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<p>PRKAG2 syndrome is an autosomal dominant cardiac glycogenosis caused by mutations in the PRKAG2 gene, clinically characterized by the triad of ventricular hypertrophy, conduction disease, and ventricular pre-excitation, along with a high propensity for supraventricular arrhythmias. This report describes a young male carrier of the R302Q mutation who developed wide QRS tachycardia due to atrial flutter with 1:1 conduction via an accessory pathway. Electrophysiological study revealed atrial flutter activation in a counterclockwise direction around the tricuspid annulus, coexisting with a left-sided accessory pathway. Following radiofrequency ablation, the patient’s left ventricular ejection fraction markedly improved from 16% to 60%. Furthermore, 68Ga-FAPI PET/CT imaging, performed for the first time in this disease, revealed enhanced fibroblast activity in the left ventricular endocardial and mid-myocardial layers, consistent with the distribution of late gadolinium enhancement on cardiac magnetic resonance but extending beyond it. This finding indicates that FAPI PET/CT detects active myocardial remodeling before irreversible fibrosis develops. Thus, ⁶⁸Ga‑FAPI PET/CT provides a complementary molecular imaging tool to assess disease activity and potentially guide early intervention in PRKAG2 cardiomyopathy.</p>

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Radiofrequency ablation of atrial flutter with 1:1 accessory pathway conduction improved cardiac function in a patent with PRKAG2 Cardiomyopathy. Insights with 68Ga-FAPI PET/CT imaging

  • 期刊论文

  • 发表日期：

    2026年4月16日

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  • Jiali Fan,
  • Yuzhen Zhang,
  • Jingfen Zhu,
  • Min Dai,
  • Heng Wang,
  • Pengcheng Hu,
  • Tingbo Jiang,
  • Bingyuan Zhou,
  • Changsheng Ma

摘要

PRKAG2 syndrome is an autosomal dominant cardiac glycogenosis caused by mutations in the PRKAG2 gene, clinically characterized by the triad of ventricular hypertrophy, conduction disease, and ventricular pre-excitation, along with a high propensity for supraventricular arrhythmias. This report describes a young male carrier of the R302Q mutation who developed wide QRS tachycardia due to atrial flutter with 1:1 conduction via an accessory pathway. Electrophysiological study revealed atrial flutter activation in a counterclockwise direction around the tricuspid annulus, coexisting with a left-sided accessory pathway. Following radiofrequency ablation, the patient’s left ventricular ejection fraction markedly improved from 16% to 60%. Furthermore, 68Ga-FAPI PET/CT imaging, performed for the first time in this disease, revealed enhanced fibroblast activity in the left ventricular endocardial and mid-myocardial layers, consistent with the distribution of late gadolinium enhancement on cardiac magnetic resonance but extending beyond it. This finding indicates that FAPI PET/CT detects active myocardial remodeling before irreversible fibrosis develops. Thus, ⁶⁸Ga‑FAPI PET/CT provides a complementary molecular imaging tool to assess disease activity and potentially guide early intervention in PRKAG2 cardiomyopathy.