Introduction
Premature ventricular contractions are the most common arrhythmia in children. They often resolve spontaneously over several years without treatment, unless ventricular tachycardia develops or cardiac function declines due to frequent premature ventricular contractions.Reference Cohen1,Reference Iwamoto, Niimura and Shibata2
In this report, we describe a successful radiofrequency catheter ablation performed in a paediatric patient with cardiac decompensation caused by frequent, drug-refractory, multifocal premature ventricular contractions originating from Purkinje fibres.
Case report
An 11-year-old boy with trisomy 21 presented to our hospital with frequent premature ventricular contractions. At 2 months of age, the patient underwent intracardiac repair of a ventricular septal defect and patent ductus arteriosus. A 12-lead electrocardiogram showed frequent premature ventricular contractions with right bundle branch block-type and left bundle branch block-type QRS morphologies during sinus rhythm (Figure 1 a ). Right bundle branch block-type premature ventricular contractions were predominant. Echocardiography showed reduced cardiac function (left ventricular diastolic diameter, 51.1 mm; left ventricular ejection fraction, 44.9%). A Holter electrocardiogram revealed premature ventricular contractions throughout the day, with a higher frequency during daytime hours than at night. The total number of premature ventricular contractions was 28,325 over 24 hours. The premature ventricular contractions were polymorphic, with both wide and relatively narrow QRS morphologies, and four different premature ventricular contraction patterns were recorded. The total premature ventricular contraction burden was 22.5%, with 93.6% originating from the left ventricle and 6.4% from the right ventricle. Premature ventricular contractions were detected persistently throughout the 24-hour recording period (Figure 1 b ). After medical therapy, the premature ventricular contractions decreased but did not disappear completely. Echocardiography showed a gradual decline in cardiac function (left ventricular ejection fraction, 37.4%), and exercise tolerance decreased in daily life. One year and eight months after initiation of medical therapy, an electrophysiological study and radiofrequency catheter ablation were performed under general anaesthesia. At the time of electrophysiological study, the patient was 149.6 cm tall and weighed 52.6 kg. An 8.5 Fr long sheath (SL-0) and a bidirectional deflectable long sheath (CARTO VIZIGO, Biosense Webster Inc.) were inserted through the right femoral vein. A geometry of the left ventricle and papillary muscles was created using intracardiac echocardiography (SOUNDSTAR ultrasound catheter, Biosense Webster Inc.). A transseptal puncture was then performed. Left ventricle mapping with a multi-electrode mapping catheter (OPTRELLTM mapping catheter, Biosense Webster Inc.) demonstrated that the earliest activation site of the premature ventricular contractions was located in the posterolateral left ventricle wall between the anterior and posterior papillary muscles. Purkinje potentials preceding the QRS complexes during premature ventricular contractions were recorded around the earliest activation site (area size, 1.8 cm2) (Figure 2 a – d ). At the earliest activation site identified using the OPTRELL catheter, a 3.5-mm open-irrigated tip catheter (ThermoCool SmartTouch SF, Biosense Webster Inc.) recorded an abnormal Purkinje potential that preceded the QRS onset by 21 ms during premature ventricular contractions and was buried within the QRS complex during sinus rhythm (Figure 2 e , f ). The PASO score for pace mapping at the same site was 94, and the QRS morphology was consistent with the most frequent clinical premature ventricular contraction. Intracardiac electrogram revealed a sharp Purkinje potential preceding the QRS during the premature ventricular contraction. Since the premature ventricular contractions occurred spontaneously and could not be induced or terminated by programmed ventricular stimulations, abnormal automaticity originating from the Purkinje fibres was suspected. Consequently, radiofrequency catheter ablation was targeted at the site of the Purkinje potential showing the earliest activation site. During radiofrequency catheter ablation at this site, repetitive ventricular responses of the clinical premature ventricular contraction appeared and disappeared during RF application (40 W, 60 s, contact force 6–7 g, ablation index 605) (Figure 2 g ). The premature ventricular contraction morphology slightly changed after the initial RF application. Therefore, additional RF applications were delivered at sites where Purkinje potentials were recorded around the earliest activation site identified by 3D mapping (Figure 2 h ). Repetitive ventricular responses appeared, and the clinical premature ventricular contractions were eliminated during these RF applications. After the seventh RF application, the area with recorded abnormal Purkinje potentials was ablated, and the repetitive ventricular responses no longer appeared. Following this, right bundle branch block-type premature ventricular contractions were completely eliminated. No procedure-related complications occurred.
Electrocardiogram findings. ( a ) A 12-lead ECG showing five types of PVCs (PVC1-5) with slightly varied RBBB-pattern QRS morphology, including both wide and narrow QRS. ( b ) Holter ECG demonstrating multifocal frequent PVCs, totalling 28,325 beats per 24 hours with a burden of 22.5%. The majority consisted of RBBB-pattern QRS morphology and appeared to approximate the PVC1, PVC2, and PVC5 morphologies on the 12-lead ECG. ECG = electrocardiogram; PVCs = premature ventricular contraction; RBBB = right bundle branch block.

Figure 1. Long description
Panel A: The image shows a 12-lead electrocardiogram (ECG) with six columns labeled SR, PVC 1, PVC 2, PVC 3, PVC 4, and PVC 5. Each column contains multiple rows of ECG traces. The SR column shows normal sinus rhythm, while the PVC columns display five types of premature ventricular contractions with varied right bundle branch block (RBBB)-pattern QRS morphology, including both wide and narrow QRS complexes. Panel B: The image consists of two parts. The left part shows a Holter ECG with three columns labeled PVC 1, PVC 2, and PVC 5, each containing three rows of ECG traces. The right part features three vertical bar graphs representing the frequency of PVCs over time. The x-axis of each bar graph indicates time in hours, and the y-axis shows the number of beats on a logarithmic scale. The graphs display the total number of PVC beats per 24 hours, with PVC 1 having 14803 beats (52.3 percent), PVC 2 having 11722 beats (41.3 percent), and PVC 5 having 1798 beats (6.4 percent). The bar graphs illustrate the distribution and frequency of different types of PVCs over a 24-hour period.
High-density three-dimensional mapping, catheter position, intracardiac electrogram at the successful ablation site, and radiofrequency application at the earliest activation site. ( a ) Fluoroscopic image of catheter positions: upper panel, RAO 30°; lower panel, LAO 60°. ( b ) 3D mapping and intracardiac electrogram showing abnormal Purkinje potentials during PVCs recorded by the OPTRELL catheter. ( c ) Electroanatomical map showing abnormal Purkinje potentials at the earliest activation site. ( d ) Area of abnormal Purkinje potentials, measuring 1.8 cm2. ( e ) Fluoroscopic image of catheter position. ( f ) Intracardiac electrogram. ( g ) Intracardiac electrogram during RF application. ( h ). RF applications shown on the electroanatomical map. Blue tag indicates a good pace-mapping site (PASO score 94). Pink tag indicates ablation site. Yellow tag indicates abnormal Purkinje potentials during PVCs. ABL = ablation catheter; APM = anterior papillary muscle; LAO = left anterior oblique; PPM = posterior papillary muscle; RV = right ventricle; RAO = right anterior oblique; PVC = premature ventricular contraction. Arrow indicates abnormal Purkinje potential during RF application.

One month after radiofrequency catheter ablation, the Holter electrocardiogram demonstrated that premature ventricular contractions were reduced to 7 beats per 24 hours, and the right bundle branch block-type premature ventricular contractions targeted during radiofrequency catheter ablation were no longer present. Echocardiography showed improvement in cardiac function, with a marked increase in exercise tolerance. Based on the clinical course, the final diagnosis was premature ventricular contraction-induced cardiomyopathy secondary to frequent multifocal premature ventricular contractions.
Discussion
Radiofrequency catheter ablation was performed, successfully eliminating polymorphic left ventricle premature ventricular contractions originating from Purkinje fibres, guided by abnormal Purkinje potentials identified using high-density three-dimensional mapping. All premature ventricular contractions were eliminated at the earliest activation site and the surrounding area where abnormal Purkinje potentials were recorded during premature ventricular contractions.
Idiopathic left ventricular arrhythmias often originate from Purkinje fibres. These ventricular arrhythmias can be either reentrant or focal.Reference Nogami, Komatsu and Talib3 Reentrant tachycardias are typically verapamil-sensitive, whereas focal ventricular arrhythmias are often refractory to multiple drugs. In the present patient, multifocal premature ventricular contractions without sustained ventricular tachycardia were repeatedly detected on Holter electrocardiogram monitoring. An electrophysiological study demonstrated that the premature ventricular contractions occurred spontaneously and could not be induced or terminated by programmed ventricular stimulations. Suspecting that the mechanism of these multiple premature ventricular contractions was abnormal automaticity originating from the Purkinje fibres, RF applications were performed at the earliest activation site and the adjacent localised area where abnormal Purkinje potentials were recorded.
Multifocal His-Purkinje system ventricular arrhythmias can occur and often present with preferential conduction from proximal-to-distal breakout sites within the His-Purkinje system, accompanied by abnormal Purkinje potentials and/or altered conduction properties, as previously reported by Itoh et al.Reference Itoh and Yamada4 In this patient, ripple mapping demonstrated proximal-to-distal conduction within the area where abnormal Purkinje potentials were recorded. The premature ventricular contraction morphology changed after the initial RF application, and all right bundle branch block-type premature ventricular contractions were completely eliminated following RF applications targeting the small area with recorded abnormal Purkinje potentials. After radiofrequency catheter ablation, the Holter electrocardiogram showed only 7–8 beats over 24 hours, consisting of left bundle branch block-type premature ventricular contractions. The reduction in left bundle branch block-type premature ventricular contractions was considered secondary to the elimination of right bundle branch block-type premature ventricular contractions, which had been inducing the left bundle branch block-type premature ventricular contractions. Even when a patient presents with multifocal premature ventricular contractions on a 12-lead electrocardiogram, a radical cure with radiofrequency catheter ablation targeting a limited area may be possible if the QRS morphology changes are minimal. The abnormal Purkinje potential detected by the multipolar mapping catheter appeared to be a reliable indicator of successful radiofrequency catheter ablation.
In children, premature ventricular contractions are often detected during school-based electrocardiogram screening and are commonly of outflow tract origin. These premature ventricular contractions are generally benign and may spontaneously disappear within a few years.Reference Iwamoto, Niimura and Shibata2 Blaye-Felice et al. reported that the absence of palpitations, high premature ventricular contraction burden, and epicardial origin are risk factors for premature ventricular contraction-induced cardiomyopathy.Reference Blaye-Felice, Hamon and Sacher5 Baser et al. reported that premature ventricular contraction recurrence can lead to recurrence of cardiomyopathy, and follow-up is important in patients who are symptomatic from premature ventricular contractions and have polymorphic premature ventricular contractions.Reference Baser, Bas and LaBounty6 In this patient, premature ventricular contractions were high burden, drug-refractory, and polymorphic, resulting in premature ventricular contraction-induced cardiomyopathy. Although frequent premature ventricular contractions with left ventricle dysfunction are rare in children, radiofrequency catheter ablation is an effective treatment for premature ventricular contraction-induced cardiomyopathy that is refractory to medical therapy.
In conclusion, all polymorphic premature ventricular contractions originating from left ventricle Purkinje fibres were successfully eliminated by radiofrequency catheter ablation at the earliest activation site and at surrounding sites where abnormal Purkinje potentials were recorded during premature ventricular contractions. In patients with polymorphic premature ventricular contractions originating from left ventricle Purkinje fibres, abnormal Purkinje potentials detected by a multi-electrode mapping catheter may serve as a reliable indicator of effective radiofrequency catheter ablation.
Acknowledgements
The authors would like to thank Enago (www.enago.jp) for the English language review.
Financial support
This research received no specific grant from any funding agency, commercial, or not-for-profit sectors.
Competing interests
The authors declare no conflict of interest.
Consent statement
This study was approved by our institutional ethics committee, and written informed consent was obtained from the parents of the patients.
Declaration of generative AI and AI-assisted technologies in the writing process
During the preparation of this work, the author used DeepL to translate the manuscript.