294
Images for surgeons
Cardiac cavernous haemangioma: diagnosis and treatment Cardiac haemangiomas are exceptionally rare with only a 1–2% incidence in all detected benign heart neoplasms.1 The resectability depends on the tumour’s location and extent of invasion in the cardiac structures. Patients with an unresectable tumour may have a poor prognosis because of ventricular tachycardia, sudden death and local progression.2 We are presenting the successful treatment of a 40-yearold woman with a cavernous haemangioma of the right atrium (RA). A 40-year-old female patient was admitted to our hospital with the diagnosis of a mass in the mediastinum noted during a medical check-up. The patient was asymptomatic. Chest X-ray showed a well-circumscribed shadow in the right para-cardiac field (Fig. 1a). Transthoracic echocardiography (TTE) revealed a mass near the RA and the right ventricule (RV) (Fig. 1b). The left ventricular ejection fraction (LVEF) was 65% with mild tricuspid valve (TV) regurgitation and the mass compressed the RA. Chest computed tomography (CT) showed a mass 5.1 × 5.2 × 8.8 cm in the pericardium and near the anterior wall of the RA and the anterior wall of the RV without invasion of the diaphragm (Fig. 1c). The presence of the RA mass was confirmed by cardiac magnetic resonance imaging (MRI) (Fig. 1d). The patient underwent open heart operation with standard sternotomy. Cardiopulmonary bypass (CPB) was established with bicaval cannulation, caval snaring and arterial return to the ascending aorta. After opening the pericardium, the tumour was located in the anterior wall of the RA along the right-anterior atriovenricular groove (Fig. 2a,b). When the heart had arrested with cold blood cardioplegia, the tumour was resected en bloc with anterior wall of the RA, the second and third part of the right coronary artery (RCA) because of invasion into this part of the coronary artery. The RCA was bypassed with the saphenous vein graft. The deficit of the
anterior wall of the RA was closed with untreated fresh autologous pericardium with 4-0 Prolene suture. The patient could be weaned easily off bypass. Perioperative transoesophageal echocardiography indicated no TV regurgitation. Pathological examination revealed that a mass was a cavernous-type haemangioma. The post-operative course was uneventful and the patient was discharged home on post-operative day 5. At 2 months follow-up, the TTE echo showed no recurrence, LVEF was 60% with normal TV function without regurgitation. Haemangiomas are benign and non-malignant vascular lesions, and occasionally found in internal organs. Cardiac haemangioma may be located in the pericardium, endocardium or myocardium. Despite their vascular origin, haemangiomas do not metastasize or undergo malignant transformation. The symptoms depend on the location of the tumour and the resultant cardiac haemodynamic consequences. Manifestations of the symptomatic patients are arrhythmias, heart failure, embolic episodes, pericardial effusion, cardiac tamponade, myocardial ischaemia and sudden death.3 In our case, the patient was asymptomatic, but the TTE study showed that the RA and RV were compressed by the tumour. These findings in combination with the size and location of the mass were suggested possible haemodynamic consequence. Echocardiography is the imaging method typically used to identify a cardiac mass, but CT and MRI are superior to echocardiography for further characterizing cardiac haemangiomas. MRI is considered the diagnostic tool to differentiate between benign and malignant tumours.4 On MRI, haemangiomas are characterized as a region of increased signal intensity on T1-weighted images, compared with the myocardium, due to slow flowing blood. On the other hand, multi-
Fig. 1. Preoperative imaging assessment: (a) chest X-ray showed a wellcircumscribed shadow in the right paracardiac field (white arrows); (b) transthoracic echocardiography showed a mass near of the right atrium and the right ventricule (black line); (c) chest computed tomography images of the chest showed a mass (axial plan); (d) cardiac magnetic resonance imaging showed a tumor in the anterior mediastinum. A mass compresses the right atrium and the right ventricule (white arrow).
© 2013 Royal Australasian College of Surgeons
Images for surgeons
295
with cardiac haemangioma were described in the literature and their management consists of surgical resection using CPB with or without a cardiac arrest.6–10 Surgical approaches depend on the tumour location. Bicaval cannulation offers the safe conditions and clear surgical fields for complete and successful resection of the haemangioma, which may be invading more than one other cardiac structure. We believe that this approach ensures the better exposure and complete resection of the tumour. Resection should be complete and a margin of the resection site as permitted must be disease-free. In conclusion, it should be noted that the preoperative accurate assessment of location of the tumour, CPB establishment from beginning of the operation and complete excision of the tumour contribute to acceptable results.
References 1. Verunelli F, Amerini A, D’Alfonso A et al. Left atrial cardiac hemangioma: a report of two cases. Ital. Heart J. 2004; 5: 299–301. 2. Tomizawa Y, Endo M, Nishida H, Kikuchi C, Koyanagi H. Reconstruction of the left ventricle in a patient with cardiac hemangioma at the apex. Ann. Thorac. Surg. 2001; 71: 2032–4. 3. Patel J, Sheppard MN. Sudden death owing to right atrial hemangioma. J. Forensic Sci. 2011; 56: 529–30. 4. Oshima H, Hara M, Kono T, Shibamoto Y, Mishima A, Akita S. Cardiac hemangioma of the left atrial appendage: CT and MR findings. J. Thorac. Imaging 2003; 18: 204–6. 5. Zerbo S, Argo A, Maresi E, Liotta R, Procaccianti P. Sudden death in adolescence caused by cardiac hemangioma. J. Forensic. Leg. Med. 2009; 16: 156–8. 6. Chen X, Lodge AJ, Dibernardo LR, Milano CA. Surgical treatment of a cavernous haemangioma of the heart. Eur. J. Cardiothorac Surg. 2012; 41: 1182–3. 7. De Filippo M, Corradi D, Nicolini F et al. Hemangioma of the right atrium: imaging and pathology. Cardiovasc. Pathol. 2010; 19: 121–4. 8. Botha J, Ihlberg L, Elhenawy A et al. A giant cavernous hemangioma of the heart. Ann. Thorac. Surg. 2010; 90: 293–5. 9. Sata N, Moriyama Y, Hamada N, Horinouchi T, Miyahara K. Recurrent pericardial tamponade from atrial hemangioma. Ann. Thorac. Surg. 2004; 78: 1472–5. 10. González López MT, Aranda Granados PJ, Delange Segura L, Gutiérrez de Loma J. An unexpected left atrial cavernous hemangioma: the cardiac surgeon needs an optimal preoperative study! Interact. Cardiovasc. Thorac. Surg. 2011; 13: 529–31. Fig. 2. Intraoperative views: (a) tumour invasive in the anterior wall of the right atrium (black arrow); (b) tumour located along in the right-anterior atrioventricular groove (black arrow).
detector computed tomography is now being widely applied for diagnosing cardiovascular anomalies and cardiac tumour, but may be inferior to MRI in exact evaluation of the type of tumour. When a clinical diagnosis of cardiac haemangioma has been made, resection should be performed because of the high risk of sudden death from myocardial ischaemia and cardiac tamponade as a consequence of tumour rupture in the pericardium.5 Several cases
© 2013 Royal Australasian College of Surgeons
George Samanidis,* MD Mazen Khoury,* MD, PhD Marina Balanika,† MD Stergios Dimitriou,* MD Alkiviadis Michalis,* MD, FACS *2nd Department of Adult Cardiac Surgery, Onassis Cardiac Surgery Center, Athens, Greece and †Department of Anesthesiology, Onassis Cardiac Surgery Center, Athens, Greece doi: 10.1111/ans.12256
Images for surgeons
Cardiac cavernous haemangioma: diagnosis and treatment Cardiac haemangiomas are exceptionally rare with only a 1–2% incidence in all detected benign heart neoplasms.1 The resectability depends on the tumour’s location and extent of invasion in the cardiac structures. Patients with an unresectable tumour may have a poor prognosis because of ventricular tachycardia, sudden death and local progression.2 We are presenting the successful treatment of a 40-yearold woman with a cavernous haemangioma of the right atrium (RA). A 40-year-old female patient was admitted to our hospital with the diagnosis of a mass in the mediastinum noted during a medical check-up. The patient was asymptomatic. Chest X-ray showed a well-circumscribed shadow in the right para-cardiac field (Fig. 1a). Transthoracic echocardiography (TTE) revealed a mass near the RA and the right ventricule (RV) (Fig. 1b). The left ventricular ejection fraction (LVEF) was 65% with mild tricuspid valve (TV) regurgitation and the mass compressed the RA. Chest computed tomography (CT) showed a mass 5.1 × 5.2 × 8.8 cm in the pericardium and near the anterior wall of the RA and the anterior wall of the RV without invasion of the diaphragm (Fig. 1c). The presence of the RA mass was confirmed by cardiac magnetic resonance imaging (MRI) (Fig. 1d). The patient underwent open heart operation with standard sternotomy. Cardiopulmonary bypass (CPB) was established with bicaval cannulation, caval snaring and arterial return to the ascending aorta. After opening the pericardium, the tumour was located in the anterior wall of the RA along the right-anterior atriovenricular groove (Fig. 2a,b). When the heart had arrested with cold blood cardioplegia, the tumour was resected en bloc with anterior wall of the RA, the second and third part of the right coronary artery (RCA) because of invasion into this part of the coronary artery. The RCA was bypassed with the saphenous vein graft. The deficit of the
anterior wall of the RA was closed with untreated fresh autologous pericardium with 4-0 Prolene suture. The patient could be weaned easily off bypass. Perioperative transoesophageal echocardiography indicated no TV regurgitation. Pathological examination revealed that a mass was a cavernous-type haemangioma. The post-operative course was uneventful and the patient was discharged home on post-operative day 5. At 2 months follow-up, the TTE echo showed no recurrence, LVEF was 60% with normal TV function without regurgitation. Haemangiomas are benign and non-malignant vascular lesions, and occasionally found in internal organs. Cardiac haemangioma may be located in the pericardium, endocardium or myocardium. Despite their vascular origin, haemangiomas do not metastasize or undergo malignant transformation. The symptoms depend on the location of the tumour and the resultant cardiac haemodynamic consequences. Manifestations of the symptomatic patients are arrhythmias, heart failure, embolic episodes, pericardial effusion, cardiac tamponade, myocardial ischaemia and sudden death.3 In our case, the patient was asymptomatic, but the TTE study showed that the RA and RV were compressed by the tumour. These findings in combination with the size and location of the mass were suggested possible haemodynamic consequence. Echocardiography is the imaging method typically used to identify a cardiac mass, but CT and MRI are superior to echocardiography for further characterizing cardiac haemangiomas. MRI is considered the diagnostic tool to differentiate between benign and malignant tumours.4 On MRI, haemangiomas are characterized as a region of increased signal intensity on T1-weighted images, compared with the myocardium, due to slow flowing blood. On the other hand, multi-
Fig. 1. Preoperative imaging assessment: (a) chest X-ray showed a wellcircumscribed shadow in the right paracardiac field (white arrows); (b) transthoracic echocardiography showed a mass near of the right atrium and the right ventricule (black line); (c) chest computed tomography images of the chest showed a mass (axial plan); (d) cardiac magnetic resonance imaging showed a tumor in the anterior mediastinum. A mass compresses the right atrium and the right ventricule (white arrow).
© 2013 Royal Australasian College of Surgeons
Images for surgeons
295
with cardiac haemangioma were described in the literature and their management consists of surgical resection using CPB with or without a cardiac arrest.6–10 Surgical approaches depend on the tumour location. Bicaval cannulation offers the safe conditions and clear surgical fields for complete and successful resection of the haemangioma, which may be invading more than one other cardiac structure. We believe that this approach ensures the better exposure and complete resection of the tumour. Resection should be complete and a margin of the resection site as permitted must be disease-free. In conclusion, it should be noted that the preoperative accurate assessment of location of the tumour, CPB establishment from beginning of the operation and complete excision of the tumour contribute to acceptable results.
References 1. Verunelli F, Amerini A, D’Alfonso A et al. Left atrial cardiac hemangioma: a report of two cases. Ital. Heart J. 2004; 5: 299–301. 2. Tomizawa Y, Endo M, Nishida H, Kikuchi C, Koyanagi H. Reconstruction of the left ventricle in a patient with cardiac hemangioma at the apex. Ann. Thorac. Surg. 2001; 71: 2032–4. 3. Patel J, Sheppard MN. Sudden death owing to right atrial hemangioma. J. Forensic Sci. 2011; 56: 529–30. 4. Oshima H, Hara M, Kono T, Shibamoto Y, Mishima A, Akita S. Cardiac hemangioma of the left atrial appendage: CT and MR findings. J. Thorac. Imaging 2003; 18: 204–6. 5. Zerbo S, Argo A, Maresi E, Liotta R, Procaccianti P. Sudden death in adolescence caused by cardiac hemangioma. J. Forensic. Leg. Med. 2009; 16: 156–8. 6. Chen X, Lodge AJ, Dibernardo LR, Milano CA. Surgical treatment of a cavernous haemangioma of the heart. Eur. J. Cardiothorac Surg. 2012; 41: 1182–3. 7. De Filippo M, Corradi D, Nicolini F et al. Hemangioma of the right atrium: imaging and pathology. Cardiovasc. Pathol. 2010; 19: 121–4. 8. Botha J, Ihlberg L, Elhenawy A et al. A giant cavernous hemangioma of the heart. Ann. Thorac. Surg. 2010; 90: 293–5. 9. Sata N, Moriyama Y, Hamada N, Horinouchi T, Miyahara K. Recurrent pericardial tamponade from atrial hemangioma. Ann. Thorac. Surg. 2004; 78: 1472–5. 10. González López MT, Aranda Granados PJ, Delange Segura L, Gutiérrez de Loma J. An unexpected left atrial cavernous hemangioma: the cardiac surgeon needs an optimal preoperative study! Interact. Cardiovasc. Thorac. Surg. 2011; 13: 529–31. Fig. 2. Intraoperative views: (a) tumour invasive in the anterior wall of the right atrium (black arrow); (b) tumour located along in the right-anterior atrioventricular groove (black arrow).
detector computed tomography is now being widely applied for diagnosing cardiovascular anomalies and cardiac tumour, but may be inferior to MRI in exact evaluation of the type of tumour. When a clinical diagnosis of cardiac haemangioma has been made, resection should be performed because of the high risk of sudden death from myocardial ischaemia and cardiac tamponade as a consequence of tumour rupture in the pericardium.5 Several cases
© 2013 Royal Australasian College of Surgeons
George Samanidis,* MD Mazen Khoury,* MD, PhD Marina Balanika,† MD Stergios Dimitriou,* MD Alkiviadis Michalis,* MD, FACS *2nd Department of Adult Cardiac Surgery, Onassis Cardiac Surgery Center, Athens, Greece and †Department of Anesthesiology, Onassis Cardiac Surgery Center, Athens, Greece doi: 10.1111/ans.12256
