Ann Thorac Surg 2003;75:1306-1308
© 2003 The Society of Thoracic Surgeons
Case report
Cardiac fibroma in an infant: single ventricle palliation as a bridge to heart transplantation
Benjamin R. Waller, MDa,
Scott M. Bradley, MDb*,
A. Jackson Crumbley, III, MDa,b,c,
Henry B. Wiles, MDa,
Tim C. McQuinn, MDa,
Allan T. Bennett, MDc
b Division of Cardiothoracic Surgery, Charleston, SC, USA
a Division of Pediatric Cardiology, Charleston, SC, USA
c Division of Pathology, Medical University of South Carolina, Charleston, South Carolina, USA
Accepted for publication October 14, 2002.
* Address reprint requests to Dr Bradley, Division of Cardiothoracic Surgery, Medical University of South Carolina, 96 Jonathan Lucas St, Charleston, SC 29425, USA
e-mail: bradlesm{at}musc.edu
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Abstract
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A prenatal echocardiogram revealed a large right ventricular mass. Following birth, there was obstruction to pulmonary blood flow and cyanosis. The tumor’s size and location prevented resection. The patient underwent "single ventricle palliation," including placement of a systemic-to-pulmonary shunt as a newborn. This palliation served as a successful bridge to heart transplantation at 7 months of age. Pathologic examination revealed cardiac fibroma.
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Introduction
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Cardiac fibroma is the second most common cardiac tumor of childhood (after rhabdomyoma). Although benign, fibromas may cause chest pain, arrhythmias, or, if large enough, obstruction to blood flow with congestive heart failure or cyanosis [1, 2]. Symptomatic fibromas have been successfully treated by resection, either partial or complete, or by heart transplantation [1, 3–8]. We present the case of a neonate with an unresectable right ventricular fibroma and cyanosis. He underwent "single ventricle palliation," which served as a successful bridge to heart transplantation.
Fetal echocardiography at 33 weeks’ gestation revealed a large right ventricular mass and a pericardial effusion. Following delivery at 37 weeks, physical examination was notable for slight prominence of the right hemithorax, and a grade 2/6 systolic ejection murmur along the left sternal border. Chest roentgenogram illustrated a large cardiac silhouette extending well into the right chest and obscuring the right lung. Echocardiography revealed a mass occupying most of the right ventricle and atrium. There was right-to-left flow across a small, restrictive patent foramen ovale, and minimal antegrade flow across the right ventricular outflow tract. Pulmonary flow was provided by a patent ductus arteriosus. A large pericardial effusion was removed by pericardiocentesis and prostaglandin E-1 was administered to maintain ductal patency. Cardiac magnetic resonance imaging (MRI) verified a large mass centered in the right ventricle with compression of the right atrium and much of the right lung. Attempts to wean the prostaglandin were unsuccessful due to hypoxia.
As the tumor appeared unresectable, it was decided to carry out "single ventricle palliation," consisting of atrial septostomy and placement of a systemic-to-pulmonary artery shunt. The aim of this palliation was to allow weaning from prostaglandin E-1, and to serve as a possible bridge to heart transplantation. Balloon atrial septostomy was successfully performed in the cardiac catheterization lab. Angiography confirmed near obliteration of the right ventricular cavity and indicated that the right coronary artery was draped over the center of the mass. At 1 week old the patient was explored through a median sternotomy. A large, firm mass was contained within the pericardium, but had expanded to fill most of the mediastinum and right pleural space. The mass occupied the entire anterior surface of the right ventricle, displaced the left ventricle leftward, and prevented access to the right atrium, vena cavae and right pulmonary artery. A 4-mm ringed Gore-Tex shunt (W.L. Gore & Associates, Flagstaff, AZ) was placed from the base of the innominate artery to the main pulmonary artery and the ductus arteriosus was ligated. Biopsies of the mass characterized a spindle cell tumor with collagen deposition and no mitotic activity. Postoperatively the patient was successfully extubated with a systemic oxygen saturation of 87% on room air. As the natural history of his tumor was unclear, he was discharged and followed with serial echocardiograms.
By 4 months of age, echocardiography and cardiac MRI demonstrated interim enlargement of the tumor, with further compression of the right lung (Fig 1).
Evaluation indicated him to be a candidate for heart transplantation. Respiratory failure led to readmission to the hospital, and subsequent need for mechanical ventilation. At 7 months of age, a suitable donor became available. At repeat sternotomy, the tumor once again prevented access to the right atrium and vena cavae. Therefore, the patient was initially placed on bypass utilizing left atrial and ascending aortic cannulation, and converted to bicaval cannulation once cardiopulmonary bypass support allowed manipulation of the tumor. The tumor and native heart were completely excised en bloc, leaving cuffs of the superior and inferior vena cavae for implantation of the donor heart using bicaval anastomoses. The excised tumor measured 7.0 x 4.0 x 5.5 cm, considerably larger than the patient’s heart (Fig 2).
Final pathology revealed the tumor to be a cardiac fibroma.
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Fig 1. Cardiac magnetic resonance imaging scan (coronal view on left, sagittal on right) illustrating a large mass filling most of the right ventricle, mediastinum, and right chest.
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The patient was successfully extubated after 7 days and discharged 3 weeks following cardiac transplant. Immunosupression has been maintained with tacrolimus (FK 506) and prednisone. He continues to do well at 5.5 years posttransplant.
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Comment
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Cardiac fibroma is the second most common cardiac tumor of childhood (after rhabdomyoma). Although benign, fibromas may become symptomatic by causing arrhythmias, or obstruction to blood flow, resulting in either congestive heart failure or cyanosis [1, 2]. Cardiac fibromas are typically firm masses originating in the ventricular septum or ventricular free wall. Symptomatic fibromas have been successfully treated by resection, either partial or complete [1–4, 8]. Whereas complete resection is preferable, even partial resection can result in long-term relief of symptoms [2, 3]. However, our patient’s tumor was not amenable to resection due to its size and the extent of the cardiac structures involved (right ventricle and atrium, tricuspid valve, and right coronary artery). Instead, our patient was palliated by performance of a balloon atrial septostomy and placement of a systemic-to-pulmonary artery shunt. This palliation allowed systemic venous return to pass unobstructed to the left atrium, the left ventricle to support both the systemic and pulmonary circulations, and prostaglandin E-1 to be discontinued. There have been several other reports of successful "single ventricle palliation" for cardiac tumors: by placement of a systemic-to-pulmonary shunt in a 1-month-old patient with a cardiac fibroma [1]; by creation of a bidirectional cavopulmonary anastomosis in a 3-month-old patient with a cardiac fibroma [3]; and by performance of a Fontan procedure in a 24-year-old patient with a rhabdomyoma [4]. In our patient, palliation allowed discharge from the hospital, and provided good relief of cyanosis until it became clear that the tumor was continuing to grow.
Our patient’s palliation also provided a successful bridge to heart transplantation. To our knowledge, this is the first report of single ventricle palliation for a cardiac tumor serving as a bridge to transplant. Although infrequent, heart transplantation for unresectable cardiac fibromas has been reported previously in 4 patients less than 1 year old [5–8]. An alternative to our approach would have been to maintain ductal patency with prostaglandin E-1 until a heart donor became available. Surgical palliation had the advantage of allowing a period of observation and growth without the requirements of hospital admission and intravenous access.
In summary, large cardiac fibromas producing obstruction to blood flow can be managed by either partial or complete resection, when technically feasible. When resection is not possible, and forward flow through the right ventricle is obstructed, "single ventricle palliation" can provide pulmonary blood flow and allow discharge from the hospital as well as time for growth and transplant evaluation. Cardiac transplantation is then a viable end-stage therapy for unresectable cardiac fibromas in children.
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References
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Abstract
Introduction
