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Tumour-anchored Purkinje-mediated ventricular tachycardia in a child with cardiac rhabdomyoma

Published online by Cambridge University Press:  30 October 2026

Gaku Izumi Open the ORCID record for Gaku Izumi [Opens in a new window] ,
Daisuke Sasaki  and
Taro Temma
Gaku Izumi*
Affiliation:
Department of Pediatrics, Hokkaido University Graduate School of Medicine, Sapporo, Japan
Daisuke Sasaki
Affiliation:
Department of Pediatrics, Hokkaido University Graduate School of Medicine, Sapporo, Japan
Taro Temma
Affiliation:
Division of Cardiology, Sakakibara Heart Institute, Fuchu, Japan Department of Cardiovascular Medicine, Hokkaido University Graduate School of Medicine, Sapporo, Japan
*
Corresponding author: Gaku Izumi; Email: gaku-izumi0920@med.hokudai.ac.jp
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Abstract

Cardiac rhabdomyomas are associated with ventricular arrhythmias, but mechanisms remain uncertain. A 10-year-old boy with tuberous sclerosis complex developed verapamil-sensitive fascicular ventricular tachycardia adjacent to a residual cardiac rhabdomyoma. Electrophysiological mapping demonstrated Purkinje-mediated reentry. Catheter ablation at a tumour-adjacent site eliminated the arrhythmia, suggesting that residual rhabdomyoma may anchor a localised reentrant substrate.

Keywords

Cardiac rhabdomyomafascicular ventricular tachycardiaPurkinje potentialreentrytuberous sclerosis complexcatheter ablation

Information

Type
Case Report
Information
Cardiology in the Young , First View , pp. 1 - 4
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Copyright
© The Author(s), 2026. Published by Cambridge University Press

Introduction

Cardiac rhabdomyoma is the most common cardiac tumour in childhood and may be associated with ventricular arrhythmias. Most reported arrhythmias have been attributed to tumour automaticity. Reference Uzun, Wilson, Vujanic, Parsons and De Giovanni1,Reference Tyebally, Chen and Bhattacharyya2 We describe a child with fascicular ventricular tachycardia in whom electrophysiological findings suggested a tumour-associated Purkinje-mediated reentrant mechanism.

Case report

A 10-year-old boy with tuberous sclerosis complex was referred because of recurrent palpitations. Multiple cardiac rhabdomyomas had been identified prenatally and regressed spontaneously during childhood. At birth, the cardiac tumours were extensive, involving the interventricular septum and left ventricle and causing clinically relevant narrowing of the right ventricular outflow tract. Precise measurements of the tumours at birth were not available from the historical imaging records. At presentation, episodes of sustained tachycardia occurred at approximately 170 beats/min with preserved haemodynamics.

Twelve-lead electrocardiography demonstrated a relatively narrow QRS tachycardia (140 ms) with right bundle branch block morphology, left-axis deviation, and atrioventricular dissociation, consistent with left fascicular ventricular tachycardia. Oral verapamil completely suppressed the arrhythmia. Echocardiography and cardiac CT demonstrated a residual tumour measuring approximately 4 × 8 mm adjacent to the posterior papillary muscle near the expected course of the left posterior fascicle (Figure 1).

Figure 1.

Clinical findings. Twelve-lead electrocardiogram demonstrating fascicular ventricular tachycardia and imaging showing a residual cardiac rhabdomyoma adjacent to the posterior papillary muscle. APM = anterior papillary muscle, PPM = posterior papillary muscle, red arrow: tumour.

Three images showing cardiac data and imaging related to a child with fascicular ventricular tachycardia.
Figure 1. Long description

Panel A: A twelve-lead electrocardiogram displaying fascicular ventricular tachycardia. The graph shows multiple leads with distinct waveforms. Panel B: An echocardiogram image highlighting a cardiac rhabdomyoma near the posterior papillary muscle. The red arrow points to the tumor, with labels indicating the anterior papillary muscle (APM) and posterior papillary muscle (PPM). Panel C: A cardiac MRI image showing the same tumor adjacent to the posterior papillary muscle, with the red arrow indicating the tumor location and labels for APM and PPM.

Electrophysiological study reproducibly induced ventricular tachycardia with a cycle length of 360 ms using programmed atrial and ventricular stimulation. During ventricular tachycardia, presystolic Purkinje potentials (P1) preceded ventricular activation and propagated in a proximal-to-distal sequence along the left posterior fascicular region. Entrainment pacing demonstrated fusion, and ventricular tachycardia could be terminated by programmed stimulation, supporting a reentrant mechanism.

Electroanatomical mapping localised presystolic Purkinje activity to the left posterior fascicular network. Preprocedural cardiac CT images were integrated with both live fluoroscopy and the three-dimensional electroanatomical map. For CT–fluoroscopy fusion, the syngo Fusion package on an ARTIS icono D-Spin angiography system (Siemens Healthineers) was used. The reconstructed three-dimensional CT image automatically followed changes in C-arm rotation and angulation, thereby maintaining the spatial relationship between the residual tumour and the catheter across different fluoroscopic projections. Together, CT–fluoroscopy fusion and integration of CT with the three-dimensional electroanatomical map demonstrated that the catheter tip at the critical basal region was closely apposed to the endocardial region corresponding to the residual rhabdomyoma. Radiofrequency ablation at a more apical Purkinje site failed to terminate ventricular tachycardia despite recording presystolic Purkinje potentials. The catheter was subsequently repositioned to the basal tumour-adjacent region. Because of the patient’s small body size and the retrograde transaortic approach, a 5-French ablation catheter without contact-force sensing was used. Although catheter–tissue contact could not be quantitatively assessed, advancement of the catheter at the target site resulted in deflection of the catheter shaft without further advancement of the tip, supporting tissue contact. Radiofrequency energy delivered at this site progressively slowed and terminated ventricular tachycardia. Intracardiac echocardiography was not used because of vascular-access limitations associated with the patient’s body size and because adequate spatial information was obtained from the two complementary image-integration methods. Additional lesions were avoided because of concern for left posterior fascicular injury.

After ablation, delayed Purkinje potentials were recorded during sinus rhythm at the successful site, and ventricular tachycardia was no longer inducible. The patient remained free from arrhythmia recurrence during 8 months of follow-up without antiarrhythmic medication (Figure 2).

Figure 2.

Electrophysiological findings and ablation. Left ventricular electrograms were recorded using a 10-pole electrode catheter positioned in the left ventricle through a retrograde transaortic approach. Purkinje potentials (P1) preceding ventricular activation with a proximal-to-distal (basal to apical) sequence along the left ventricular septal region ( a ). Entrainment pacing from the right ventricular apex demonstrating constant fusion at a pacing cycle length (PCL) of 350 ms and progressive fusion at 340 ms ( b ). Electroanatomical mapping localised the critical region adjacent to the residual tumour. Radiofrequency ablation at the basal tumour-adjacent site terminated the ventricular tachycardia (VT) and rendered it noninducible ( c – e ). Proposed mechanism of tumour-anchored Purkinje reentry ( f ). ABL = ablation catheter, LV = left ventricle, PPM = posterior papillary muscle, RA = right atrium, RAO = right anterior oblique, RV = right ventricle, red arrow: tumour.

Electrophysiological findings and ablation related to a cardiac tumor.

Discussion

This case provides electrophysiological evidence that a residual cardiac rhabdomyoma may serve as an anatomical anchor for a Purkinje-mediated reentrant ventricular tachycardia.

Several observations support this interpretation. First, the arrhythmia demonstrated classical features of fascicular ventricular tachycardia, including verapamil sensitivity, reproducible inducibility, entrainment with fusion, and presystolic Purkinje potentials. Reference Ohe, Aihara, Kamakura, Kurita, Shimizu and Shimomura3–Reference Bogun, Good and Reich6 These findings strongly favour a reentrant Purkinje-dependent mechanism rather than automaticity. Second, the successful ablation site was not the most distal Purkinje site exhibiting presystolic activity but a basal region immediately adjacent to the residual tumour. Finally, focal ablation at this location terminated ventricular tachycardia and rendered it noninducible.

Most ventricular arrhythmias associated with cardiac rhabdomyoma have been presumed to result from abnormal automaticity arising from tumour tissue. In contrast, the present case behaved electrophysiologically as fascicular ventricular tachycardia while demonstrating a close spatial relationship between the critical circuit component and a residual structural lesion. The combined use of CT–fluoroscopy fusion and integration of CT with three-dimensional electroanatomical mapping demonstrated close spatial colocalization between the successful ablation site and the residual tumour. These findings suggest that the tumour may have contributed to the formation or maintenance of a localised reentrant substrate within the adjacent Purkinje network.

The precise mechanism remains uncertain. Regression of the tumour may have altered its anatomical relationship with the Purkinje network. One possibility is that tumour regression brought the Purkinje network closer to the endocardial surface. Alternatively, the residual tumour may have altered the local architecture or conduction properties of the surrounding Purkinje network, thereby facilitating reentry. Direct participation of the tumour or tumour-associated tissue in the reentrant circuit also cannot be excluded. However, the present imaging and electrophysiological findings cannot distinguish among these possibilities. Previous pathological studies have suggested developmental and morphologic similarities between rhabdomyoma cells and Purkinje cells, Reference Elliott, Bargeron, Soto and Bream7 raising the possibility that residual tumours may influence adjacent Purkinje conduction properties. Similar interactions between structural abnormalities and Purkinje fibres have been described in other forms of ventricular tachycardia.

This report has several limitations. Cardiac MRI was not performed; therefore, the depth of the residual tumour and its extension into the deeper myocardium could not be determined. Catheter–tissue contact could not be quantified because a contact-force-sensing catheter was not used. Moreover, direct catheter contact with the tumour tissue could not be definitively established because intracardiac echocardiography was not used. Histological confirmation of tumour involvement in the reentrant substrate was also unavailable. The proposed mechanism is therefore based on anatomical and electrophysiological colocalization rather than definitive confirmation of tumour involvement. Consequently, these observations should be regarded as hypothesis-generating.

Conclusion

Residual cardiac rhabdomyoma may be associated with a localised Purkinje-mediated reentrant substrate amenable to catheter ablation. In patients with tumour-associated ventricular tachycardia, detailed electrophysiological evaluation should be considered before attributing the arrhythmia solely to tumour automaticity. This case expands the spectrum of arrhythmogenic mechanisms associated with cardiac rhabdomyoma beyond tumour automaticity.

Data availability statement

The data that support the findings of this study are available on request from the corresponding author. The data are not publicly available due to privacy or ethical restrictions.

Acknowledgements

None.

Financial support

This research received no specific grant from any funding agency or from any commercial or not-for-profit sectors.

Competing interests

All authors declare that they have no conflict of interest.

Informed consent

Informed consent was obtained from the participant included in the study.

References

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Tyebally, S, Chen, D, Bhattacharyya, S, et al. Cardiac tumors: JACC CardioOncology state-of-the-art review. JACC CardioOncol 2020; 2: 293–311.10.1016/j.jaccao.2020.05.009CrossRefGoogle ScholarPubMed
Ohe, T, Aihara, N, Kamakura, S, Kurita, T, Shimizu, W, Shimomura, K. Long-term outcome of verapamil-sensitive sustained left ventricular tachycardia in patients without structural heart disease. J Am Coll Cardiol 1995; 25: 54–58.10.1016/0735-1097(94)00324-JCrossRefGoogle ScholarPubMed
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Elliott, GB, Bargeron, LM Jr, Soto, B, Bream, PR. The monster Purkinje-cell nature of so-called congenital rhabdomyoma of heart. Am Heart J 1962; 63: 194–201.10.1016/0002-8703(62)90007-8CrossRefGoogle ScholarPubMed
Figure 0

Figure 1. Figure 1 long description.Clinical findings. Twelve-lead electrocardiogram demonstrating fascicular ventricular tachycardia and imaging showing a residual cardiac rhabdomyoma adjacent to the posterior papillary muscle. APM = anterior papillary muscle, PPM = posterior papillary muscle, red arrow: tumour.

Figure 1

Figure 2. Electrophysiological findings and ablation. Left ventricular electrograms were recorded using a 10-pole electrode catheter positioned in the left ventricle through a retrograde transaortic approach. Purkinje potentials (P1) preceding ventricular activation with a proximal-to-distal (basal to apical) sequence along the left ventricular septal region (a). Entrainment pacing from the right ventricular apex demonstrating constant fusion at a pacing cycle length (PCL) of 350 ms and progressive fusion at 340 ms (b). Electroanatomical mapping localised the critical region adjacent to the residual tumour. Radiofrequency ablation at the basal tumour-adjacent site terminated the ventricular tachycardia (VT) and rendered it noninducible (c–e). Proposed mechanism of tumour-anchored Purkinje reentry (f). ABL = ablation catheter, LV = left ventricle, PPM = posterior papillary muscle, RA = right atrium, RAO = right anterior oblique, RV = right ventricle, red arrow: tumour.