Cardiovascular

Electrocardiographic and Arrhythmic Manifestations of End-Stage Atrial Myopathy in Patients With Longstanding Persistent or Permanent Atrial Fibrillation: A 3-Patient Case Series.

TL;DR

Patients with longstanding persistent or permanent atrial fibrillation may demonstrate distinct findings suggestive of advanced atrial myopathy, including loss of fibrillatory waves on ECG, low-amplitude atrial tachycardias, atrial standstill, junctional rhythms, absent atrial sensing or capture on device interrogation, and extensive atrial scarring on electrophysiology mapping.

Key Findings

End-stage atrial myopathy can manifest as progression from atrial fibrillation to complete atrial standstill with junctional rhythms.

  • Two of the three reported patients demonstrated progression from AF to atrial standstill with junctional rhythms.
  • Serial ECGs in both patients showed loss of fibrillatory activity over time.
  • Device interrogation in both patients demonstrated absent atrial sensing and capture.
  • This progression represents a severe end-stage manifestation of atrial electrical dysfunction.

Extensive biatrial scarring from advanced atrial myopathy can result in recurrent low-amplitude atrial tachycardias following AF ablation.

  • One patient with extensive biatrial scar developed recurrent low-amplitude atrial tachycardias after AF ablation.
  • The low amplitude of the tachycardias was attributed to the extensive scarring of atrial tissue.
  • This finding illustrates how advanced atrial remodeling can complicate post-ablation arrhythmia management.

Absent atrial sensing or capture can inform device implantation strategy, including omission of atrial lead placement.

  • One patient underwent pacemaker implantation without atrial lead placement due to absence of atrial sensing or capture on electrophysiology mapping.
  • Device interrogation findings of absent atrial sensing or capture were consistent with electrophysiology mapping findings of extensive atrial scarring.
  • This case suggests that pre-implantation electrophysiological assessment may guide pacing strategy in end-stage atrial myopathy.

Several coexisting cardiac conditions were identified as important substrates contributing to atrial remodeling in these patients.

  • Coexisting substrates for atrial remodeling included hypertrophic cardiomyopathy, mitral valve disease, and prior cardiac surgeries.
  • All three patients had longstanding persistent or permanent atrial fibrillation as a common feature.
  • These comorbidities likely contributed to progressive atrial fibrosis and electrical dysfunction.

The clinical manifestations of end-stage atrial myopathy form a recognizable constellation of electrocardiographic and electrophysiologic findings.

  • Identified manifestations include loss of fibrillatory waves on ECG, low-amplitude atrial tachycardias, atrial standstill, junctional rhythms, absent atrial sensing or capture on device interrogation, and extensive atrial scarring on electrophysiology mapping.
  • Advanced atrial remodeling is often associated with atrial fibrosis and severe atrial electrical dysfunction.
  • Recognition of these findings may potentially inform AF ablation and atrial pacing strategies.
  • The authors note that data on clinical features of this process are limited and further studies are needed to define mechanisms, prevalence, and clinical significance.

What This Means

This research describes three patients whose hearts showed signs of severe, end-stage damage to the upper chambers (atria), a condition the authors call 'end-stage atrial myopathy.' All three patients had long-standing atrial fibrillation (AF), an irregular heart rhythm, and all showed evidence of extensive scarring in the atrial tissue. In two patients, the heart's upper chambers stopped generating any electrical activity at all — a condition called atrial standstill — and the heart's rhythm was taken over by a backup pacemaker region called the junction. In the third patient, ablation (a procedure to treat AF by destroying problematic heart tissue) led to a different problem: very low-amplitude irregular rhythms that were difficult to detect and treat. This research suggests that in some patients with long-standing AF, the atria can become so scarred and electrically silent that they no longer function as a meaningful part of the heart's electrical system. This showed up on routine heart tracings (ECGs) as a loss of the characteristic irregular waves of AF, and on pacemaker device checks as an inability to sense or stimulate the atrial tissue. In one patient, this information was used to make a clinical decision not to place an atrial lead when implanting a pacemaker, since the atrial tissue was non-functional. This research suggests that clinicians should be aware of this constellation of findings — loss of AF waves on ECG, junctional rhythms, and absent atrial electrical responses — as potential markers of advanced atrial disease. Recognizing these signs could help guide decisions about heart rhythm procedures like ablation and device implantation. The authors call for further research to understand how common this condition is and what its long-term implications are for patients.

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Citation

Serpa F, Patel N, Joglar J. (2026). Electrocardiographic and Arrhythmic Manifestations of End-Stage Atrial Myopathy in Patients With Longstanding Persistent or Permanent Atrial Fibrillation: A 3-Patient Case Series.. The American journal of case reports. https://doi.org/10.12659/AJCR.953661