Primary Cardiac Tumours in Infancy and Youth in the Small Population: a Seven Year Review

Introduction. Primary tumours of the heart are rare in fetuses, neonates and children; the incidence varies from 0.003% - 0.08% (4, 6, 9, 10) up to 0.2% in children referred for cardiac examination (3, 15). Rhabdomyoma is the most common cardiac tumour during fetal life (60-75%). Teratoma is less common (14 to 19%) with fibroma, myxoma, hemangioma also being described (3, 4, 6, 10, 16, and 18). Multiple rhabdomyomas are associated with tuberous sclerosis in up to 90-95% of cases (3, 4, 5, 6, 10, 12, and 16). No previous study was done on this topic in Latvia so our aim was to determine the incidence, the course and the outcome of primary cardiac tumours in children in our small population. Aim of the study. The aim of our study was to determine the incidence of primary cardiac tumours in our paediatric population, to investigate the nature of the pathology, the course and the outcome of the disease in childhood in Latvia and to compare our results with data from the international literature. Materials and methods. We reviewed retrospectively the clinical, echocardiographic, operative, histological and follow-up data on 17 cases of a primary heart tumours detected within the period of January 1, 2000 till December 31, 2006 in the Clinic for Pediatric Cardiology and Cardiac Surgery of the University Children's Hospital in Riga, Latvia. Results. the incidence of primary heart tumours in the paediatric population in Latvia is 2.4+/-1.4 cases per year. 94% (16) of the primary cardiac tumours in children were benign and 6% (1) malignant. Radical excision performed in all 7 cases of surgical treatment. Rhabdomyomas comprise 47% (n=8) of all the benign tumours with tuberous sclerosis present in 88% of the cases. Conclusions. Most primary cardiac fetal tumours tend to appear in the third trimester of pregnancy, a normal early fetal scan might not rule out cardiac tumours. Relatively often (29% of the cases) the cardiac tumours were an incidental finding. The localization, number and visual appearance of the tumours in echocardiography was indicative of the type of tumour. 41% of the patients with primary cardiac tumours were in need of urgent surgical treatment. Almost all benign primary cardiac tumours were not the cause of death and with the exception of tuberous sclerosis there is good overall prognosis, in the case of malignant primary cardiac tumour the prognosis is poor.