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

Janusz Skalski,Joanna Kohut,Beata Radzymińska-Chruściel,Tomasz Mroczek,Andrzej Rudziński,Aleksandra Morka,Piotr Weryński,Marcin Gładki,Lesław Szydłowski

doi:10.3390/ijerph17155471

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.

Highlights

Primary heart tumors (PHTs) in children are rare, and their frequency varies depending on the evaluation method used
Of all 61 cases, only five (8.2%) children had been referred for suspected cardiac tumor, while all the other members of the sample had a previous neurological diagnosis of sclerosis tuberosa (ST)
Twenty-four patients were subjected to brain ultrasonography and one presented hemorrhagic alterations connected with deep fetal bradycardia and resuscitation on the first day of life

Summary

Introduction

Primary heart tumors (PHTs) in children are rare, and their frequency varies depending on the evaluation method used. In post-mortem examination, heart tumors are diagnosed in 0.0017–0.028%. Due to the wider application of echocardiographic screening during cardiological and prenatal consultations, PHTs are detected more frequently. During both prenatal and postnatal periods, there may be no clinical symptoms of tumors, or they may cause outflow obstruction. Less commonly, they may be located in the atrioventricular groove and cause arrhythmias related to the creation of an accessory pathway. Results: Out of 61 PHT diagnoses, 56 (91.8%) were circumstantial including all 16

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