Rare heart condition cause of teen's death

Elisa Feldman

When Elisa Feldman, 13, died on June 29, 2006, her family and doctors had little clue as to the nature of the disease that killed her.

Elisa, a resident of Simi Valley, had just graduated from Lindero Canyon Middle School in Agoura Hills and faced a bright and promising future.

After months of research, doctors now say the most likely cause of the girl's death was a condition known as Long QT.

The family contacted an agency that supports research and provides information on sudden arrhythmia deaths in children, the Sudden Arrhythmia Death Syndrome Foundation. The foundation stated that, while sudden death without warning is relatively rare in young people, it is often caused by a hidden heart arrhythmia or electrical defect called Long QT Syndrome, believed to be about three times more common than childhood leukemia and kills as many as 2,000 to 3,000 children and young adults in the U.S. each year.

Long QT refers to an interval on the electrical pattern of the heart as seen on an electrocardiogram. The repeating pattern of an ECG is labeled with the points PQRST to indicate from the beginning to the end of the cycle. The time interval between points Q and T is the QT interval, measured in seconds. It represents the part of the heartbeat called repolarization, when the heart muscle recharges itself electrically before the beginning of the next beat.

If the QT interval, after correction for faster or slower pulse rates, is longer than about 0.48 to 0.50 seconds or so, it may be too long. This can leave the heart vulnerable to falling suddenly into a condition called ventricular fibrillation, where the heartbeat becomes too rapid and uncoordinated, which causes the blood flow out of the heart to drop to zero. At this point ventricular fibrillation may be fatal within a few minutes unless the normal heart rhythm can be restored with a cardiac defibrillator.

Research in the last 15 years by physicians and scientists at the University of Utah, Mayo Clinic, and others has shown that Long QT is usually inherited, and several Long QT genetic mutations have been identified.

It is still not known why only a certain portion of the people who receive the gene will never experience any effects of the disorder, which can include sudden fainting spells or sudden death.

Because Long QT is relatively rare and can be inherited without causing any symptoms until an episode of fainting or sudden death occurs, it is often missed on a routine ECG.

ECGs are not typically given to normal healthy children on a routine basis unless there is a family history of cardiac issues or other reasons for concern. When one family member is found to have Long QT Syndrome, it is important for all other near relatives to consult a cardiologist to be evaluated for risk of having a cardiac rhythm problem or being a carrier of a mutation for Long QT.

Because genetic testing is now available for most of the mutations that account for Long QT, it can be valuable to identify the specific mutation carried by the family member who is diagnosed first. Then other relatives can have a test for this exact mutation to find out if they are at risk or if their children may be at risk for the condition.

With help from the Cardiac Arrhythmia Research and Education Foundation, the family met Dr. David Cannom, the director of cardiology and an electrophysiologist at Good Samaritan Hospital in Los Angeles, who is known for research and treatment of Long QT Syndrome. Several months after Elisa's autopsy was completed, the family also contacted Dr. Michael Ackerman at the Mayo Clinic in Minnesota, one of the prominent researchers and clinicians in the field of Long QT arrhythmias.

Ackerman obtained contacted the Los Angeles coroner to obtain a blood sample, and determined that Elisa had a rare Long QT version, labeled Long QT 6. Because this specific version is so rare, there is little clinical information about how it behaves, although it appears to be similar to other more common forms of Long QT, some of which are known to increase the risk of a sudden cardiac arrest during sleep.

Elisa's funeral, attended by approximately 600 people, was held on July 2, 2006 at Mount Sinai Memorial Park in Simi Valley. The Unveling for Elisa will be 10:30 a.m. Sun., June 3 at the memorial park. All interested persons are welcome to attend.

Scholarships have been set up in Elisa's name. To make a donation, contact Temple Etz Chaim of Thousand Oaks at www. templeetzchaim.org, The Calabasas Shul at www. calabasasshul.org or Shomrei Torah Synagogue in West Hills at www.shomreitorahsynagogue.org.

For more medical information about the condition, go to www.long-qt-syndrome.com and also www.sads.org, the site of the Sudden Arrhythmia Death Syndrome Foundation.