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J Thorac Cardiovasc Surg 2005;129:1358-1363
© 2005 The American Association for Thoracic Surgery
Surgery for Congenital Heart Disease |
a Division of Pediatric Cardiovascular Surgery, University of Padova Medical School, Padova, Italy.
b Institute of Pathology, University of Padova Medical School, Padova, Italy.
c Department of Pediatrics, University of Padova Medical School, Padova, Italy.
Received for publication June 4, 2004; revisions received September 20, 2004; accepted for publication October 11, 2004. * Address for reprints: Cristina Basso, MD, PhD, Institute of Pathology, University of Padova Medical School, Via A. Gabelli, 61 35121 Padova, Italy. (Email: cristina.basso{at}unipd.it).
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Abstract |
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MATERIALS AND METHODS: Between 1986 and 2003, 8 children (3 males and 5 females, age ranging 5 days to 6.7 years, median 78 days) with a clinical diagnosis of cardiac mass were treated with surgery. Diagnosis was made by prenatal echocardiography in 3 patients and by 2-dimensional Doppler echocardiography in 5 patients.
RESULTS: Complete surgical excision of the cardiac mass was feasible in all but 1 patient who underwent orthotopic heart transplantation. Surgical pathology examination revealed myxoma in 2 patients, fibroma in 2 patients, rhabdomyoma in 2 patients (multiple in 1), hamartoma in 1 patient, and teratoma in 1 patient. One patient died of cerebral malignancy 38 months after cardiac transplantation. At a mean follow-up of 69.2 months (range 3–190 months), all the remaining patients are asymptomatic, with good ventricular function on 2-dimensional echocardiography and no signs of residual or recurrent tumor.
CONCLUSION: Surgical excision of obstructive cardiac tumors in childhood is safely feasible. Heart transplantation may represent the only therapeutic option when the tumor extensively invades the ventricular walls. Although 2-dimensional echocardiography remains a reliable diagnostic tool, a definite diagnosis of tumor histotype requires a thorough histopathologic characterization.
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Primary heart tumors are uncommon in patients of pediatric age,1 with a reported incidence of 0.2% in children referred for cardiac disease,2 whereas only 14.2% of all cardiac tumors occur in patients aged less than 16 years.3 Although cardiac myxoma is by far the most common primary heart tumor in adults,4,5 rhabdomyoma is the most frequently encountered heart tumor in infancy and childhood.2,6–11 We report on our experience with surgical treatment of primary cardiac tumors in infancy and childhood.
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Material and Methods |
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Follow-up data were obtained from clinical visits or collected from their referring physicians.
Demographic, clinical, and diagnostic data are summarized in Table 1. The median age at operation was 78 days, ranging from 5 days to 6.7 years; 6 of 8 patients (75%) were younger than 5 months.
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Results |
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Prenatal 2-dimensional echocardiographic diagnosis of cardiac mass was achieved in 3 patients (patients 2, 3, and 6) and then confirmed postnatally. In the 5 remaining patients, the cardiac mass was discovered postnatally as part of the diagnostic investigation by 2-dimensional echocardiography with Doppler examination.
All patients but 1 (patient 3, who presented with paroxysmal supraventricular tachycardia) were in sinus rhythm at preoperative electrocardiogram.
Chest x-ray film showed cardiomegaly in 5 patients, with a mean cardiothoracic ratio of 0.64 and signs of pulmonary congestion in 3 patients. Additional cardiac imaging techniques were used in patients 2 and 4 (computed tomography scan and magnetic resonance imaging, respectively) to clarify the extension of the mass and myocardial wall involvement. Preoperative surgical myocardial biopsy was performed in patient 4, and intraoperative surgical pathology examination of the cardiac mass was performed in patient 8. Surgical intervention consisted of excision of the cardiac mass in 7 patients, and orthotopic heart transplantation was performed in 1 patient (patient 2). Cardiopulmonary bypass was used in all but patient 6 (Table 1). Main gross and histopathologic features are reported in Table 2.
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Two patients had ventricular fibroma. Patient 4 underwent extended demolition of the right ventricular (RV) free wall to excise a huge fibroma and required RV free-wall reconstruction with a patch. A surgical biopsy was performed first to clarify diagnosis, and it showed abundant proliferation of fibroblasts with collagen deposition, in keeping with cardiac fibroma. Diagnosis was confirmed subsequently by histologic examination of the resected mass. Surgical resection of the mass was not feasible in patient 2, who presented with a huge fibroma invading the whole left ventricular cavity, mimicking a hypoplastic left heart syndrome. This patient had been kept on prostaglandin E1 since birth for 38 days, until a suitable heart donor became available. Orthotopic heart transplantation was successfully performed after the Shumway technique, with no complications. The donor was a 3-month-old baby weighing 7 kg. Histologic and immunohistochemical examination revealed a non-encapsulated proliferation of fibroblasts embedded within a network of collagen and elastic fibers that dissociated and entrapped few myocytes.
A sessile vascular hamartoma (hemangioma) occupying the whole right atrial cavity was detached from the fossa ovalis in 1 patient (patient 3, Figure 1). The resected mass consisted of dilated vascular channels (cavernous-capillary type) lined by endothelial cells and filled with blood; calcific deposits and areas of hemorrhage were also visible.
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In 1 case (patient 6, Figure 2), there was a pedunculated extracardiac teratoma, originating from the ascending aorta, with arterial supply originating from the aortic vasa vasorum. Histology revealed a typical teratoma with multicystic appearance and evidence of endodermal-, ectodermal-, and mesodermal-derived cells.
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At a mean follow-up of 69.2 months (3–190 months), patient 2 died of cerebral astrocytoma 38 months after heart transplantation, whereas all the remaining patients are alive and in good clinical condition with no cardiac-related therapy. Patients 1 and 5 are receiving oral therapy for epilepsy and tuberous sclerosis, respectively. Electrocardiogram shows that sinus rhythm and Holter monitoring are within normal limits in all patients. Two-dimensional echocardiography detects good ventricular function (ejection fraction: 75%; shortening fraction: 43.5%); no residual valve regurgitation or recurrent cardiac masses are found.
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Discussion |
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Despite the relatively high frequency in our series, cardiac myxoma in infancy is usually rare compared with the adult population.4,7 Clinical manifestation includes symptoms and signs of tumor embolism, cavity or valve obstruction, and constitutional symptoms.6,7 Because of the rapid growth of tumor mass in small-sized cavities, congestive heart failure has been frequently reported in childhood and surgical treatment is lifesaving.
Cardiac fibroma is the second most common tumor of childhood in autopsy series after rhabdomyoma, but it ranks first in surgical series.3,4 The tumors are usually single masses and most often arise in the left ventricular free wall, whereas RV free-wall and atrial involvement are rare.6,12 The spectrum of clinical presentation is wide, including congestive heart failure, heart murmurs, and sudden death.7 Both of our patients presented with congestive heart failure caused by the large size of the tumor. Spontaneous regression of fibromas has never been described,6 and surgery is usually required. Midterm excellent results after partial resection of fibromas, with 100% freedom from recurrence of tumoral mass, have been reported.13 Bidirectional cavopulmonary connection has been associated with subtotal resection of RV fibroma to unload the RV.14 In the setting of huge, unresectable mass, infiltrating cardiac walls transplantation may be the only therapeutic option.15
Rhabdomyoma is the most common intracardiac tumor diagnosed in infancy.2,6–8 The tumors are usually multiple, most often involving the ventricular myocardium but sometimes projecting into the cavity or freely moving as a pedunculated mass.6 Ventricular inflow or outflow tracts obstruction, arrhythmia, atrioventricular block, pericardial effusion, and even sudden death have been reported as clinical presentation.7,16 Potential spontaneous regression,2,4,7,8 which justifies eventual subtotal resection or even a nonsurgical approach, and the close association with tuberous sclerosis7,8,17 are described. However, the presence of severe ventricular outflow tract obstruction, as in our cases, may require emergency surgical treatment to avoid hemodynamic impairment.9–11
Vascular hamartomas (or hemangiomas) are rare benign neoplasms.18 They have been described at all ages; they may present with arrhythmias, pericardial effusion, congestive heart failure, extracardiac obstruction, and coronary insufficiency.19 In symptomatic patients, surgery is usually a highly effective and safe therapy.
Intrapericardial teratomas are rare primary cardiac tumors that typically arise within the pericardium from a pedicle at the base of the great vessels.3,6,7 Although benign, they may vary in size, often enlarging up to 15 cm.20 Virtually all intrapericardial teratomas are associated with pericardial effusion,21,22 which can cause signs and symptoms of heart compression.7 Complete surgical excision of these tumors is usually curative, without the need for cardiopulmonary bypass.20,22
In our limited but heterogenous experience with cardiac tumors in infancy, surgical resection of cardiac tumor has proved to be safe and technically feasible, even in early infancy; surgery usually has been definitive, and reoperation has not been required. The only limitation to a conservative surgical approach has been the extensive invasion by the tumor mass of the myocardium, a condition in which transplantation may remain the only therapeutic chance.
Experience with fetal recognition of cardiac tumors has been gained,6 and the incidence of cardiac tumor at prenatal diagnosis has been estimated to be 0.14%.23 Prenatal diagnosis of cardiac tumors was feasible in 38% of cases in our series. Thus, primary cardiac tumors are now amenable to effective surgery even in the first days of life, resulting in the early restoration of the normal cardiac physiology and prevention of life-threatening complications.
According to our experience, 2-dimensional echocardiography remains a highly reliable imaging diagnostic tool, and all other diagnostic imaging techniques remain complementary.16,24 However, precise information on the tumor histotype is not attainable by echocardiography. A correct therapeutic plan requires an accurate histopathologic diagnosis of the resected mass to rule out the rare case of a malignancy. Preoperative or intraoperative surgical pathology diagnosis on frozen sections may be useful in some instances, as in 2 of our cases, to determine the feasibility and extent of surgical resection.
Despite the severity of presenting symptoms of some tumors in the neonatal period, midterm follow-up results are strongly encouraging. Thus, we advocate surgical treatment of symptomatic cardiac tumors in childhood, because although they are mostly benign, they frequently present a "malignant" hemodynamic behavior.
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References |
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This article has been cited by other articles:
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  H. Gasparovic, V. Coric, D. Milicic, G. Rajsman, I. Burcar, R. Stern-Padovan, and I. Jelic Left Ventricular Fibroma Mimicking an Acute Coronary Syndrome Ann. Thorac. Surg., November 1, 2006; 82(5): 1891 - 1892. [Abstract] [Full Text] [PDF]
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