Pseudo-pacemaker syndrome with prolonged PR interval: A narrative review

Pseudo-pacemaker syndrome (PPMS) is a rare complication of first-degree atrio-ventricular (AV) block in which a very prolonged PR interval causes AV dyssynchrony and subsequent symptoms of hemodynamic instability in the absence of an implanted pacemaker. The aim of this manuscript was to describe a unique case of PPMS and to provide a comprehensive review of the topic to help clinicians in the diagnosis and management of this condition. Through systematic research on PubMed, Google Scholar, EBSCO, and Ovid MEDLINE and using the search strings “pseudo-pacemaker syndrome” and “symptomatic first-degree AV block,” we identified 14 articles accounting for 17 cases of PPMS, including our case report. The most common age group for PPMS was middle-aged and young adults, with an average age of 47 years. Palpitations were the most common presenting symptom and four main etiologies of PPMS were identified, as follows: (1) Idiopathic PPMS with evidence of impaired conduction over the AV node (20% of cases), (2) PPMS associated with reversable inflammatory causes (13%) or (3) associated with iatrogenic surgical or interventional procedures leading to the permanent damage of the normal AV conduction system (20%), and, finally, (4) PPM related to dual AV nodal physiology (DAVNP) as a primary finding (27%) or occurring after fast or slow pathway ablation for treatment of AV nodal re-entrant tachycardia (AVNRT) (20%). Treatment should be patient-tailored and based on the specific etiology once identified. However, the treatment of PPMS due to DAVNP without AVNRT presentation is yet to be clarified.