Cardiac Angiosarcoma Presenting With Right Coronary Artery Pseudoaneurysm Mark F. Berry, MD,* Matthew Williams, MD,* Ian Welsby, MD,† and Shu Lin, MD*
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NGIOSARCOMA OF THE HEART, although rare, is the most common primary malignant cardiac tumor.1 Prognosis is poor regardless of treatment, although survival is longest when surgical resection is possible.2,3 Diagnosis is usually delayed because this tumor is typically associated with a variety of nonspecific symptoms.3,4 The authors describe a patient with metastatic cardiac angiosarcoma whose presentation before diagnosis included a pericardial hematoma as a result of a coronary artery pseudoaneurysm. CASE REPORT A 31-year-old man went to an emergency room with substernal chest pain radiating to the right. The patient’s history had been unremarkable until 9 months before this presentation when he developed intermittent chest pain and dyspnea with a syncopal episode. At that time, the patient underwent a subxyphoid pericardial window for drainage of a bloody pericardial effusion. Pathology, cytology, and cultures from the pericardial fluid were all negative, and a follow-up echocardiogram performed because of mild recurrent chest pain after the window did not show any abnormalities. The mild recurrent chest pain after the window was attributed to gastroesophageal reflux disease, although medical treatment did not change his symptoms. The patient had not had any other constitutional complaints such as weight loss or fatigue before presenting to the emergency room. A complete blood count and basic metabolic panel at the time of presenting to the emergency room did not show any significant abnormalities, whereas a computerized tomographic angiogram of the chest performed to evaluate the patient’s pain revealed a 9-cm mass compressing the right atrium (Fig 1A). The patient was transferred urgently to a tertiary care hospital for further management. On arrival from the outside hospital, the patient had a blood pressure of 74/48 mmHg and a heart rate of 130 beats/min, was peripherally cool, and was poorly perfused with a metabolic acidosis (pH ⫽ 7.31, pCO2 ⫽ 34 mmHg, bicarbonate ⫽ 17 mmol/L, base excess ⫽ ⫺8, and lactate ⫽ 6.2 mmol/L). The patient was brought immediately to the operating room after a transthoracic echocardiogram performed at the bedside showed a large mass impinging on the right atrium and right ventricle and obstructing right ventricular filling. A right radial 20-G arterial catheter and a right internal jugular central venous pressure catheter were placed after 1 mg of intravenous midazolam was administered with the patient receiving oxygen at 15 L/min via a nonrebreathing facemask. The patient had marked jugular venous distension, and the central venous pressure catheter was placed while the patient was maintained at a 45° angle to the horizontal. The patient during this time was tachycardic with a heart rate of 125 beats/min in sinus rhythm, with systolic blood pressure ranging from 90 to 110 mmHg with more than 15-mmHg respiratory variation consistent with pericardial tamponade. Anesthesia was carefully induced with 50 mg of ketamine infused into the central catheter over 2 minutes while the patient was breathing 2% sevoflurane in 100% oxygen. The patient received 0.5 mg of phenylephrine over the period of induction to maintain a systolic blood pressure above 100 mmHg. Once it was established that positive-pressure ventilation by mask was tolerated with only moderate hemodynamic compromise, succinylcholine was used to facilitate endotracheal intubation, limiting the inspired pressure to less than 15 cmH2O and avoiding positive end-expiration pressure. A median sternotomy was performed. An intraoperative transesophageal echocardiogram confirmed the presence of the mass alongside the right heart (Fig 1B). The patient was found to have significant adhesions throughout his pericardium, with a large hematoma that, probably
because of the pericardial adhesions, was isolated to the right side of the pericardium with perforation into the right pleural cavity. The reason for perforation into the pleural space was not clear, although it could have been through the site of the previous pericardial window. This hematoma was evacuated, including all clots external to the heart from the level of the superior vena cava to the inferior vena cava. The patient had required an additional 0.5 mg of phenylephrine before sternotomy, but significant hemodynamic improvement did not occur until compression of the right atrium was relieved by release of the hematoma from the pericardium. Cultures of the clot were negative. Pathologic examination of samples of the clot taken from multiple areas, including from within the right pleural space, within the pericardium, and near where the pseudoaneurysm was found, showed a combination of thrombus, fibrinous debris, and inflammatory debris. Further intraoperative evaluation showed a large amount of clot near the right coronary artery in the atrioventricular groove, with distinct bleeding from rupture of the right coronary artery just adjacent to an acute marginal branch (Fig 1C). No other pseudoaneurysms were observed. The pseudoaneurysm was repaired primarily; however, significant narrowing at the site of repair was noted and bypass grafting was therefore planned. The patient was placed on cardiopulmonary bypass via cannulation of the ascending aorta and the right atrial appendage. Heparinization was achieved by using standard doses of 300 to 400 U/kg and activated coagulation time monitoring to maintain the activated coagulation time greater than 480 seconds. A saphenous vein graft from the aorta to the distal right coronary artery was performed. A 10-g load of epsilon aminocaproic acid was followed by an infusion of 1 g/h until drainage from the thoracostomy and mediastinal drains was minimal. The patient recovered well and was discharged home on postoperative day 5 but developed increasing shortness of breath 3 months later. A computed tomography scan of the chest showed a mass adjacent to the right heart similar in location to the hematoma found on the previous presentation as well as innumerable tiny pulmonary nodules, bilateral adrenal nodules, and several low-attenuation liver lesions (Fig 2A). A cardiac magnetic resonance imaging study showed an 11 ⫻ 9 ⫻ 7 cm soft-tissue mass pressing on the right atrium, superior vena cava, inferior vena cava, and right pulmonary veins (Fig 2B). The presumed diagnosis of nonresectable and metastatic cardiac angiosarcoma was made based on the computed tomography scan findings and confirmed by wedge biopsy of several nodules from the left lung. Four cycles of chemotherapy with ifosfamide, mesna, and doxorubicin resulted in complete resolution of the pulmonary metastases and radiographic decrease in the right-heart mass followed by courses of paclitaxel and bevacizumab. Subsequent brain metastases were treated with steroids, whole-brain radiation, and radiosurgery, with 3 additional cycles of chemotherapy given for progression of the cardiac mass and new hepatic lesions, with good radiographic response. The patient is alive 16 months after his presentation with the coronary pseudoaneurysm.
From the *Division of Cardiothoracic Surgery, Department of Surgery, and †Department of Anesthesia, Duke University Medical Center, Durham, NC. Address reprint requests to Mark F. Berry, MD, Division of Cardiothoracic Surgery, Duke University Medical Center, Box 31095, Durham, NC 27710. E-mail:
[email protected] © 2010 Elsevier Inc. All rights reserved. 1053-0770/2404-0016$36.00/0 doi:10.1053/j.jvca.2009.04.002 Key words: sarcoma, aneurysm, pericardium
Journal of Cardiothoracic and Vascular Anesthesia, Vol 24, No 4 (August), 2010: pp 633-635
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Fig 1. (A) A computed tomographic angiogram showing a 9-cm mass (white arrow) compressing the right atrium. RA, right atrium; RV, right ventricle; LVOT, left ventricular outflow tract; LA, left atrium. (B) A transesophageal echocardiogram showing a mass alongside the right heart. PC, pericardium; RA, right atrium; RV, right ventricle; LVOT, left ventricular outflow tract. (C) Pseudoaneurysm of the right coronary artery just adjacent to an acute marginal branch (black arrow). (Color version of figure is available online.)
DISCUSSION
Primary tumors of the heart are uncommon and most commonly benign.1 Angiosarcoma is the most common primary malignant cardiac tumor.1,2,3,5 Angiosarcoma of the heart can occur in any cardiac chamber but involves the right atrium most commonly.1-3 Cardiac angiosarcoma growth can be rapid, although growth within the myocardial wall can be difficult to detect with imaging such as echocardiography, computed tomography scans, and magnetic resonance imaging. Optimal treatment includes complete surgical resection, which has a median survival of only 17 to 24 months.1,2,3,5 Unfortunately,
cardiac angiosarcomas often cannot be completely resected or are metastatic upon presentation and must be treated nonsurgically, which has a median survival of only 6 to 7 months.4,5 Although there does not appear to be an accepted standard chemotherapy regimen for this uncommon cancer, most regimens discussed in the literature include doxirubicin alone or in combination with other agents.3,5,6 The young patient presented in this report has been treated aggressively with multimodal therapies directed at both his primary tumor as well as metastatic disease and has had a survival thus far of 16 months, which is longer than the reported median survival for this stage of disease.
Fig 2. (A) A computed tomography scan of the chest showing an intermediate attenuation structure (black arrow) adjacent to the right heart, with displacement of the heart to the left and loss of the fat plane between the structure and the right heart border as well as innumerable tiny pulmonary nodules (white arrows). (B) A cardiac magnetic resonance imaging study showing an 11 ⴛ 9 ⴛ 7 cm soft-tissue mass (white arrow) pressing on the right atrium, superior vena cava, inferior vena cava, and right pulmonary veins.
CARDIAC ANGIOSARCOMA
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One reason for advanced stage upon diagnosis is that the presentation of cardiac angiosarcoma generally includes a variety of nonspecific symptoms.3,4 The most common initial presenting symptom is dyspnea on exertion followed by chest pain.3 Patients present less commonly with systemic symptoms such as weight loss or fatigue.7 Patients can also present with embolic events or arrhythmia.3,7 Cardiac angiosarcoma can have a tendency to bleed spontaneously and can also present with a new or recurrent pericardial effusion, with or without signs of tamponade.3,8,9 Before the diagnosis of cardiac angiosarcoma was made in this report, the patient had syncope because of a bloody pericardial effusion and then chest pain along with a pericardial hematoma and a coronary artery pseudoaneurysm. Pathology was benign in both instances with no etiology identified. Cardiac angiosarcoma should be considered when unexplained pericardial blood is found, and close follow-up with echocardiogram or other imaging should be considered in these cases, especially when symptoms persist. This may allow tumor detection either when still completely resectable surgically or when metastatic disease is limited so that aggressive therapy can be used to potentially prolong shortterm survival, as was shown in this case report.2,6 The presentation of cardiac angiosarcoma with coronary artery pseudoaneurysm has been reported once previously.10
Coronary artery pseudoaneurysms are rare and are mostly caused by dissection or perforation after infection, trauma, or catheter intervention.11-15 The most common symptoms associated with pseudoaneurysms are those of myocardial ischemia.16 Although pseudoaneurysms can close spontaneously, untreated pseudoaneurysms have a risk of thrombosis with distal embolization or rapid enlargement and rupture leading to cardiac tamponade.16 Options for treatment include resection with bypass grafting, coil embolization, and stenting.16 The pseudoaneurysm discovered in this case could have been a direct result of the angiosarcoma, although it also could have been secondary to the presence of pericardial blood from angiosarcomainduced bleeding or even trauma during the previous pericardial window procedure. In summary, a 31-year-old man with a previous history of a bloody pericardial effusion of unknown etiology who required emergent operative exploration because of hypotension caused by a large hematoma compressing the right heart and a right coronary artery pseudoaneurysm was presented. Despite pathologic examination of the hematoma showing benign findings, the patient ultimately developed a recurrent right-sided cardiac mass and was found to have metastatic angiosarcoma 3 months later. The patient has survived 16 additional months with multiple treatments with chemotherapy.
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