Syncope due to acute rheumatic fever with pacemaker-like syndrome

Abstract

The prolongation in the PR interval on the electrocardiogram is one of the minor criteria of Jones. Abnormal increases in the PR interval lead to hemodynamic impairments caused by atrioventricular asynchrony and is called pseudo-pacemaker or pacemaker-like syndrome in the literature. A 13-yearold boy who had polyarthralgia for six weeks was referred to the emergency room because of syncope while exerting effort. In the electrocardiography, the P wave was regularly seen at the onset of the T wave immediately after the QRS wave with extremely prolonged PR interval and mild tachycardia. Also, 24 hours rhythm Holter recording showed atrioventricular dissociation. The echocardiography revealed findings of severe carditis. Diagnosis of acute rheumatic fever accompanying pacemaker-like syndrome was made. Although the recommendation for marked first-degree atrioventricular block that causes hemodynamic impairment is pacemaker implantation, a significant improvement in the PR prolongation was observed in the short term with anti-inflammatory treatment because the impairment of conduction in the patient was due to inflammation. The pacemaker-like syndrome in a child is being reported for the first time in the literature.

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

“…As for the presenting symptoms, patients with PPMS typically experienced a variety of symptoms similar to those with heart failure or AV block of a higher degree, with palpitation being the most common presenting symptom, followed by syncope, dyspnea, and the perception of neck pulsation (Table 1). Only one author described a connection between the development of symptoms with prolongation of the PR interval 12 and one documented AV dissociation 13 . As to the charted PR intervals, only seven cases of PPMS with an extremely prolonged PR interval (≥0.50 s) have been reported in the literature so far, and our case displayed the longest PR interval to date associated with this syndrome.…”

“…Apart from our case and the one published by Ogunlade et al 6 . where no clear association between the patient’s past medical history and the presence of PPMS could be found, four etiologies of PPMS were identified in the remaining cases (n. 15), as follows: (1) Idiopathic PPMS related to poor AV nodal conduction (20% of cases); 2,14,16 (2) reversible inflammatory causes (13%) such as acute myocarditis 15 or acute rheumatic fever; 13 (3) cardiac surgery, 20 interventional procedures 11 or chest radiation 17 leading to the permanent damage of the normal AV conduction system (20%), and, finally, (4) PPMS due to manifest dual AV nodal physiology (DAVNP) occurring as a primary finding (27%) 10,12,16,19 or post slow pathway ablation for treatment of AVNRT (20%) 10,18,21 . In fact, in this latter group, two patients (66%) had residual DAVNP due to damage to the fast pathway, either as a complication of slow pathway ablation 21 or deliberate ablation as a treatment option for AVNRT 10 .…”

“…The retrieved 17 cases of PPMS—including our case report—are outlined in Table 1 2,6,10,12–21 . Most (88.2%) were adults with an average age of 47 years ranging from 13 to 86 years old.…”

“…Finally, except for patients with reversable causes of PPMS, 13,15 most were successfully treated by permanent pacemaker implantation 2,10,11,14,17,18,21 including PPMS cases with past medical history of pathway ablation as treatment of AVNRT 10,18,21 . However, patients with PPMS and DAVNP were variably treated with medications such as ivabradine (25%) or slow‐pathway ablation (75%).…”

“…The identification of patients with reversable PPMS—such as infections, radiation, or rheumatologic conditions—is paramount as they are generally young and with the possibility of normalization of the PR either with appropriate treatment of the underlying cause 13 or self‐resolved 15 . In this regard, the development of PPMS has been linked to gastroenteritis in patients with viral infections 2,14 .…”