Coroner finds heart condition caused girl’s death

SAN MARCOS — A coroner determined an undetected heart condition known as Long QT Syndrome as the probable cause of death for a 14-year-old girl who died suddenly during her eighth-grade dance at Woodland Park Middle School on June 9.
Sabrina Keller had been dancing with friends as they celebrated promotion into high school when she sat down, slumped over, suffered cardiac arrest and died later that night at Rady Children’s Hospital.
“We believe the cause was a heart condition,” said Deputy Medical Examiner Christopher Swalwell, M.D., of the San Diego County Coroner’s Office.
Sabrina’s autopsy was released by the coroner’s office on Nov. 14 after extensive toxicology and family history screening, and noted that Sabrina’s mother, Jill, had been diagnosed with the heart condition Long QT Syndrome just months after her daughter’s death.
“My wife’s showed up immediately following the EKG,” said Brian Keller, Sabrina’s father.
Labor Day almost turned into a second tragedy for the Keller family after Jill Keller left a family holiday dinner early because she wasn’t feeling well, and ended up requiring surgery for a defibrillator implant, Brian Keller said.
Her heart rate clocked more than 280 beats per minute and she went into cardiac arrest that required shock (using an automated external defibrillator, or AED) to bring her back, he said.
“I thought I was losing her but they knew exactly what to do,” he said of the staff at Palomar Medical Center.
Brian Keller said his wife’s congenital Long QT Syndrome actually presented itself in a 2006 EKG, but went undiagnosed until a certain medication administered during the Sept. 4 hospital visit triggered a spike in her heart rate, which is another symptom of the heart condition.
“I’ve never heard anything about it until Sabrina passed away. If someone would have said something to us, we would have had our kids tested,” he said.
The Sudden Arrhythmia Death Syndromes Foundation, or SADS, contacted the Keller family once they learned of Sabrina’s autopsy results through a Google-alert.
“We’re here to support families, save lives, educate physicians and advocate change,” said Laura Wall, vice president of marketing for the foundation.
One of the changes SADS Foundation is aiming for is on a federal level, and would require that all students learn CPR and how to use an AED before they graduate high school, she said.
“We lose 4,000 kids a year to this but if we can diagnose, we can treat,” Wall said.
Warning signs of the disease are estimated to have been present in at least half of the yearly deaths, according to SADS Foundation.
Wall said that fainting during exercise, or becoming startled during an activity is one of the signs, another is if there has been an unexplained death of a child in the family.
Brian Keller said that about a week before Sabrina passed away, she had told him that she felt like she was going to black out while running during PE at school.
Long QT Syndrome has been around for 50 years and is a potentially lethal but highly treatable condition, according to the president of SADS Foundation, Mike Ackerman, M.D., Ph.D.
He co-authored clinical guidelines on genetic testing for Long QT Syndrome in “The Heart Rhythm Journal” published in August 2011.
He stressed the importance of genetic testing for Jill Keller to determine whether Sabrina’s cause of death can be positively attributed to Long QT Syndrome.
“Genetic testing is clearly indicated for the decedent’s mother for Long QT Syndrome,” he said.
If Jill Keller’s mutation tests positive, they can easily test Sabrina’s sample, he said.
Swalwell said in Sabrina’s case, the coroner’s office did extensive drug testing and expensive genetic testing because of her age and the lack of other causes of death or factors.
Her genetic testing for cardiac channelopathies did not detect any deleterious mutations for the genes tested, but the testing only identifies about 65 percent of people with Long QT Syndrome, he said.
The toxicology report showed that Sabrina had marijuana in her system, but no other drugs or alcohol.
Swalwell said he doesn’t know of a connection between smoking marijuana and heart disease.
“I don’t think it had anything to do with her death,” he said.
Brian Keller said his daughter was a Girl Scout and was working toward her Gold status, which is comparable to a Boy Scout who becomes an Eagle Scout.
He said Sabrina didn’t use drugs or alcohol and learned from a friend of Sabrina’s that the night of the dance was her first time experimenting with marijuana.
Ackerman said there is no good data to support that marijuana can cause a fatal heart rhythm known as ventricular fibrillation.
According to the autopsy report, Sabrina was initially in ventricular fibrillation when paramedics arrived at the school, and resuscitative efforts continued during the hospital transport but were unsuccessful.
Brian Keller said that even though Sabrina’s testing was negative for the responsible Long QT Syndrome gene, the family is awaiting further testing on his wife.
“We have quite a few people that need to be tested when we figure out what gene to test for,” he said.
The testing is expensive, he said, and it is more affordable to find the responsible gene in his wife’s makeup and then test the family for matches of that gene, which can be indicative of a heart condition.
The Sabrina Keller Foundation was set up by a close friend of the Keller family, and a 5K run is planned for April 15 to raise scholarship money for qualified Girl Scouts and to raise awareness of Long QT Syndrome.
“She really was just an all-American girl,” said Darcy Rouse, founder of the Sabrina Keller Foundation.
“It didn’t matter, she was everybody’s friend. If somebody had a bad day, she made it better. She set goals. She met them, exceeded them,” she said.

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