Primary cardiac tumors in infants and children: immediate and long-term operative results.

BACKGROUND: The literature contains few large or long-term series involving infants and children with primary cardiac tumors. This article summarizes our 35-year experience with such lesions.

METHODS: Between January 1961 and January 1996, 40 infants and children (mean age, 3.3 years; range, 2 days to 17 years; 65% female) were diagnosed at our institution with primary cardiac tumors. Of these tumors, 37 (92%) were benign and 3 (8%) were malignant. Tumors were resected in 38 patients (95%). In 2 patients (5%), biopsy confirmed rhabdomyoma; however, presenting symptoms spontaneously resolved, so these patients did not undergo tumor resection. Follow-up echocardiographic studies showed a diminishing tumor mass in each of these patients.

RESULTS: Immediate, symptom-free status was achieved in all patients. There were two early deaths, for an operative mortality of 5%. Three late postoperative deaths (7.5%) occurred as follows: 1 patient with a myocardial hamartoma died at 3 months of congestive heart failure. Another patient with a recurrent rhabdomyosarcoma died at 6 months, and a third patient with a recurrent fibrosarcoma died at 28 months. Long-term follow-up was available for 34 survivors (97% complete) and totaled 240.2 patient-years (mean, 7.1 years/patient). All remaining survivors were without evidence of presenting symptoms and tumor recurrence or progression.

CONCLUSIONS: The data suggest that an aggressive operative approach is warranted for benign symptomatic and malignant tumors. This aggressive approach has resulted in extended symptom-free status in patients with benign lesions, and significant palliation and longer survival in patients with malignant lesions, with acceptably low operative risk.