Long QT Syndrome is an inherited defect which is likely to affect the rhythm of the heart. In the U.S., it is said to be affecting around 4000 children and young adults; and what makes the situation even grimmer is the fact that this condition is fatal. It is said to be one of the major causes behind the death of athletes; as well as the culprit behind the unexplained drowning.
The first sign of this syndrome might be cardiac death. When a person suffers from Long QT, his heart might abruptly go into fibrillation. This would mean that the heart will begin beating at an abnormally rapid pace and this will become so ineffective that blood circulation to and from the heart will cease completely. In such cases, then, the heart will have to be defibrillated – a medical procedure where the heart is shocked back into its normal rhythm. This has to be done within a few minutes of the person going into fibrillation, if the person has to survive.
The name comes from the QT segment that is visible on the tracing of the electrocardiogram (ECG). In the case of patients suffering from Long QT, this segment will end up lasting longer than in the case of a normal person. Certain conditions will be known to act as a trigger in the case of Long QT Syndrome. These would include the likes of being startled or frightened, swimming, excessive physical exercise, etc.
Quite a few people with long QT might not even be aware of their condition, since they will not display very diverse signs and symptoms that point in that direction. These people might become aware of their condition only when they watch the results arising from an electrocardiogram (ECG) which can be performed for an unrelated reason.
Some of the common tell-tale signs of this condition might include the following:
Fainting:
This is perhaps one of the most common symptoms of this condition. Fainting spells or syncope are extremely common among these people because of the heart beating in an erratic way for temporary periods. When the person becomes overtly excited, scared or angry, a fainting spell could occur. In the case of people suffering from this condition, fainting could occur without any warning.
Seizures:
When the heart continues to beat erratically for long periods, it may cause the brain to get deprived of oxygen. This could then be the cause of a generalized seizure.
Sudden Death:
In most cases, the heart is known to come back to its normal rhythm after a few minutes or seconds. But if this doesn’t happen naturally or spontaneously, then it could turn fatal. Paramedics might be able to help, by using a defibrillator to convert the heartbeats back to their normal rhythm; but if that doesn’t happen in time, it could cause for the death of the person.
The Long QT Syndrome is generally known to be inherited. It is caused by a genetic mutation in the gene which is responsible for regulating the electrical system of the human heart. Owing to this mutation, there might be a defect that occurs in the structure of the heart’s muscles and these are called ion channels. Due to this, the heart might lapse into fibrillation. Given the fact that this mutation, which is responsible for long QT Syndrome, is inherited in the form of an autosomal dominant, it would mean that each child born of an affected parent has around 50% chances of being affected himself/herself.
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