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References 1. Kim CY, Kim W, Kwak JG, et al. Surgical management of left ventricular outflow tract obstruction after biventricular repair of double outlet right ventricle. J Korean Med Sci 2010;25: 374–9. 2. Russell HM, Johnson SL, Katherine C, Wurlitzer C, Backer CL. Outcomes of surgical therapy of infective endocarditis in a pediatric population: a 21-year review. Ann Thorac Surg 2013;96:171–5. 3. Tissieres P, Jaeggi ET, Beghetti M, Gervaix A. Increase of fungal endocarditis in children. Infection 2005;33:267–72. 4. Ferrieri P, Gewitz MH, Gerber MA, et al. Unique features of infective endocarditis in childhood. Circulation 2002;105: 2115–26. 5. Koolbergen DR, Manshanden JS, Yazdanbakhsh AP, et al. Reoperation for neoaortic root pathology after the arterial switch operation. Eur J Cardiothorac Surg 2014 Feb 23; [E-Pub ahead of print]. 6. Mavroudis C, Stewart RD, Backer CL, Rudra H, Vargo P, Jacobs ML. Reoperative techniques for complications after arterial switch. Ann Thorac Surg 2011;92:1747–55.
Takuya Kawabata, MD, PhD, Shingo Kasahara, MD, PhD, Shin-ichi Ohtsuki, MD, PhD, Yosuke Kuroko, MD, PhD, Yasuhiro Kotani, MD, PhD, Yasuhiro Fujii, MD, PhD, Ko Yoshizumi, MD, PhD, Sadahiko Arai, MD, PhD, and Shunji Sano, MD, PhD Department of Cardiovascular Surgery, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences; and Department of Pediatric Cardiology of Okayama University Hospital, Okayama, Japan
Myxoma is the most common primary cardiac tumor in adults; however, it is extremely rare in infants. Acute occlusion of the abdominal aorta by a cardiac myxoma is also rare. We report the case of an infant with acute occlusion of the suprarenal abdominal aorta by a left ventricular myxoma. The patient underwent successful catheter embolectomy of the abdominal aorta and surgical resection of the cardiac myxoma. This is a very rare case report of the combination of infantile left ventricular myxoma and acute occlusion of the abdominal aorta. (Ann Thorac Surg 2015;100:309–11) Ó 2015 by The Society of Thoracic Surgeons
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ardiac myxoma is the most common cardiac tumor in adults [1]. However, it is rare in children, especially in infants [2]. Acute occlusion of the abdominal aorta by a cardiac myxoma is also rare [1]. Here we describe an infant with acute occlusion of the suprarenal abdominal
Accepted for publication Aug 29, 2014. Address correspondence to Dr Kawabata, Department of Cardiovascular Surgery, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, 2-5-1 Shikata, Okayama, 700-8558 Japan; e-mail:
[email protected].
Ó 2015 by The Society of Thoracic Surgeons Published by Elsevier
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aorta by a left ventricular (LV) myxoma who underwent successful catheter embolectomy of the abdominal aorta and surgical resection of the myxoma. A 6-month-old girl presented to a referring hospital with vomiting and hematochezia. She had been doing well before the onset, but a heart murmur had been identified 3 days prior for which further study had been planned. At the referring hospital, she had been diagnosed with intussusception of the intestine. Initial therapy, however, made her condition worse, with progression of her anemia, acidosis, and anuria. The following day, contrastenhanced computed tomography (CT) showed total embolic occlusion of the suprarenal abdominal aorta (Fig 1A) and an abnormal LV shadow defect (Fig 1B). Bowel ischemia due to a cardiac tumor embolism or thromboembolism of the abdominal aorta was highly suspected. She was transferred to our hospital for further therapy. Emergency embolectomy of the abdominal aorta by a bilateral femoral approach was successfully performed by pediatric cardiologists. After the procedure she underwent embolectomies of the bilateral femoral arteries because of multiple emboli in the lower legs. The embolectomies, although successful, resulted in compartment syndrome of both legs, requiring emergency fasciotomies. She also required continuous hemofiltration for 7 days after the procedures because of acute renal failure due to acute rhabdomyolysis. Further studies of the LV tumor by ultrasound echocardiography and cardiac magnetic resonance imaging showed a solid LV tumor originating from a ventricular septum (Fig 1C). Head CT and magnetic resonance imaging showed no cerebral infarction. Results of pathologic studies of the embolectomy specimens were inconclusive. She underwent LV tumor resection under cardiopulmonary bypass and mild hypothermia (34 C) 22 days after the onset the tumor embolism. The LV tumor was visualized by a transseptal approach and was resected en bloc. It was a gelatinous, sessile ovoid and had a gently lobulated surface (Fig 2A). No tumor was detected in the other cardiac chambers. Cardiopulmonary bypass time and cardiac arrest time were 76 and 62 minutes, respectively. The patient’s postoperative course was uneventful. She was discharged from the hospital on postoperative day 40 with mild contracture of her right ankle. Pathologic study confirmed the diagnosis of cardiac myxoma (Fig 2B). She has been followed for 6 months and has shown no signs of recurrence.
Comment Primary cardiac tumors in the pediatric age group are rare, with a reported incidence of 0.2% in children referred for cardiac disease [3]. The most frequently occurring tumors in children are rhabdomyomas, followed by fibromas [3]. The relative frequency of myxoma in pediatric heart tumors is less than 5%, and most are identified in older children [4]. Although some children with familial syndrome myxoma have been reported [5] there was no specific family history in the present case. 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2014.08.066
FEATURE ARTICLES
Left Ventricular Myxoma Occluding the Suprarenal Abdominal Aorta in an Infant
CASE REPORT KAWABATA ET AL LV MYXOMA IN AN INFANT
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CASE REPORT KAWABATA ET AL LV MYXOMA IN AN INFANT
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FEATURE ARTICLES
Fig 1. Images of contrast-enhanced computed tomographic scan performed at the referring hospital and an image of the tumor in the left ventricle by transthoracic echocardiography. (A) A suprarenal abdominal aortic embolism measuring 16.4 9.4 mm is detected (arrow). (B) An abnormal shadow defect is identified in the left ventricle (arrow). (C) Transthoracic echocardiography shows a solid left ventricle tumor on the ventricular septum in four-chamber view (arrow). The tumor has no stalk. No obstruction of the mitral valve or the outflow tract of the left ventricle is detected. The tumor measures 16.2 10.6 mm. (Ao ¼ aorta; IVC ¼ inferior vena cava; LA ¼ left atrium; LV ¼ left ventricle; RA ¼ right atrium; RK ¼ right kidney; RV ¼ right ventricle.)
The most common and next most common sites of cardiac myxoma are the left and right atria, with incidences of 75% and 15% to 20%, respectively [1]. Only 3% to 4% of myxomas occur in the LV and only 3% to 4% in the right ventricle [1]. Of the 26 primary cardiac tumors resected in our unit over the past 20 years, no myxoma in an infant and no LV myxoma has occurred other than those in the present case. Congestive heart failure, embolism, and constitutional symptoms comprise the clinical triad of cardiac myxoma [1]. In small children, congestive heart failure has frequently been reported because of the rapid growth of a tumor mass in small-sized cavities [3]. Although our patient had no signs or symptoms of heart failure, a cardiac murmur had been identified 3 days before the onset but had disappeared before presenting to our hospital, suggesting that the cardiac murmur was produced by the large myxoma with intracardiac obstruction before embolism. Fig 2. Pathologic findings. (A) Gross features of the excised left ventricular myxoma. It is a gelatinous, sessile, and ovoid with a gently lobulated surface and measures 25 15 8 mm. (B) Highpower microscopic appearance (40) with hematoxylin and eosin staining. Stellate or spindle cells are seen surrounded by myxoid stroma. Mitosis, pleomorphism, and necrosis are absent. (HE ¼ hematoxylin and eosin.)
Embolism has been reported to occur in 30% to 40% of all patients with myxoma [1]. The incidence of embolism is higher in papillary than in solid myxoma [6]. The emboli obstruct the cerebral arteries in approximately half of embolism cases [6]. Embolism of the peripheral, coronary, and visceral arteries by small fragments has also been reported [6]. Although rare, acute occlusion of the abdominal aorta or saddle emboli by large fragments of cardiac myxoma has been observed in adult cases, with high mortality and morbidity [1]. With regard to tumor location, an LV tumor is also considered to be a risk factor for systemic embolism, which occurs in nearly 65% of cases [7]. In the present case, despite its being a sessile myxoma with a low incidence of embolism [6], it may have cracked under the high mobility of the LV septum and high pressure in the left ventricle, and a large fragment may have embolized the abdominal aorta. Management of acute occlusion of the abdominal aorta includes catheter embolectomy, open embolectomy, and
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extra-anatomic bypass [8]. For open embolectomy, median laparotomy or thoraco-retroperitoneal approach with clamping the supraceliac aorta would be needed because the embolic site was suprarenal abdominal aorta. Aggressive open surgical approach requiring abdominal organ ischemia might not be ideal considering her poor clinical status. Hence, a catheter approach was chosen with a satisfactory result. We experienced an extremely rare case of an infant with acute occlusion of the suprarenal abdominal aorta by an LV myxoma. Using a staged approach, the embolus and the LV tumor were successfully resected.
References
Thrombus in the Proximal Aorta: Cardiopulmonary Bypass Strategy and Surgical Management Rachit Saxena, MCh, Manikala Vinod Kumar, MS, Sanjeev Kumar, MD, Parag Gharde, DM, Sachin Talwar, MCh, and Shiv Kumar Choudhary, MCh Cardiothoracic Centre, All India Institute of Medical Sciences, New Delhi, India
De novo noninfective thrombus formation in the ascending aorta is rare. We report two cases of ascending aortic thrombus, their surgical management, and cardiopulmonary bypass strategy. (Ann Thorac Surg 2015;100:311–3) Ó 2015 by The Society of Thoracic Surgeons
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scending aorta is one of the rarest sites for origin of noninfective thrombus. We report two cases of thrombus in the ascending aorta. The thrombus Accepted for publication July 21, 2014. Address correspondence to Dr Choudhary, Department of Cardiothoracic and Vascular Surgery, All India Institute of Medical Sciences, New Delhi110029, India; e-mail:
[email protected].
Ó 2015 by The Society of Thoracic Surgeons Published by Elsevier
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originated from normal aortic wall and was the only identifiable source of peripheral embolism. Both patients underwent emergency surgical removal of the pedunculated aortic thrombus under mild hypothermic (32 C) cardiopulmonary bypass. We describe the strategy to excise the thrombus safely, which was extending across the aortic arch in both the cases without the need of deep hypothermic circulatory arrest.
Case Reports Patient 1 A 35-year-old male, nondiabetic, nonhypertensive, smoker presented with effort-induced right upper extremity pain for 2 months. Right upper limb pulsations were absent, and the results of the cardiovascular examination was normal. Duplex ultrasonic examination showed thrombus in the right subclavian artery and the axillary artery, with monophasic low-velocity flow in the right brachial artery. Transthoracic echocardiography revealed mobile pedunculated thrombus in the ascending aorta arising from the right coronary sinus and extending into the arch. There was no evidence of any intracardiac clot or any valvular abnormality. Left ventricular contractility was normal, and cardiac chambers and aorta were of normal dimensions. Computed tomographic angiography confirmed the presence of a curvilinear thrombus attached to the right coronary sinus arising just above the right coronary ostium without obstructing the coronary ostium and freely floating in the ascending aorta, extending into the arch of aorta up to the level of origin of left common carotid artery. There was another thrombus in the proximal portion of the right subclavian artery extending into the right axillary artery (Fig 1). The rest of the aorta, arch vessels, and upper extremity vessels were unremarkable. The right subclavian artery occlusion was responsible for the present symptoms of this patient. The hematologic investigations, including lipid profile, hematocrit, platelet count, protein C, protein S, homocysteine level, and antiphospholipid antibody, were all within the normal range. The patient underwent emergency surgery to prevent additional embolic events. Intraoperative monitoring included invasive arterial pressure monitoring in the right radial and left femoral arteries, central venous line through the left femoral vein, and neuromonitoring by near infrared spectroscopy (NIRS).The right axillary and subclavian artery thrombus was removed through a right infraclavicular incision. Considering the possibility of more thrombus at the origin of subclavian artery, it was decided to use the right common carotid artery for arterial inflow during cardiopulmonary bypass. The right common carotid artery was exposed by a separate right cervical incision (parallel to the right sternocleidomastoid muscle) for arterial cannulation. After systemic heparinization, an 8-mm Dacron tube graft was anastomosed end to side to the right common carotid artery. The arterial end of the cardiopulmonary bypass circuit was bifurcated using a Y connector, one 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2014.07.078
FEATURE ARTICLES
1. Klaus Reynen MD. Cardiac myxomas. N Engl J Med 1995;333: 1610–7. 2. Burke A, Virmani R. Pediatric heart tumors. Cardiovasc Pathol 2008;17:193–8. 3. Padalino MA, Basso C, Milanesi O, et al. Surgically treated primary cardiac tumors in early infancy and childhood. J Thorac Cardiovasc Surg 2005;129:1358–63. 4. Isaacs H Jr. Fetal and neonatal cardiac tumors. Pediatr Cardiol 2004;25:252–73. 5. Maraj S, Pressman GS, Figueredo VM. Primary cardiac tumors. Int J Cardiol 2009;133:152–6. 6. Garatti A, Nano G, Canziani A, et al. Surgical excision of cardiac myxomas: twenty years experience at a single institution. Ann Thorac Surg 2012;93:825–31. 7. Kapoora A, Radhakrishnan S, Sinha N. Unusual presentation during childhood of left ventricular myxoma. Cardiol Young 1998;8:126–7. 8. Crawford JD, Perrone KH, Wong VW, et al. A modern series of acute aortic occlusion. J Vasc Surg 2014;59: 1044–50.
CASE REPORT SAXENA ET AL THROMBUS IN PROXIMAL AORTA