Primary cardiac tumours in infants and children: surgical strategy and long-term

Objective: This report is a comprehensive review of our 26-year experience with primary cardiac tumours in infants and children with emphasis on surgical indications, strategies and long-term outcome.

Methods: Between 1986 and 2012, 46 infants and children (mean age 6.6 ±2.4 months, range 1 day-17 years ) underwent either subtotal or total resection of primary cardiac tumors (rhabdomyoma n=13, fibroma n=10, teratoma n=9, myxoma n=8, hemangioma n=2, rhabdomyosarcoma n=1, non-Hodgin’s lymphoma n=1, lymphangioma n=1). There were 6 asymptomatic patients. Indications of resection were presence of hemodynamic and respiratory compromise, severe arrhythmia and a significant risk of embolization. Approach and strategy of resection varied according to tumor location and hemodynamic status without damage to adjacent structures.

Results: Morbidity included bleeding in a patient and a transient low output state in another. A 5-month old girl with left ventricular fibroma underwent left ventricular assist device implantation secondary to failure from weaning off cardiopulmonary bypass and she eventually underwent heart transplantation 17 days later. Early mortality included a 5-month old boy who underwent complete resection of rhabdomyoma located in the left ventricle. Late mortality occurred in a 16-year old boy with non-Hodgkin’s lymphoma who died 7 months after the surgery.

Mean duration of follow-up is 11.6±3.5 years. Forty-three patients are alive (93.4%) and well, the majority of whom are in Ross functional classification 1.

Conclusions: This study illustrates that individualized approach to tumour resection allows restoration of an adequate hemodynamic function and satisfactory long-term tumour-free outcome.