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Background: Cardiac tumors in childhood are rare, although they would probably appear with greater frequency in fetal life upon closer examination during obstetric ultrasound studies. Prevalence is approximately 0.13%, as reported by fetal echocardiographic studies. We sought to determine the prevalence and natural history of cardiac tumors in patients referred to our laboratory for fetal echocardiography (FE). Methods: Between May 1998 and May 2003, five fetuses with cardiac tumors have been sent to FE out of 8,589 obstetric echographic studies. Gestational age varied from 20 to 34 weeks and maternal age from 19 to 38 years. Results: Prevalence of fetal cardiac tumors was 5 out of 8,589 fetuses: 0.05% in the general population, and 5 of 66: 7.5% of congenital heart disease. Two patients presented with multiple rhabdomyomas in the left venticle (LV), interventricular septum (IVS), and left ventricular outlet; they also had tuberous sclerosis (TS) with intracerebral tumors detected by magnetic resonance imaging (MRI) and cardiac tumors regressed during the first year of life and were asymptomatic. Another fetus with a single tumor with a rhabdomyoma echocardiographic appearance with attachment at the IVS and with a 50-mmHg LV outlet gradient did well in postpartum, and at 5 months of life the mass became smaller and the gradient decreased to 35 mmHg. One patient with a large LV mass, which in the echo appeared as fibroma, required early surgery at 10 days; the tumor was almost completely resected, and 4 years later the small residual mass has not grown. Histopathological exams revealed cardiac fibrosarcoma. The last fetus, with a single intraseptal tumor, which also appeared as a large fibroma in the echo and that had increased in size after birth, died at 7 months with low cardiac output. MRI did not identify brain tumors. Autopsy was not authorized by the parents. All the prenatal diagnoses were confirmed after birth. Conclusions: (1) Prevalence of fetal cardiac tumors in our hospital was 0.05% and represented 7.5% of congenital heart disease in a 5-year period; (2) the majority were rhabdomyomas with spontaneous regression after birth; (3) multiple rhabdomyomas were associated with TS; (4) no deaths occurred during fetal life in this series, one died at 7 months due to progresive invasion of the LV cavity and another patient required surgery to relieve the obstruction; (5) fetal echocardiography permits an accurate diagnosis, which allows for pregnancy follow-up and parental counselling. [ABSTRACT FROM AUTHOR]

Competing Interests: Conflict of interest: none declared.

Cardiac arrhythmias in pediatric patients have different mechanisms and frequencies compared to adult patients. There are many physiological differences between children and adults that may affect the pharmacodynamic and pharmacokinetic of the antiarrhythmic drugs in pediatric population. Children, and specially breast feeding children, cannot be considered low weighted adults to select antiarrhythmic drug doses. Although radiofrequency ablation has experienced great technological advances, it is performed in selected pediatric patients. Therefore, the main therapeutic strategy is the use of antiarrhythmic drugs in children. The medical management of arrhythmias in pediatric patients is challenging and complex. There are few clinical guidelines. There is scarce and incomplete information about the efficacy and safety of antiarrhythmic drugs in pediatric population. Most of the doses and drug administration intervals are extrapolated from adult population and applied to children. Antiarrhythmic drug doses have been extensively studied in adult population. However, in pediatric population, there are very few clinical trials and the safety of these drugs is not well known. In general, dose regimens are based on small uncontrolled studies, extrapolation of drug doses from studies performed in the adult population or physician experience. As a consequence, there is a need for further studies to assess the most effective antiarrhythmic drug regimens in children reducing the risk of side effects. Evidence suggests that medical research in pediatric population is necessary and morally valuable. But investigators involved must take care of moral and ethical values, including the respect for the child-subject and his parents or legal representatives, and this respect compels them to consider the patient and family in the decision making process. The participation request and the informed consent must be obtained according to the competitions the patient exhibits, trying to anticipate information about benefits and possible damages derived from the investigation in an understandable language for him. In our opinion the pharmacologic clinical investigation of antiarrhythmic treatments in pediatrics is necessary. More clinical studies must be carried out under rigorous scientific rules that contemplate the particular ethical dilemmas this population faces.