Echocardiography and Newer Imaging Techniques in Diagnosis and Long-Term Follow-Up of Primary Heart Tumors in Children

Abstract

Background: Primary heart tumors (PHTs) in the pediatric population are very rare and do not manifest any characteristic symptoms.

Methods: A retrospective analysis of 61 cases was undertaken. Data from three centers for the years 2003-2018 were gathered. The tumors' clinical course, location, number, hemodynamic, treatment, and follow-up were evaluated. Echocardiography was complemented with magnetic resonance imaging, computer tomography, and histopathological examination.

Results: Out of 61 PHT diagnoses, 56 (91.8%) were circumstantial including all 16 (26.2%) prenatal tumors. The reasons for cardiological consultations were arrhythmia, syncopes, lowered physical performance, and murmurs. Only five patients (8.2%) were suspected of tumors based on previous symptoms of sclerosis tuberosa. Rhabdomyoma was the most frequently found PHT (60.7%). The tumors were predominantly located in the ventricles (49.1%) and intraventricular septum (14.9%) and tended to be single (70.5%). About 37.7% of patients suffered from coexistent multi-organ problems, two (3.28%) from congenital heart defects and one (1.64%) from Carney's syndrome. Tumor resection was performed on 26 (42.7%) patients, of which 16 (61.5%) underwent total and 10 (38.5%) partial tumor resection. During the follow-up (mean 4.3 years), 54 patients (88.5%) have improved or were stable, while seven (11.5%) died.

Conclusions: Primary pediatric heart tumors are diagnosed completely circumstantially, and the most common is rhabdomyoma, although arrhythmia may suggest fibroma. Diagnosis of a heart tumor in children is not synonymous with fatal prognosis, and most of them require only constant observation. Life-saving operation allows improvement, while the prognosis for malignant tumors in children is definitely unfavorable.

Keywords: children; primary heart tumor; rhabdomyoma; sclerosis tuberosa.

Figure 1

Two-dimensional echocardiography: parasternal long axis image obtained in a 16-year boy shows a huge hyperechoic mass (*) in the left atrium (LA) which was significantly hindering the flow of blood to the left ventricle through the mitral valve. RV—Right Ventricle; IVS—Interventricular Septum; LV— Left Ventricle; LA—Left Atrium; Ao—Aorta.

Figure 2

Two-dimensional echocardiography: parasternal long axis image obtained in the same patient as in Figure 1, after subtotal resection of tumor. Although the tumor was removed completely, infiltration of the left atrial wall was found. The thickening of the left atrial wall and its irregular shape are clearly visible (*). Pericardial effusion (PEff) is also present about 1 cm behind the posterior wall of the LV. Abbreviations as in Figure 1.