[INSERT_DATE]
RE:
Genetic Testing for Comprehensive Cardiac Channelopathy Letter of Medical Necessity
Patient Name:
DOB:
Subscriber/ID Number:
To Whom It May Concern:
I am writing on behalf of my patient to request authorization for genetic testing for Comprehensive Cardiac
Channelopathy: CPT codes [INSERT CPT CODES] with diagnosis code(s) of
[INSERT_ICD10_CODES].
I suspect my patient has an inheritable arrhythmia disorder caused by cardiac ion channel genes, based on the
following medical history, signs and symptoms: [INSERT DETAILS, e.g., abnormal ECG findings,
ventricular fibrillation, polymorphic ventricular tachycardia, cardiac conduction system disease, family
member(s) with inherited arrhythmia disorder, syncope, aborted sudden cardiac arrest, unexplained
sudden cardiac death in relatives]
Cardiac channelopathies are genetic heart rhythm disorders caused by mutations in cardiac ion channel
genes, which help regulate the electrical impulses that stimulate the chambers of the heart. For people
affected by cardiac channelopathies, these ion channels do not function properly and cause potentially lethal
cardiac arrhythmias. Cardiac channelopathies are typically characterized clinically by unexplained syncope
and sudden death in the setting of a structurally normal heart (i.e., normal echocardiograph and cardiac MRI)
and ECG findings that may or may not be normal. Cardiac channelopathies are genetically heterogeneous
and include Long QT Syndrome (LQTS), Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT),
Brugada Syndrome (BrS), and Short QT Syndrome (SQTS). An accurate diagnosis of a specific
channelopathy is important for patient management and treatment decisions. For example, Long QT
Syndrome (LQTS) is recognizable by a prolonged QT interval on the ECG; however its diagnosis by ECG
alone often fails since nearly one-third of LQTS patients have a corrected QT interval that overlaps those of
normal, healthy individuals1. Moreover, LQTS is made up of several different subtypes that can only be
determined by a genetic test. Determination of the specific subtype involved is important because each is
associated with a different risk for sudden cardiac death, different event triggers, and different
pharmacological therapies and interventional strategies. The differential diagnosis of LQTS and CPVT is
also clinically challenging as the clinical presentation of CPVT is often similar to LQTS. However, unlike
some forms of LQTS, anti-arrhythmic drug therapies are ineffective in approximately half of CPVT patients
and may require an implantable cardiac defibrillator (ICD) 2-3. Thus, an accurate diagnosis between LQTS
and CPVT is imperative for proper management.
The value of genetic testing for cardiac channelopathies has been documented extensively in the medical
literature, and American College of Cardiology, American Heart Association, European Society of
Cardiology, Heart Rhythm Society, and European Heart Rhythm Association have issued evidence-based
practice guidelines recommending genetic testing for all suspected cardiac channelopathy patients and their
potentially at-risk family members.4-6
The FAMILION Comprehensive Cardiac Channelopathy test is an accurate test for patients suspected to have
genetic heart rhythm disorders caused by mutations in cardiac ion channel genes, and is performed in a
CLIA-certified laboratory that meets all applicable state and federal guidelines. The results of this test are
medically necessary to guide treatment of this patient. Thank you for your time and consideration of my
request. Please contact me if you wish to discuss my patient’s treatment plan or require additional
information.
Respectfully,
Dr. Name
Address
Phone Number
References: 1) Taggart NW, Haglund CM, Tester DJ, Ackerman MJ. Diagnostic miscues in congenital long-QT syndrome.
Circulation. 2007;115(20):2613-20. 2) Priori SG, Napolitano C, Memmi M, Colombi B, Drago F, Gasparini M, et al. Clinical and
molecular characterization of patients with catecholaminergic polymorphic ventricular tachycardia. Circulation. 2002;106(1):69-74.
3) Napolitano C, Priori SG. Diagnosis and treatment of catecholaminergic polymorphic ventricular tachycardia. Heart rhythm : the
official journal of the Heart Rhythm Society. 2007;4(5):675-8. 4) Zipes DP, Camm AJ, Borggrefe M, et al. ACC/AHA/ESC 2006
Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death. Circulation.
2006;114:e385-484. 5) Ackerman MJ, Priori SG, Willems S, et al. HRS/EHRA expert consensus statement on the state of genetic
testing for the channelopathies and cardiomyopathies. Heart Rhythm. 2011;8:1308-39. 6) Priori SG, Wilde AA, Horie M, et al.
HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia
syndromes. Heart Rhythm. 2013;10:1932-63.