Surgically treated primary cardiac tumors in early infancy and childhood

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.