Surgery for Primary Cardiac Tumors in Children: Early and Late Results in a Multi-Center European Congenital Heart Surgeons Association (ECHSA) Study.

BACKGROUND: To evaluate indications and results of surgery for primary cardiac tumors in children. METHODS AND RESULTS: Eighty-nine patients aged ≤ 18 years undergoing surgery for cardiac tumor between 1990 and 2005 from 16 centers were included retrospectively (M/F =41/48; median age 4.3 months, range 1 day-18 years). Sixty-three patients (68.5%) presented with symptoms. Surgery consisted in complete resection in 62 (69.7%), partial in 21 (23.6%), and cardiac transplant in 4 (4.5%). Most frequent histotypes (93.2%) were benign (rhabdomyoma, myxoma, teratoma, fibroma and hemangioma). Post-operative complications occurred in 29.9%. Early and late mortality were 4.5% each (mean follow up: 6.3 ± 4.4 years); major adverse events occurred in 28.2% of the patients; 90.7% of patients are in NYHA class I. There were no statistically significant differences in survival, post-operative complications or adverse events after complete and partial resection in benign tumors other than myxomas. Cardiac transplant was associated significantly with higher mortality rate (p 0.006). Overall mortality was associated to malignancy (p 0.0008), and adverse events during follow up (p 0.005). CONCLUSIONS: Surgery for primary cardiac tumors in children has good early and long term outcomes, with low recurrence rate. Rhabdomyomas are the most frequent surgical histotypes. Malignant tumors affect negatively early and late survival. Heart transplant is indicated when conservative surgery is not feasible. Lack of recurrence after partial resection of benign cardiac tumors indicates that a less risky tumor debulking is effective for a subset of histotypes such as rhabdomyomas and fibromas.