Permanent junctional reciprocating tachycardia (PJRT)
Introduction:
Permanent junctional reciprocating tachycardia, more commonly referred to as PJRT, is a rare form of supraventricular tachycardia (SVT), or abnormal fast heartbeats, typically seen in infants and children. This type of SVT can be very incessant or constant. The child may be constantly in and out of tachycardia. Due to this, the heart muscle can become tired and function more poorly, which is called tachycardia-induced cardiomyopathy. This is a reversable problem that can normalize once the tachycardia is treated.
Signs and Symptoms:
Unlike other types of SVT where heart rates can be above 200 bpm, patients with PJRT often can have slower rates, making the diagnosis more difficult because symptoms can be more mild and the patient is not aware of the rhythm abnormality. Symptoms can be similar to those of SVT and include but are not limited to:
- Sensation of rapid, fluttering or pounding heartbeats (palpitations)
- Dizziness
- Chest discomfort
- Difficulty breathing
- Lightheadedness
- Exercise intolerance
- Anxiety
It is possible for patients that develop tachycardia induced cardiomyopathy (poor squeezing function of the main pumping chamber) due to PJRT to experience symptoms of:
- Shortness of breath
- Poor feeding or lethargy in babies, nausea in older children
- Exercise intolerance
- Worsening fatigue
- Swelling of abdomen or lower extremities
Again – this is reversible once the PJRT is controlled.
Diagnosis:
Your doctor or health care team may refer you to a pediatric electrophysiologist (EP) or someone who specializes in children with heart rhythm disorders. Your EP team may use one or multiple tools to help diagnose PJRT similar to how other forms of SVT would be diagnosed. These could include but not limited to:
- ECG
- Holter monitor
- Event monitor
- Exercise test
Treatment:
Your EP team may prescribe medication for treatment. Medication is not a cure but can decrease the number of episodes and help to control symptoms. In most cases, these medications are taken every day.
An electrophysiology study with ablation is a curative procedure for SVT.
Certain maneuvers, called vagal maneuvers, are designed to potentially interrupt the PJRT and slow down the heart rate.
For older children, vagal maneuvers include:
- “Bearing down”: pretending as if you are having a bowel movement - blowing on your thumb
- Doing a headstand, if you already know how to do one.
- Putting very cold water/ice on your face
For babies or young children who can’t follow these directions, vagal maneuvers include:
- Place an ice pack over the eyes for 15-30 sec. A frozen bag of vegetables works really well. Avoid placing anything around the infant’s nose.
- Pressing the infant’s knees to the chest for 15-30 seconds
These maneuvers might be effective at terminating the arrhythmia but in this type of tachycardia the arrhythmia is very likely to recur.
If the episode is lasts a long time, your child is ill or has fainted or you don’t feel comfortable doing these maneuvers at home, you can call your doctor or go to an emergency room. If your child appears very ill, you should call 911. In the emergency room, your child may receive an intravenous (IV) medication to stop the SVT. If the medication is not successful and your child remains ill, a brief electrical shock (cardioversion), may be required. This is usually done while your child is sedated. This is an arrhythmia where a more long-lasting medication may be needed to keep the child in a normal rhythm.
Lifestyle changes:
Typically, children and adolescents with PJRT do not have activity restrictions but understanding what triggers an individual’s SVT like exercise or strenuous activity can help avoid further episodes. You should speak with your child’s cardiology provider about any activity restrictions.
