What is the treatment for Permanent Junctional Reentrant Tachycardia (PJRT)?

Treatment of Permanent Junctional Reciprocating Tachycardia (PJRT)

Catheter ablation of the concealed accessory pathway is the definitive treatment for PJRT, with a success rate of approximately 90% and should be considered first-line therapy, particularly in symptomatic patients or those with tachycardia-induced cardiomyopathy. 1

Understanding PJRT

PJRT is a rare form of atrioventricular reentrant tachycardia (AVRT) characterized by:

• Retrograde conduction through a slowly conducting, decremental accessory pathway, typically located in the posteroseptal region (59-76% of cases) 2, 3, 4
• Narrow QRS complex tachycardia with a long RP interval and inverted P waves in inferior leads 5
• Incessant or near-incessant nature in many patients (47-69% have tachycardia present >50% of the time on Holter monitoring) 2, 4
• Risk of tachycardia-induced cardiomyopathy (TIC) in 16-28% of patients if left untreated 2, 4

Treatment Algorithm

First-Line: Catheter Ablation

Catheter ablation is recommended as definitive therapy for PJRT with the following outcomes:

• Success rate: 90-98.4% after accounting for repeat procedures 1, 3, 4
• Single procedure success: 90-94% 3, 4
• Long-term success: 100% with repeat ablation if needed 3, 4
• Major complication risk: 3%, including a 5-10% risk of AV block requiring permanent pacing 1, 6

Complete reversal of tachycardia-induced cardiomyopathy occurs in all patients after successful ablation 2, 3, 4

Ablation should be prioritized in:

• Patients with frequent or incessant tachycardia 2
• Those with impaired left ventricular function or TIC 2, 5, 4
• Symptomatic patients of any age 2, 4
• Patients who have failed medical therapy 7, 2

Medical Therapy (When Ablation Not Feasible or Preferred)

Medical therapy is less effective but reasonable in specific circumstances:

First-Line Medical Options:

Oral beta blockers, diltiazem, or verapamil are indicated for ongoing management in patients without pre-excitation on resting ECG (Class I recommendation):

• Effective in approximately 50% of patients with concealed accessory pathways 1
• Favorable side effect profile 1
• Best suited for: infants and young children as initial therapy, or patients with infrequent episodes 2

Second-Line Medical Options:

Oral flecainide or propafenone (Class IIa recommendation):

• Reasonable for patients without structural heart disease or ischemic heart disease who are not candidates for or prefer not to undergo catheter ablation 1
• Effective in approximately 85-90% of patients, with 30% reporting complete absence of tachycardia 1
• Contraindicated in structural heart disease due to increased risk of proarrhythmic VT 1
• Side effects occur in up to 60% of patients; 20% discontinue due to adverse effects 1

Third-Line Medical Options:

Oral dofetilide or sotalol (Class IIa recommendation):

• May be reasonable for patients not candidates for ablation 1
• Can be used in structural heart disease unlike flecainide/propafenone 1
• Requires inpatient monitoring with serial ECGs due to risk of QT prolongation and torsades de pointes 1

Oral amiodarone (Class IIb recommendation):

• May be considered when other agents are ineffective or contraindicated 1
• Reserved as third-line therapy due to significant long-term toxicity 1

Age-Specific Considerations

Infants and Young Children:

• Medical therapy recommended as first option in infancy and early childhood 2
• Patients with slow tachycardia rate and infrequent episodes may not need therapy 2
• Ablation reserved for: medically refractory cases, TIC, or before surgery limiting future catheter access 1

Older Children and Adults:

• Catheter ablation is first-line therapy in symptomatic patients 2, 4
• PJRT in adults is often paroxysmal (53%) rather than incessant 4
• Accessory pathways in adults may be in atypical (non-posteroseptal) locations in 24% of cases 4

Critical Pitfalls to Avoid

Do NOT use AV nodal blocking agents in pre-excited atrial fibrillation:

• Intravenous digoxin, amiodarone, beta blockers, diltiazem, or verapamil are potentially harmful (Class III) in pre-excited AF 1
• These medications may enhance accessory pathway conduction, increase ventricular rate, and provoke life-threatening ventricular arrhythmias 1

Monitor for tachycardia-induced cardiomyopathy:

• Longer symptom duration correlates with higher risk of TIC 7, 2
• Patients with depressed ejection fraction had significantly longer symptom duration (258 vs 64.5 months) 7
• All cases of TIC are reversible after successful ablation 7, 2, 3, 4

Recognize that PJRT may present with variable clinical severity:

• Some patients have infrequent episodes requiring no therapy 2
• Others have incessant tachycardia leading to heart failure 2, 5
• Correlation exists between symptom duration and tachycardia rate 4