Permanent Junctional Reciprocating Tachycardia (PJRT) in Children
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. The child may constantly be in and out of tachycardia. Consequently, the heart muscle can become tired and function more poorly, which is called tachycardia-induced cardiomyopathy. This is a reversible problem that can normalize once the tachycardia is treated.
Signs and Symptoms
Unlike other types of SVT during which heart rates rise above 200 bpm, patients with PJRT can often have slower heart rates, making the diagnosis more difficult because symptoms can be milder 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)
- Chest discomfort
- Difficulty breathing
- Exercise intolerance
It is possible for patients that develop tachycardia-induced cardiomyopathy (a poor squeezing function of the main pumping chamber) due to PJRT and 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, these are reversible once the PJRT is controlled.
Your child's doctor or healthcare team may refer you to a pediatric electrophysiologist (EP) or someone specializing 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 are diagnosed. These could include but not limited to:
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 daily.
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 or 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 children who are too young to follow these directions, alternative vagal maneuvers include:
- Placing an ice pack (or a frozen bag of vegetables as an alternative) over the eyes for 15-30 seconds. Avoid placing anything around the infant’s nose.
- Pressing the infant’s knees to their chest for 15-30 seconds
These maneuvers may briefly terminate the arrhythmia, but in this type of tachycardia, the arrhythmia is likely to recur. If the episode lasts a long time, your child is ill or has fainted, or you do not feel comfortable doing these maneuvers at home, call your doctor or go to an emergency room. If your child appears very ill, call 911. Your child may receive an intravenous (IV) medication in the emergency room 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 your child in a normal rhythm.
Typically, children and adolescents with PJRT do not have activity restrictions. However, understanding what exercise or strenuous activities trigger your child's SVT will help avoid further episodes. Speak with your child's cardiologist about any activity restrictions.