary capillary wedge and
R. Rubinshtein et al. / International Journal of Cardiology 116 (2007) e76–e77
e77
LVDP due to partial obstruction of the mitral valve by residual growth in the left atrium. One month post-operatively local recurrence and tumor proliferation within the left atrium were identified by echocardiography. The patient died of heart failure three months following surgery. 2. Discussion Cardiac tumors may interfere with blood flow or valve function, and cause distal embolization, metastatic involvement of adjacent organs or cardiac tamponade. Constitutional syndromes are common [1]. The sarcomas are the most common primary malignant tumors of the heart and are usually associated with poor prognosis [1]. Left atrial sarcomas frequently interfere with mitral valve opening and may cause functional mitral stenosis [2]. It has been suggested that tumor histology may identify those patients in whom intervention may improve prognosis but only few patients may benefit from an aggressive surgical approach [3,4]. Despite the operative findings in our patient of obstruction of the left pulmonary venous drainage and probable invasion of the left upper and lower pulmonary veins, the absence of single lung pulmonary edema and the CT (and invasive) angiographic findings as well as of the Doppler echocardiography suggested residual pulmonary venous drainage from the left lung into the left atrium. We considered possible explanations for this unusual phenomenon of complete obliteration of pulmonary veins accompanied by (partially) effective drainage of the left lung. Although increased collateral flow through the left bronchial vessels may
Fig. 2. Highly angiogenic undifferentiated sarcoma of the left atrium as demonstrated by anti-CD31 staining for vascular endothelium.
have contributed to left lung blood drainage, we suggest, based on CT and coronary angiography and tumor histology, a mechanism of drainage via highly vascularized tumor that serves as sponge which transfers blood from the higher-pressure compartment (pulmonary veins) to the lower-pressure compartment (left atrium). Highly vascular tumors in which vascular endothelial growth factor is the angiogenic trigger are typical of some cardiac tumors such as myxoma [5]. In summary, this patient presentation and unusual findings suggest a novel mechanism of pulmonary drainage in an obliterating tumor. Based on the vascularity of the tumor itself acting as a drainage sponge this possibility should be kept in mind and accounted for the absence of unilateral pulmonary edema that usually characterizes such patients. References [1] Roberts WC. Cardiac neoplasms. In: Topol EJ, editor. Textbook of Cardiovascular Medicine. 2nd edition. Philadelphia USA: Lippincot Williams & Wilkins publishers; 2002. p. 917–33. [2] Domanski MJ, Delaney TF, Kleiner Jr DE, et al. Primary sarcoma of the heart causing mitral stenosis. Am J Cardiol 1990;66:893–5. [3] Burke AP, Cowan D, Virmani R. Primary sarcomas of the heart. Cancer 1992;69:387–95. [4] Talbot SM, Taub RN, Keohan ML, Edwards N, Galantowicz ME, Schulman LL. Combined heart and lung transplantation for unresectable primary cardiac sarcoma. J Thorac Cardiovasc Surg 2002;124:1145–8. [5] Kono T, Koide N, Hama Y, et al. Expression of vascular endothelial growth factor and angiogenesis in cardiac myxoma: a study of fifteen patients. J Thorac Cardiovasc Surg 2000;119:101–7.
Fig. 1. Highly vascularized left atrial tumor (white arrows) and attenuated drainage of an AV fistula from the left main coronary artery into left atrium (yellow arrows) as shown by coronary angiography. (For interpretation of the references to color in this figure legend, the reader is referred to the web version of this article.)