Living with Long QT Syndrome

A diagnosis of any type of heart defect can be especially troubling. It invites uncertainty and fear into the lives of the affected person as well the lives of their loved ones. Long QT syndrome (LQTS) is no exception. This particular disorder produces rapid heartbeats that can prompt seizures and fainting spells. In some cases, if the heartbeat continues at a rapid pace for a long period of time, sudden death can occur. Luckily, LQTS is treatable and patients that understand the mechanics of this disorder can take precautions to prevent seizures, blackouts, and sudden death.

Causes of LQTS

In many cases, LQTS is an inherited, genetic disorder. It often runs in families and researchers have identified 12 genes that are typically associated with the disorder. Of these 12 genes, three of the genes mutate to account for between 70 and 75 percent of the inherited cases of LQTS. The most common inherited forms of LQTS are: Romano-Ward syndrome and Jervell and Lange-Nielsen syndrome.

Although the majority of LQTS diagnoses are due to genetic factors, there are certain medical conditions and medications that can trigger LQTS in patients. In fact, there are over 50 medications, according to the Mayo Clinic, that include LQTS as possible side effects of usage. These medications treat a variety of conditions, including the following:

  • Depression
  • Heart disease
  • Diabetes
  • High cholesterol
  • Allergies

Some researches and medical professionals believe that some patients that develop LQTS as a result of taking certain medications may have a genetic mutation that makes them prone to developing the disorder.

Diagnosing LQTS

Doctors begin to diagnose LQTS by looking for certain symptoms such as the following:

  • Unexplained fainting spells
  • Seizures
  • Heart palpitations
  • Blurred vision
  • Muscle weakness
  • Irregular heartbeat

Most signs of genetic LQTS develop during the first few months of a child’s life; however, symptoms may not fully appear until middle life. The majority of patients with inherited LQTS will begin to show symptoms by the age of 40.

Medical professionals that suspect their patient has LQTS will order a variety of tests, including: ¬†an electrocardiogram (ECG), ambulatory ECG monitoring, and event ECG recording. Doctors may also order a non-exercise stress test to monitor the heart’s reaction to certain stimuli and genetic testing may also be considered to make a complete diagnosis.

Treating LQTS

When caught in the early stages, LQTS is highly treatable. The goal is to improve the heart’s rhythm and prevent it from going into an erratic state that could lead to sudden death. Doctors will try to treat the disorder using medications first. Popular medications include beta blockers and Mexiletine. Supplements like fish oil and minerals such as potassium may also be prescribed to stabilize the heart’s rhythm.

In some cases, when a patient may be at high risk of sudden death or they cannot tolerate medications, surgery may be recommended. If this is the case, the patient has the option of a pacemaker to stabilize heart rhythms or left cardiac sympathetic denervation surgery.

Medical professionals will discuss all options with their patients to determine a long-term treatment plan that will enable the individual to live happy and long-lasting life.

A Guide to living with Long QT Syndrome