Indian pacing and electrophysiology journal
September 17, 2026 · via Europe PMC
Accessory pathways in arrhythmogenic right ventricular cardiomyopathy: A two-case series and literature review.
Leiria TLL, de Lima GG, Baroni AC, Essebag V.
AI summary — for doctors, generated from the abstract only
This case series describes two young patients with a genetic heart muscle disease prone to sudden death who also had extremely rare hidden extra electrical pathways in their hearts, highlighting that recognizing and ablating these pathways is important both to control fast heart rhythms and to prevent inappropriate shocks from their life-saving defibrillators.
What was studied
A two-case series with literature review describing young patients with arrhythmogenic right ventricular cardiomyopathy (a genetic heart muscle disease associated with dangerous arrhythmias and sudden death) who also had concealed accessory electrical pathways (extremely rare in this condition), describing their presentations, diagnostic workup, and treatment with catheter ablation and defibrillator implantation.
Key findings
- Case 1 was a 27-year-old man with a family history of sudden cardiac death who had a supraventricular tachycardia caused by a concealed accessory pathway; after successful ablation, dangerous ventricular tachycardia was still inducible, leading to defibrillator implantation.
- Case 2 was a 22-year-old woman who survived cardiac arrest, found to have right ventricular aneurysms and scarring on MRI, along with a different type of concealed pathway causing a specific tachycardia type; ablation eliminated this tachycardia, and a defibrillator was implanted for ongoing protection.
- Literature review confirmed that while atrial fibrillation and flutter are relatively common in this cardiomyopathy (occurring in about 25% of patients), concealed accessory pathways remain exceptionally rare.
Clinical relevance
This case series highlights that recognizing and ablating concealed accessory pathways in patients with this genetic cardiomyopathy is important for both symptom control and for reducing the risk of inappropriate defibrillator shocks (which could otherwise be triggered by the fast heart rates from the accessory pathway), which could inform clinicians evaluating similar rare, overlapping arrhythmia presentations.
Who should care
Limitations
This is a two-case series combined with literature review rather than a larger systematic study; findings cannot be generalized to establish the true prevalence or standard management approach for this rare combination of conditions.
Doctors describe two rare cases where young patients with a dangerous genetic heart muscle disease also had hidden extra electrical wiring in their hearts, showing why catching and fixing these extra pathways matters both for symptom control and for preventing their defibrillators from firing unnecessarily.
This is an AI-generated summary of the published abstract, not a clinical recommendation. It may not reflect the full text or subsequent updates — verify against the original source before acting on it.
Abstract
Background
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetic cardiomyopathy characterized by fibrofatty replacement of ventricular myocardium, associated with ventricular arrhythmias and sudden cardiac death (SCD). While supraventricular arrhythmias occur in approximately 25% of ARVC patients, the coexistence of accessory pathways is exceptionally rare, particularly concealed pathways without ventricular pre-excitation.
Case presentation
We describe two young patients with ARVC and concealed accessory pathways. Case 1: A 27-year-old male with family history of SCD presented with supraventricular tachycardia mediated by a left lateral retrograde-only accessory pathway. Following successful ablation, sustained ventricular tachycardia (VT) was inducible, prompting implantable cardioverter-defibrillator (ICD) implantation. Case 2: A 22-year-old female survived out-of-hospital cardiac arrest. Cardiac MRI revealed right ventricular aneurysms and late gadolinium enhancement. Electrophysiological study demonstrated a decremental right posterior pathway consistent with permanent junctional reciprocating tachycardia (PJRT). Ablation successfully eliminated the tachycardia, and an ICD was implanted for secondary prevention.
Discussion
Both patients exhibited concealed accessory pathways with exclusive retrograde conduction-one fast, one decremental-without surface ECG pre-excitation. This rare association increases arrhythmic burden and risk of inappropriate ICD therapies. Catheter ablation was essential for eliminating supraventricular tachycardias and preventing ICD complications. Literature review confirms that while atrial fibrillation and flutter are relatively common in ARVC, accessory pathways remain exceptionally rare.
Conclusion
The coexistence of ARVC and concealed accessory pathways is exceedingly rare. Recognition is crucial, as catheter ablation prevents supraventricular arrhythmias and inappropriate ICD therapies, while ICD implantation remains essential for protection against malignant ventricular arrhythmias.
DOI
10.1016/j.ipej.2026.09.003
PMID
42749081
Research metadata (titles, abstracts, authors, publication dates) is sourced from Europe PMC, which aggregates content from PubMed/MEDLINE, PubMed Central, and other providers under the U.S. National Library of Medicine (NLM/NCBI). NLM does not claim copyright on abstracts; the original journal publishers or authors may hold rights to the full articles. AI-generated summaries are derived only from the abstract, never the full text, and are always labeled as AI-generated.
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