Ann Thorac Surg 1999;68:1074-1075
© 1999 The Society of Thoracic Surgeons
Case Reports
Resection of a large right atrial hemangioma in a neonate after prenatal diagnosis
Friedrich S. Eckstein, MDa,
Markus K. Heinemann, MDa,
Gunther J. Mielke, MDb,
Annette Greschniok, MDc,
Peter Bader, MDd,
Gerhard Ziemer, MDa
a Division of Thoracic, Cardiac, and Vascular Surgery, University of Tübingen, Tübingen, Germany
b Department of Obstetrics and Gynecology, University of Tübingen, Tübingen, Germany
c Department of Pathology, University of Tübingen, Tübingen, Germany
d Department of Pediatric Cardiology, University of Tübingen, Tübingen, Germany
Address reprint requests to Dr Eckstein, Division of Thoracic, Cardiac, and Vascular Surgery, University of Tübingen, Hoppe-Seyler-Str 3, D-72076 Tübingen, Germany
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Abstract
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Cardiac hemangiomas are extremely rare benign tumors. We describe the case of a right atrial hemangioma in a neonate diagnosed prenatally and successfully operated 10 days after spontaneous delivery. This unusual case illustrates the importance of prenatal diagnosis and delivery of the baby next to a pediatric center with a department of appropriately specialized cardiovascular surgery.
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Introduction
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Primary cardiac tumors of the heart are rare, their incidence ranging from 0.0017% to 0.28% in clinical postmortem series [1]. A total of 2.8% of primary cardiac tumors are hemangiomas [2], with very few having been reported in children. We describe the successful surgical removal of a symptomatic right atrial hemangioma in a neonate diagnosed prenatally.
Routine prenatal ultrasound (34th gestation week) in a 36-year-old woman during her first pregnancy suggested a fetal cardiac anomaly. Two months before the expected delivery date, ultrasound diagnosis confirmed a cardiac mass in the right atrium without hemodynamic relevance. Fetal heart rate variability tests were undertaken to evaluate the possibility of spontaneous delivery. As no pathological changes could be detected, spontaneous delivery at 40 ± 2 weeks gestational age took place in our hospital. The baby girl weighed 2,895 g. APGAR scores were 9 at 1 minute and 10 at 5 and 10 minutes. No abnormal heart sounds could be detected; the electrocardiogram showed sinus rhythm. The patient was transferred to the pediatric cardiology intensive care unit. Echocardiography and magnetic resonance imaging were performed to evaluate the intracardiac mass. Both revealed a large cardiac tumor (2.5 cm) arising from the right atrial appendage (Fig 1). There was an additional secundum atrial septal defect. As there was no apparent urgency for treatment, the operation was performed on the 10th day of life after normalization of the neonatal liver function. After median sternotomy and opening of the pericardium, a well-vascularized tumor could be seen instead of the right auricle of the heart (Fig 2). Pericardiotomy alone, however, led to a drop of central venous pressure (CVP) and heart rate, and a rise in arterial blood pressure. Therefore, in retrospect, a slight cardiac tamponade had been present. Extracorporeal circulation was installed, and in deep hypothermia with short circulatory arrest, the right atrium was opened and the mass inspected. Arising from the auricle, the capsular tumor reached from the orifice of the superior vena cava down to the margin of the tricuspid valve, externally being attached to the atrioventricular groove adjacent to the right coronary artery. The atrial septal defect was closed with a direct suture. After deairing of the heart, the operation was continued in moderate hypothermia (30°C) with the heart beating. The tumor was carefully dissected off the right coronary artery by clipping multiple small feeding vessels. Caution was taken not to damage the region of the sinus node. After excision of the tumor, the cavity of the right atrium was reconstructed with a patch of autologous pericardium. The girl was weaned off bypass and was extubated on the first postoperative day. There was a persistent sinus rhythm. By echocardiography, a competent tricuspid valve and unrestricted blood flow in the right atrium could be seen.
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Fig 2. Intraoperative view before installation of the extracorporeal circulation showing the tumor (large arrow), right coronary artery (small arrow) with right ventricle, and the ascending aorta (double arrows).
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Fig 3. Cross section of the excised hemangioma with blood vessels (large arrow) and central areas of necrosis (small arrow), partially surrounded by cardiac muscle fibers (white arrow).
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The histopathological examination revealed a benign tumor composed of blood vessels of capillary and cavernous type with areas of necrosis, hemorrhage, fibrosis, and calcifications (Fig 3). Immunohistochemically, the endothelial cells of the vascular channels and some cells of immature cell nests were immunoreactive for anti-CD34 (QBEND 10). Mitotic figures were present in moderate number and corresponded to the slightly elevated immunoreactivity for MiB1, a marker of proliferative activity. The tumor could therefore be classified as a benign cardial hemangioma of capillary-cavernous type.
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Comment
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Cardiac hemangioma in infancy is an extremely rare benign tumor. Hemangiomas derive from blood vessels and can be capillary and/or cavernous. The first clinical diagnosis of a cardiac hemangioma is often only made after serious complications, including congestive heart failure, pericardial effusion, or cardiac arrhythmias. In unresectable cardiac hemangiomas, two therapeutic options have been published so far. Yoshikawa and associates [3] cured a patient with radiotherapy, and Chang and associates [4] treated a patient successfully with steroids. The first successful resection of a hemangioma involving the myocardium was published by Hochberg and Robinson in 1950 [5]. Reported locations of the right atrial hemangiomas were similar to our case, with close contiguity to the right coronary artery and neighborhood of the sinus node [6, 7]. Benign cardiac tumors such as a hemangioma in childhood have an excellent prognosis when completely excised, and appear to have a good short-term prognosis even when excision is incomplete [8]. Diagnosis in the prenatal period can be advantageous. The pregnant woman should be transferred to a pediatric center with a department of cardiovascular surgery specialized in treatment of congenital heart disease. The possibility of spontaneous delivery should be examined. After birth, surgical resection of large tumors in the neonatal period is advised to prevent the onset of adverse sequelae.
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References
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Novitzky D., Rose A.G., Morgan J.A., Barnard C.N. Primary cardiac hemangiomas. SA Med J 1984;66:267-270.
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Chang J.S., Young M.L., Chuu W.M., Lue H.C. Infantile cardiac hemangioendothelioma. Pediatr Cardiol 1992;13:52-55.[Medline]
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Hochberg L., Robinson A. Primary tumor of the pericardium involving the myocardium. Circulation 1950;1:805-808.
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Reece I.J., Cooley D.A., Frazier O.H., Hallman G.L., Powers P.L., Montero C.G. Cardiac tumors. Clinical spectrum and prognosis of lesions other than classical benign myxoma in 20 patients. J Thorac Cardiovasc Surg 1984;88:439-446.[Abstract]
Accepted for publication February 13, 1999.
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Abstract
Introduction
