Ann Thorac Surg 1996;61:1835-1836
© 1996 The Society of Thoracic Surgeons
Case Report
Surgical Treatment of Right Ventricular Myxoma in Infancy
Daniel Talmor, MD,
Joseph Caspi, MD,
Shai Feuering, MD,
Nili Zuker, MD,
Eli Zalstein, MD,
Amir Ganiel, MD,
Azi Appelbaum, MD
Department of Cardiothoracic Surgery and Pediatric Cardiology Unit, Soroka Medical Center, Ben-Gurion University, Beer-Sheva, Israel
Accepted for publication December 9, 1995.
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Abstract
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Myxomas are the most common of all primary cardiac tumors in adults. They are extremely rare in infancy. We report on a 5-month-old infant who was admitted in a state of cardiogenic shock. Echocardiography showed a right ventricular myxoma completely occluding the right ventricular outflow tract. Prompt surgical removal of the tumor resulted in an excellent outcome.
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Introduction
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Primary cardiac tumors are uncommon. The incidence ranges from a low of 0.0017% (8 in 480,000 autopsies) to a high of 0.33% (154 in 46,072 autopsies) [1]. The most common tumor in adults is myxoma, which is most frequently located in the atria (95%) and rarely in the ventricles. Only a few cases have previously been described in infants less than 1 year of age [2]. We report on a 5-month-old infant with a right ventricular myxoma causing outflow obstruction, cyanosis, and low cardiac output state.
A 5-month-old infant was admitted to the emergency room in a state of extreme cardiorespiratory failure and remarkable cyanosis. Physical examination revealed a respiratory rate of 70 breaths/min, a blood pressure of 45/25 mm Hg, and a pulse rate of 170 beats/min. A 3/6 systolic ejection murmur was audible throughout the precordium. The liver was remarkably enlarged. The arterial blood gases showed severe hypoxemia and acidosis.
On chest roentgenogram there was marked cardiomegaly and signs of pulmonary hypoperfusion. The electrocardiogram showed sinus tachycardia. Two-dimensional Doppler echocardiography (Fig 1
) demonstrated a 3 x 3-cm mass emerging from the right ventricular septal wall and protruding into the ventricular outflow tract. A peak systolic gradient of 74 mm Hg was measured across the pulmonary valve. An additional finding was a 5-mm secundum atrial septal defect with a right-to-left shunt.
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Fig 1. . Preoperative two-dimensional echocardiographic short-axis view showing a tumor occluding the right ventricular outflow tract. (AO = aorta; RA = right atrium; RV = right ventricle.)
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After hemodynamic resuscitation the patient was transferred to the operating room. After a midsternotomy incision was performed, inspection of the heart showed a severely dilated, dark, and hypokinetic right ventricle.
Cardiopulmonary bypass was established using aortic and bicaval cannulas with moderate hypothermia. The right atrium was opened, and through the tricuspid valve a large mass occupying the cavity of the right ventricle was visible. An attempt to resect the tumor through the tricuspid valve failed because of its size, and therefore the right ventricular outflow was obliquely opened and the tumor was completely excised along its pedicle and base, great care being taken not to perforate the interventricular septum. The atrial septal defect was repaired. After rewarming, the patient was weaned off cardiopulmonary bypass without difficulty.
The postoperative course was complicated by right-sided chylothorax, which resolved after drainage and parenteral nutrition for 3 weeks. The tumor was a polypoid mass with a smooth surface covered with fibrin and measured 3.5 x 3 cm. Histologic examination showed spindle cells, small blood vessels, and cells with a myxoid appearance consistent with myxoma. At repeated follow-up visits the patient was asymptomatic. Two-dimensional Doppler echocardiography at the most recent follow-up visit showed no recurrence of tumor and good biventricular function.
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Comment
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Cardiac myxoma is the most common benign tumor in adults but is extremely rare in neonates and infants. It is found predominantly in the atria and infrequently in the ventricle (5%). With the advent of echocardiography in recent years, diagnosis has been greatly improved. The information provided by two-dimensional echocardiography is sufficient indication for operation [3]. Magnetic resonance imaging has recently been recognized as an accurate diagnostic tool in respect to the location and size of the tumor [4].
Symptoms may be variable and determined by the location and size of the tumor. Inflow or outflow obstruction, embolism, syncopal episodes, and arrhythmias are the most frequent [5].
After the diagnosis of acute right ventricular obstruction due to a cardiac mass is established by echocardiography, prompt surgical resection is indicated because of imminent embolization and inflow or outflow occlusion, resulting in sudden death. Several surgical techniques have been suggested but in each case it depends on the site of the tumor. Surgical exposure may be facilitated through the transseptal approach when the myxoma is confined to the left atrium. The surgical resection should include the full thickness of the atrial wall or septum. However, in this particular case, the size and location of the tumor dictated a different surgical approach to avoid any damage to adjacent structures such as triscuspid and pulmonary valves.
This case report highlights the fact that right ventricular outflow obstruction associated with cyanosis and low cardiac output may be related to a cardiac myxoma, despite the low incidence of this tumor in infants. This unusual position of a cardiac tumor and the histologic findings consistent with a myxoma at this age are important features of this presentation. Early diagnosis and surgical resection can achieve successful results.
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Footnotes
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Address reprint requests to Dr Talmor, Cardiothoracic Surgery, Soroka Medical Center, PO Box 151, Beer-Sheva, 84101, Israel.
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References
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- Wold LE, Lie JT. Cardiac myxoma: a clinicopathological profile. Am J Pathol 1980;101:219–40.
- Nadas AS, Ellison RC. Cardiac tumors in infancy. Am J Cardiol 1968;21:363–6.[Medline]
- Donahoo JS, Weiss JL, Gardner TJ. Current management of atrial myxoma with emphasis on a new diagnostic technique. Ann Surg 1979;189:763–8.[Medline]
- Pizarello RA, Goldbergg SM, Goldman MA, et al. Tumors of the heart diagnosed by magnetic resonance imaging. J Am Coll Cardiol 1985;5:989–91.[Abstract]
- Silvermann NA. Primary cardiac tumors. Ann Surg 1980;191:127–38.[Medline]
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Abstract
Introduction
