Long QT Syndrome
Genetic testing to confirm a diagnosis of congenital long QT syndrome (LQTS) may be considered medically necessary when signs and/or symptoms of LQTS are present, but a definitive diagnosis cannot be made without genetic testing. This includes:
Individuals who do not meet the clinical criteria for LQTS (ie, those with a Schwartz score <4) but have a moderate-to-high pretest probability (see Policy Guidelines section) based on the Schwartz score and/or other clinical criteria. Genetic testing of asymptomatic individuals to determine future risk of LQTS may be considered medically necessary when at least one of the following criteria is met: A close relative (ie, first-, second-, or third-degree relative) with a known LQTS variant; or A close relative diagnosed with LQTS by clinical means whose genetic status is unavailable. Genetic testing for LQTS for all other situations not meeting the criteria outlined above, including but not limited to determining prognosis and/or directing therapy in individuals with known LQTS, is considered investigational. Brugada Syndrome Genetic testing to confirm a diagnosis of Brugada syndrome (BrS) may be considered medically necessary when signs and/or symptoms consistent with BrS (see Policy Guidelines section) are present, but a definitive diagnosis cannot be made without genetic testing. Genetic testing of asymptomatic individuals to determine future risk of BrS may be considered medically necessary when individuals have a close relative (ie, first-, second-, or third-degree relative) with a known BrS variant. Genetic testing for BrS for all other situations not meeting the criteria outlined above is considered investigational. Catecholaminergic Polymorphic Ventricular Tachycardia Genetic testing to confirm a diagnosis of catecholaminergic polymorphic ventricular tachycardia (CPVT) may be considered medically necessary when signs and/or symptoms of CPVT are present, but a definitive diagnosis cannot be made without genetic testing. Genetic testing of asymptomatic individuals to determine future risk of CPVT may be considered medically necessary when at least one of the following criteria is met: A close relative (ie, first-, second-, or third-degree relative) with a known CPVT variant; or A close relative diagnosed with CPVT by clinical means whose genetic status is unavailable. Genetic testing for CPVT for all other situations not meeting the criteria outlined above is considered investigational. Short QT Syndrome Genetic testing of asymptomatic individuals to determine future risk of short QT syndrome (SQTS) may be considered medically necessary when individuals have a close relative (ie, first-, second-, or third-degree relative) with a known SQTS variant. Genetic testing for SQTS for all other situations not meeting the criteria outlined above is considered investigational.
