Kansas City, KS - A devastating family tragedy led to a discovery that may have saved multiple lives. After the sudden death of Jamie Furey’s teenage cousin, genetic testing revealed an inherited heart condition affecting at least seven members of her family. Now, a newer type of implantable defibrillator is giving Jamie protection from dangerous heart rhythms and new confidence to plan for the future she’s always wanted.
Jamie Furey, Patient
- Jamie was diagnosed with long QT syndrome at age four after the sudden death of her 14-year-old cousin prompted her family to look more closely at its history of unexpected cardiac deaths.
- Jamie’s family's long QT syndrome follows a dominant inheritance pattern, meaning an affected parent has a 50% chance with each pregnancy of passing the genetic mutation to a child.
- She received her first implanted heart device at age seven and says she has undergone so many procedures since then that she has lost track.
- After years of changing doctors, Jamie and her family sought another opinion at The University of Kansas Health System, where she began seeing electrophysiologist Dr. Seth Sheldon.
- In 2025, Jamie received an EV-ICD, a newer type of extravascular defibrillator with a lead that sits outside the heart and veins.
Dr. Seth Sheldon, Electrophysiologist
- Dr. Sheldon explains long QT syndrome and how the inherited condition can cause dangerous heart rhythms that may lead to sudden cardiac arrest.
- He explains why Jamie's history of multiple devices and procedures made the EV-ICD an option for her and how it differs from a traditional implantable defibrillator.
- Dr. Sheldon explains how the device identifies a dangerous heart rhythm and what it does when one occurs.
- He also treats Jamie's father, Bernie, who received a defibrillator shortly after Dr. Sheldon evaluated him during one of Jamie's appointments.
- Dr. Sheldon discusses how treatment for inherited heart conditions can go beyond preventing cardiac arrest to helping young patients safely plan for their futures.
Farah Ammouri, Genetic Cardiovascular Counselor, The University of Kansas Health System
- Farah explains why an unexpected cardiac death in a young person can be an important reason to investigate whether an inherited heart condition is present in the family.
- Jamie's extended family history included the sudden deaths of her teenage cousin and her father's 15-year-old sister, eventually prompting family members to undergo testing.
- At least seven members of Jamie's family, including Jamie, both of her brothers and her father, have been diagnosed with long QT syndrome.
- Farah explains how inherited heart conditions can move through generations and who else in a family may need genetic testing after one person is diagnosed.
- She explains what genetic testing involves and how counselors help families process results that may have implications for parents, siblings, children and future generations.
Cheryl Hunter, Clinical Nurse Coordinator, The University of Kansas Health System
- Cheryl explains what day-to-day management of an inherited heart rhythm disorder can look like for patients such as Jamie.
- She discusses what patients need to know about living with an implanted defibrillator, including potential limitations and long-term considerations.
- Hunter explains how the care team helps patients manage the fear that can come with knowing they are at risk for a potentially dangerous heart rhythm.
- She discusses how ongoing support can help patients move beyond simply preventing a cardiac event and begin focusing again on everyday life and future goals.
Bernie and Kristi Furey, Jamie's Parents
- Bernie and Kristi describe the fear of learning their four-year-old daughter had the same inherited heart condition connected to sudden deaths within their family.
- The diagnosis ultimately extended beyond Jamie, with both of her brothers and Bernie among the family members diagnosed with long QT syndrome.
- Bernie became Dr. Sheldon's patient after jokingly asking him to check his heart during one of Jamie's appointments; after evaluating him, Dr. Sheldon scheduled Bernie to receive a defibrillator the following week.
- Kristi shares what it means to hear Jamie, after years of procedures and uncertainty, now talking confidently about buying a home and potentially starting a family.
