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J Thorac Cardiovasc Surg 2005;129:1358-1363
© 2005 The American Association for Thoracic Surgery

Surgery for Congenital Heart Disease

Surgically treated primary cardiac tumors in early infancy and childhood

^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).

OBJECTIVE: Primary heart tumors in childhood are rare and mostly benign. Surgical treatment is advocated when symptoms or hemodynamic impairment is present.

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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