[Primary cardiac tumours in infancy].

Area of Science:

• Pediatric Cardiology
• Cardiovascular Pathology
• Oncology

Background:

• Primary cardiac tumors in children are exceptionally rare, with an incidence ranging from 0.0017% to 0.28%.
• Over 90% of these tumors are benign.
• Rhabdomyoma constitutes the most frequent type, occurring in over 60% of cases associated with tuberous sclerosis.

Purpose of the Study:

• To analyze the clinical characteristics, diagnosis, and outcomes of primary cardiac tumors in a pediatric cohort.
• To determine the prevalence and common types of cardiac tumors in children.
• To evaluate the diagnostic utility of various imaging modalities.

Main Methods:

• Retrospective analysis of medical records for primary cardiac tumors diagnosed between March 1977 and March 2007.
• Inclusion of 27 pediatric patients.
• Review of diagnostic methods including echocardiography, cardiac catheterization, and angioresonance.

Main Results:

• The most frequent age of diagnosis was the neonatal period, often presenting as a heart murmur.
• Echocardiography identified 20 rhabdomyomas, 2 fibromas, 2 pericardial teratomas, and 3 unclassified tumors, predominantly in the left ventricle.
• Arrhythmias occurred in 11 patients, 5 required surgery, and 3 deaths were attributed to cardiac causes. 75% of rhabdomyoma cases were associated with tuberous sclerosis, and most rhabdomyomas showed spontaneous regression.

Conclusions:

• Primary cardiac tumors are uncommon in children, with rhabdomyoma being the most prevalent, frequently associated with tuberous sclerosis.
• Echocardiography is the primary diagnostic tool, with fetal echocardiography enabling early detection.
• While most tumors have a benign course and rhabdomyomas tend to regress, monitoring for arrhythmias and surgical intervention for obstructive symptoms are crucial.