Every two minutes, someone in the U.S. dies of Sudden Cardiac Arrest (SCA). Unlike a heart attack, SCA is caused by an electrical issue with the heart. The heart has an internal electrical system that controls the speed and rhythm of the heartbeat. Problems with the electrical system can cause abnormal heart rhythms called arrhythmias.

SCA is the leading cause of natural death in the U.S. and kills more than 325,000 Americans each year - more than breast cancer, lung cancer, and AIDS combined.

Sudden Arrhythmia Death Syndrome (SADS) causes sudden and unexpected death of children and young adults. In 1991, the University of Utah provided a landmark study that helped recognize and explain this often misdiagnosed death in the young.

SADS is three times more common in the U.S. than childhood leukemia.  As many as 12 in every 5,000 young people may have one of the conditions that can result in sudden death, and most sudden deaths of children are due to hereditary predispositions; and finally, most cardiac arrhythmia and structural defects are identifiable and treatable, leading to normal life spans and life styles. Long QT Syndrome (LQTS) is just one of many cardiac arrhythmias that can cause sudden death in children and young adults, most of which are identifiable and treatable.

Cardiac arrhythmias do not only affect children but can also affect adults.  You may even know some of the names.  

Michaela Gagne, Miss Massachusetts in 2006, made headlines during the Miss America pageant after she displayed a scar on her upper chest.  The scar is from a surgery where doctors placed an implantable cardioverter defibrillator (ICD) to prevent sudden cardiac arrest.   Doctors diagnosed Miss Massachusetts with long QT syndrome when she was 17, and she now leads a normal, active life thanks to the ICD.   Michaela was the keynote speaker at the 2009 Starry Night Gala benefiting the Brianna Badger Foundation

Another Long QT survivor is former Colorado Avalanche forward Steve Konowalchuk.  A native of Salt Lake City, Utah, he was originally drafted by the Washington Capitals. Over his 13 NHL seasons, he appeared in 790 NHL games, amassing 171 goals and 225 assists. He made 52 NHL playoff appearances, tallying 21 points. After a 13-season career in the National Hockey League, Steve retired from the game of hockey in September 2006 because of a Long QT diagnosis.  Steve honored the Brianna Badger Foundation by attending and speaking at the 2008 event.

Long QT Syndrome

LQTS is an abnormality of the heart's electrical system. While the mechanical function of the heart is entirely normal, the electrical defects can create a fast heart rhythm called an arrhythmia, which can lead to sudden loss of consciousness or syncope and may cause sudden cardiac death. Long QT refers to the QT-interval measured on the electrocardiogram as being prolonged.

The "corrected" QT-interval (QTc) is adjusted for heart rate. Any QTc-interval on an ECG above 440 milliseconds is considered prolonged. Borderline QT shows a prolongation of QTc, but not prolonged enough to clearly make the diagnosis. 450 to 470 milliseconds is considered borderline. The average QTc for someone who has long-QT syndrome is 490 milliseconds.

A QTc at or above 480 milliseconds in females or 470 milliseconds in males, is probably a sign for long-QT syndrome, in the absence of drugs, electrolyte disturbance, or other conditions that might independently lengthen the QT-interval.

The typical symptoms are a sudden loss of consciousness or more severly sudden death, typically occurring during physical activity or emotional upset. These most commonly begin in preteen to teenage years, but may occur from birth to middle age. The syncopal episodes are often misdiagnosed as the common faint or a seizure as in Brianna's case. Actual seizures are uncommon in long QT syndrome, but epilepsy is one of the common errors in diagnosis. Sudden loss of consciousness during physical exertion or during emotional excitement should strongly raise the possibility of the long QT syndrome.

If you have a family history of unexplained fainting or sudden death please get an ECG and ask your physician to investigate the possibility of a cardiac arrhythmia. Nearly one third of individuals who have the long QT syndrome never exhibit symptoms, but the lack of symptoms does not exclude a person or family from having long QT syndrome. Any young person that has an unexplained cardiac arrest should be considered for long QT syndrome, as well as those with unexplained fainting.