Long-Term Outcomes of Pediatric Intracardiac Masses: Clinical Course and Tumor Behavior in a Single-Center Experience

Authors

Şule Arıcı, Ahmed Mahmud Çırakoğlu, Figen Akalın
https://doi.org/10.18621/eurj.1220

Objective: We aimed to evaluate the clinical characteristics, diagnostic pathways, echocardiographic and Holter findings, treatment strategies, and long-term outcomes of children with intracardiac masses, and to explore factors associated with tumor regression.

Methods: This retrospective observational study included children with echocardiographically confirmed intracardiac masses followed at a tertiary pediatric cardiology center between January 2010 and January 2022. Demographic and clinical characteristics, tumor features, serial echocardiographic measurements, 24-hour Holter recordings, treatment strategies, and follow-up outcomes were analyzed.

Results: A total of 20 patients were included. Rhabdomyoma was the most common tumor type (60%, n=12), followed by fibroma (30%, n=6). Most patients were asymptomatic or mildly symptomatic at presentation (90%, n=18). Clinically significant arrhythmias were observed in 15% (n=3), predominantly in patients with fibromas. Tumor size reduction was observed in 40% (n=8) of patients. All patients diagnosed during the fetal period (15%, n=3) exhibited tumor size reduction; all had rhabdomyomas. Everolimus was administered in 25% (n=5) of patients, with tumor size reduction observed in 80% (n=4) of treated cases; however, this was not statistically significant (P=0.253). Left ventricular systolic function remained preserved in all patients during follow-up.

Conclusion: Pediatric intracardiac masses are predominantly benign but show heterogeneous clinical behavior. Tumor regression was more frequent in rhabdomyomas; however, this may reflect tumor type distribution. Arrhythmias were more frequent in fibromas, supporting rhythm surveillance. Everolimus may be considered in selected patients; however, findings should be interpreted with caution due to limited sample size and potential selection bias, and require confirmation in larger studies.

Pediatric Cardiac Tumors, Rhabdomyoma, Fibroma, Arrhythmia, Echocardiography, Everolimus

1. Tao TY, Yahyavi-Firouz-Abadi N, Singh GK, Bhalla S. Pediatric cardiac tumors: clinical and imaging features. Radiographics. 2014;34(4):1031-1046. doi:10.1148/rg.344135163.

2. Bilgiç A, Ozbarlas N, Ozkutlu S, Ozer S, Ozme S. Cardiomyopathies in children. Clinical, epidemiological and prognostic evaluation. Jpn Heart J. 1990;31(6):789-797. doi:10.1536/ihj.31.789.

3. Chao AS, Chao A, Wang TH, et al. Outcome of antenatally diagnosed cardiac rhabdomyoma: case series and a meta-analysis. Ultrasound Obstet Gynecol. 2008;31(3):289-295. doi:10.1002/uog.5264.

4. Tworetzky W, McElhinney DB, Margossian R, et al. Association between cardiac tumors and tuberous sclerosis in the fetus and neonate. Am J Cardiol. 2003;92(4):487-489. doi:10.1016/s0002-9149(03)00677-5.

5. Parmley LF, Salley RK, Williams JP, Head GB 3rd. The clinical spectrum of cardiac fibroma with diagnostic and surgical considerations: noninvasive imaging enhances management. Ann Thorac Surg. 1988;45(4):455-465. doi:10.1016/s0003-4975(98)90028-5.

6. Jones JP, Ramcharan T, Chaudhari M, et al. Ventricular fibromas in children, arrhythmia risk, and outcomes: A multicenter study. Heart Rhythm. 2018;15(10):1507-1512. doi:10.1016/j.hrthm.2018.06.018.

7. Yıldırım S, Aypar E, Aydın B, et al. Cardiac rhabdomyomas: clinical progression, efficacy and safety of everolimus treatment. Turk J Pediatr. 2023;65(3):479-488. doi:10.24953/turkjped.2022.922.

8. Dhulipudi B, Bhakru S, Rajan S, Doraiswamy V, Koneti NR. Symptomatic improvement using everolimus in infants with cardiac rhabdomyoma. Ann Pediatr Cardiol. 2019;12(1):45-48. doi:10.4103/apc.APC_79_18.

9. Hurtado-Sierra D, Ramos Garzón JX, Romero-Guevara SL, Serrano-García AY, Rojas LZ. Everolimus and sirolimus in the treatment of cardiac rhabdomyomas in neonates. Pediatr Res. 2025;98(6):2045-2057. doi:10.1038/s41390-025-04043-8.

10. Mariano A, Pita A, León R, et al. Primary cardiac tumors in children: a 16-year experience. Rev Port Cardiol. 2009;28(3):279-288.

11. Kocabaş A, Ekici F, Cetin Iİ, et al. Cardiac rhabdomyomas associated with tuberous sclerosis complex in 11 children: presentation to outcome. Pediatr Hematol Oncol. 2013;30(2):71-79. doi:10.3109/08880018.2012.734896.

12. Miyake CY, Del Nido PJ, Alexander ME, et al. Cardiac tumors and associated arrhythmias in pediatric patients, with observations on surgical therapy for ventricular tachycardia. J Am Coll Cardiol. 2011;58(18):1903-1909. doi:10.1016/j.jacc.2011.08.005.

13. Jayaprakash S. Clinical presentations, diagnosis, and management of arrhythmias associated with cardiac tumors. J Arrhythm. 2018;34(4):384-393. doi:10.1002/joa3.12030

14. Beghetti M, Gow RM, Haney I, Mawson J, Williams WG, Freedom RM. Pediatric primary benign cardiac tumors: a 15-year review. Am Heart J. 1997;134(6):1107-1114. doi:10.1016/s0002-8703(97)70032-2.

15. Myers KA, Wong KK, Tipple M, Sanatani S. Benign cardiac tumours, malignant arrhythmias. Can J Cardiol. 2010;26(2):e58-e61. doi:10.1016/s0828-282x(10)70009-x.

16. Isaacs H Jr. Fetal and neonatal cardiac tumors. Pediatr Cardiol. 2004;25(3):252-273. doi:10.1007/s00246-003-0590-4.

17. Freedom RM, Lee KJ, MacDonald C, Taylor G. Selected aspects of cardiac tumors in infancy and childhood. Pediatr Cardiol. 2000;21(4):299-316. doi:10.1007/s002460010070.

18. Ying L, Lin R, Gao Z, Qi J, Zhang Z, Gu W. Primary cardiac tumors in children: a center's experience. J Cardiothorac Surg. 2016;11(1):52. doi:10.1186/s13019-016-0448-5.

19. Padalino MA, Vida VL, Boccuzzo G, et al. Surgery for primary cardiac tumors in children: early and late results in a multicenter European Congenital Heart Surgeons Association study. Circulation. 2012;126(1):22-30. doi:10.1161/CIRCULATIONAHA.111.037226.

There are 19 references in total.
1.
Arıcı Şule, Çırakoğlu AM, Akalın F. Long-Term Outcomes of Pediatric Intracardiac Masses: Clinical Course and Tumor Behavior in a Single-Center Experience. Eur Res J. Published online May 12, 2026:1-13. doi:10.18621/eurj.1220

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