There’s an Arrhythmia in Your Family—Do Your Children Have It, Too?

The Family Inherited Arrhythmia Program is designed for families like Jagrosse’s. The concept is unique in that an entire family (which can include parents, children, grandparents, and even extended relatives like aunts, uncles, and cousins) is seen during the same appointment. This makes sense for many reasons, Dr. Lampert explains. Because disorders like LQTS often run in families and can be dangerous, it’s important to know which family members are affected by them.

The program, which sees patients at the Yale New Haven Heart and Vascular Center office in North Haven, Conn., and opened in 2022, offers genetic and other needed testing. When multiple cases in a family are suspected or confirmed, the family meets with Drs. Beach or Vinocur and Dr. Lampert at the same time and have all their questions answered. This is also helpful for the physicians. “Dr. Lampert and I can get the story from multiple people at once; we can combine our efforts to determine the best diagnostic and treatment strategies for the whole family,” Dr. Beach says.

This can really benefit the family, too, Dr. Beach adds. “They see how we approach the condition, and they receive information in a streamlined way. If a mother has a question about her medication, for example, we can talk about how that same medication may or may not help her child with the same condition. The pediatric electrophysiologist is there to address those questions immediately and directly,” she says.

Furthermore, many of these arrhythmia conditions are complicated. “It can be confusing to hear different explanations from different providers. When we’re all in the same room, we can have a discussion and make sure everyone is on the same page about our thoughts and plans,” Dr. Beach says.

Families can be referred to the program by their providers. They can also call and make an appointment themselves, without having a referral.

Ultimately, the goal is to prevent sudden cardiac death, Dr. Lampert explains. “That’s why we are often evaluating asymptomatic people. Many of these genetic arrhythmia diseases carry the risk of sudden death. If we know what someone has, we can use preventive measures like medications or an at-home device like an AED,” she says.

In rare cases, an implanted defibrillator may be needed or, in some cases, medications, she adds.

Dr. Lampert prescribed a beta blocker medication called propranolol, which can reduce the risk of arrhythmic events, for Jagrosse. The medication, taken as a pill, manages her condition well. “I haven’t had any feeling of fainting or lightheadedness at all since I started it,” Jagrosse says.

When Jagrosse’s son Isaac was born in 2020, he and his 10-year-old brother, Dante, were given a genetic test (via cheek swab) for LQTS. Both boys tested positive for it. The brothers were both prescribed a beta blocker, just as their mother was. Due to his young age, Isaac was initially seen by Dr. Beach for a checkup with an ECG every six months. Now, he, Dante, and their mother go for their family appointment once a year.

“It was hard to find out that my two boys have it, too. I almost felt guilty, even though I know it’s something I couldn’t help. I’m just glad it can be managed,” Jagrosse says.

Jagrosse also has a 2-year-old, Gia, who was tested for the LQTS gene but does not have it. “In this situation, if you don’t have the gene variant that runs in the family, then we don’t need to be concerned for LQTS,” Dr. Lampert explains. “But if you do, we need to embark on a clinical evaluation to see how much it is impacting your heart.”

Jagrosse says the family-centric approach suits her well. “I really like the setup. They take us to the same room, and they come and talk to us at the same time. I like both doctors a lot, and it’s nice to get all the information we need at the same time,” Jagrosse says. “I work as a full-time technician at a doctor’s office, so it’s easier for me to schedule things this way, too.”

While both boys are being treated effectively, it’s still important that they be monitored regularly, Jagrosse says. “As they get older, we have to keep an eye on them. They don’t have a lot of restrictions, as long as they aren’t doing anything risky like skydiving,” she says.

“Exercise can be low risk for people with LQTS if their condition is well-managed,” Dr. Lampert explains. “But it’s not that the risk is zero either, so you don’t want to put yourself in situations, like open-water swimming, where you could die if something happened with your heart.”

Furthermore, LQTS can manifest differently between people in the same family, even when they carry the same genetic mutation, she adds. “It’s really important that we continue to follow them clinically because they all may develop differently,” she says.