Right atrial myxoma originating from the inferior vena cava

Right Atrial Myxoma Originating From the Inferior Vena Cava Uberto Bortolotti, MD, Giuseppe Faggian, MD, Alessandro Mazzucco, MD, Aldo Milano, MD, Gaetano Thiene, MD, Giuseppe Fasoli, MD, and Vincenzo Gallucci, MD Departments of Cardiovascular Surgery, Pathology, and Cardiology, University of Padova Medical School, Padova, Italy. A patient undergoing successful excision of a right atrial myxoma arising from the inferior vena cava is reported. The rarity of this case prompted a review of the literature in which only 2 other patients with a right atrial myxoma originating from inferior vena cava tissue were found. (Ann Thorac Surg 1990;49:1000-2) M yxomas are the most frequent benign intracardiac tumors, most commonly arising from the left atrium [l].Right atrial myxomas are much less common and are usually attached to the interatrial septum [2]. We report here a patient whose unusual feature was the presence of a right atrial myxoma originating from the inferior vena cava (IVC). A 33-year-old woman was admitted to another hospital in November 1988. Approximately 2 months previously she had delivered at term a healthy baby without complications. During the last trimester of gestation, the patient started complaining of fatigue and mild exertional dyspnea; at that time ankle swelling and increased blood pressure were first noted. After delivery, she was observed to have persistent peripheral edema and hypertension suggesting further evaluation. On admission, physical examination showed a blood pressure of 165/115 mm Hg, a pulse rate of 104 beats per minute, and a 2/6 systolic murmur at the lower sternal border; the liver was palpable 4 cm below the right costal margin, and jugular venous distention was evident. The chest roentgenogram showed moderate enlargement of the cardiac shadow, and the electrocardiogram showed sinus tachycardia without major abnormalities. A standard two-dimensional echocardiogram showed the presence of a large mass almost filling the right atrium, extending to the IVC and entering into the right ventricle during diastole (Fig 1); however, the precise site of tumor insertion could not be identified. The patient was transferred to our unit where urgent operation was performed on November 5, 1988. Cardiopulmonary bypass was instituted by individual cannulation of both venae cavae through the right atrium, avoiding any manipulation of the heart. With the patient under Accepted for publication Nov 20, 1989. Address reprint requests to Dr Bortolotti, Istituto di Chirurgia Cardiovascolare, Universita di Padova, Via Giustiniani, 2, 35128 Padova, Italy. 0 1990 by The Society of Thoracic Surgeons moderate systemic hypothermia (28°C) and after cold cardioplegic arrest of the heart, the right atrium was opened, and a large friable mass was noted to originate with a short stalk from inside the suprahepatic segment of the IVC. It became apparent that to get better exposure of the tumor insertion the IVC cannula should be temporarily removed; this was achieved after the nasopharyngeal temperature had been lowered to 25°C. During a brief period of low-flow perfusion, the mass was excised together with the entire pedicle and the underlying endothelium; the low attachment of the tumor prevented, however, a full-thickness excision of the IVC. The IVC cannula was then reinserted, the patient rewarmed, and the operation completed uneventfully. Pathologic examination showed a large, friable multilobulated mass, which at histological examination showed the classic features of a myxoma (Fig 2). Comment Right atrial myxomas usually originate from the fossa ovalis or at the base of the interatrial septum. In such situations, surgical exposure of the tumor is usually easier and its excision simpler when compared with that of a left atrial myxoma [l]. The patient described herein had the unusual finding of a right atrial myxoma arising from the suprahepatic segment of the IVC; this occurrence is quite exceptional and was not mentioned in recent reviews of patients with intracardiac myxomas [l, 31. Right atrial myxomas originating from IVC tissue have been previously reported only twice (Table 1).Devig and colleagues in 1980 [4] described a 28-year-old man who underwent excision of a right atrial myxoma inserted at the junction between the right atrial wall and the anterosuperior aspect of the IVC. The tumor was removed together with a segment of IVC. More recently, Cujec and associates [5] reported a 25-year-old man with a right atrial myxoma originating from the eustachian valve and prolapsed into the IVC causing Budd-Chiari syndrome. The mass was successfully excised, but details of the surgical technique were not mentioned. The peculiar location of a right atrial mass, as in the present case, may pose specific surgical problems. One of these concerns the best technique of venous cannulation to prevent fragmentation and possible pulmonary embolization. To minimize this potential complication, venous return can be accomplished by cannulating the superior 0003-4975/90/$3.50 CASE REPORT BORTOLOTTI ET AL RIGHT ATRIAL MYXOMA Ann Thorac Surg 1990;49:1000-2 1001 Fig 1. Two-dimensional echocardiogram showing a large mass (M) filling the right atrium (RA)(a, b) and protruding into the inferior vena cava (IVC) (c, d ) . (HV = hepatic vein; LA = left atrium;LV = left ventricle; RV = right ventricle.) vena cava and a femoral vein [4].In the present case, we used direct caval cannulation, as previously done in all other patients with a right atrial myxoma [6], because the exact site of tumor insertion was not identified on preoperative echocardiogram. We have found that this technique can be used without complications even in the presence of large intraatrial masses, provided that undue manipulation of the heart is avoided. When the myxoma is inserted deep into the IVC, as in the present case, temporary removal of the IVC cannula is needed to obtain a better exposure of the mass. However, had the precise site of tumor attachment been identified using intraoper- ative transesophageal echocardiography, which was not available for our patient, a different approach by cannulation of a femoral vein would have been probably more appropriate. To avoid recurrences, a radical operation includes removal of the myxoma and its entire base of implantation, which is easily performed when the tumor is inserted into the fossa ovalis or the free atrial wall. In the present case, however, full-thickness excision was prevented by the unusual attachment of the mass into the IVC. Despite the absence of clear signs of local infiltration, periodic noninvasive monitoring of this patient seems mandatory. Table 1. Summa y of Patients With a Right Atrial Myxoma Arising From lnferior Vena Cava Tissue Author Devig et a1 (1980) [41 Cujec et a1 (1987) 151 Bortolotti et a1 (present case) IVC = inferior vena cava; Age (Yr) Sex Symptoms Diagnosis 28 M Cardiac catheter 25 M 33 F Exertional d ysp n ea Budd-Chiari syndrome Exertional dyspnea, peripheral edema RA = right atrium; 2-D = two-dimensional. Intraoperative 2-D echocardiography Location IVC-RA junction Eustachian valve Suprahepatic IVC Surgical Technique Full thickness excision Not mentioned Excision 1002 CASE REPORT BORTOLOTTI ET AL RIGHT ATRIAL MYXOMA Ann Thorac Surg 1990;49:1000-2 We are indebted to Richard Van Praagh, MD, for help during the preparation of the manuscript and to Gian Carlo Pengo for technical assistance. References Fig 2 . Gross appearance of the excised myxoma. 1. Chitwood RW Jr. Cardiac neoplasms: current diagnosis, pathology, and therapy. J Cardiac Surg 1988;3:119-54. 2. St. John Sutton MG, Mercier LA, Giuliani ER, Lie JT. Atrial myxomas. A review of clinical experience in 40 patients. Mayo Clin Proc 1980;55:371-6. 3. Silverman NA. Primary cardiac tumors. Ann Surg 1980; 191:127-38. 4. Devig PM, Clark TA, Aaron BL. Cardiac myxoma arising from the inferior vena cava. Chest 1980;78:784-6. 5. Cujec B, Ulmer B, McKaigney JP, Bharadwaj B. Right atrial myxoma presenting as Budd-Chiari syndrome. Ann Thorac Surg 1987;4465%9. 6. Livi U, Bortolotti U, Milano A, et al. Cardiac myxomas: results of 14 years’ experience. Thorac Cardiovasc Surgeon 1984;32143-7.