Mean / Low Input / High Input / Reference
Model Parameters
Age in years at screening (Age enter model) / 12 / 8 / 25 / Estimated
Number of years for which is model run / 70 / 30 / 70 / Estimated
Discount rate / 0.03 / 0.01 / 0.05 / Estimated
Screen Parameters
ECG, probability of false negative, HCM / 0.05 / 0.02 / 0.3 / [1,2]
ECG, probability of false positive, HCM / 0.1 / 0.008 / 0.2 / [2,3]
ECG, probability of false negative, LQTs / 0.15 / 0 / 0.3 / [4,5]
ECG, probability of false positive, LQTs / 0.1 / 0 / 0.2 / Estimated
H&P, probability of false negative, HCM / 0.75 / 0.5 / 1 / Estimated
H&P, probability of false negative, LQTs / 0.9 / 0.5 / 1 / Estimated
Hypertrophic Cardiomyopathy
Probability of death, per year
LR, age <16 years / 0.01 / 0.005 / 0.02 / [3,6]
LR, age 16-55 years / 0.02 / 0.01 / 0.04 / [7-9]
LR, age >55 years / 0.005 / 0.0025 / 0.01 / [8,10,11]
HR, treated (ICD), without AF, HF, ES / 0.005 / 0.0022 / 0.009 / [3,7]
With pacemaker or ICD, with HB after myomectomy / 0.001 / 0.0005 / 0.002 / [12]
HR, with AF / 0.029 / 0.01 / 0.05 / [10,13]
HR, with HF / 0.04 / 0.02 / 0.08 / [11,13,14]
After myomectomy / 0.019 / 0.008 / 0.03 / [12]
ES, while listed for transplant / 0.11 / 0.05 / 0.2 / [11]
Other disease parameters
HCM prevalence / 0.002 / 0.001 / 0.004 / [8]
H&P, probability start LR / 0.95 / 0.8 / 1 / [7,14]
ECG/H&P, probability start LR / 0.95 / 0.8 / 1 / [7,14]
Undiagnosed, probability diagnosed / year / 0.016 / 0.008 / 0.032 / [13]
Diagnosed, probability of genetic testing / 1 / 0.9 / 1 / [15]
LR, probability of becoming HR / year / 0.008 / 0.004 / 0.016 / [14]
Lower age at which risk of dying increases / 16 / 12 / 20 / Estimated
Upper age at which risk of dying decreases / 50 / 35 / 60 / Estimated
HR, probability of developing AF / year / 0.002 / 0 / 0.004 / [10]
With AF, probability of stroke / year / 0.048 / 0.024 / 0.071 / [10,16]
HR, probability of developing HF / year / 0.006 / 0.001 / 0.01 / [12,13,17]
With HF, probability becoming ES / year / 0.012 / 0.0006 / 0.024 / [11,13]
HR, probability of developing obstruction / 0.001 / 0.0001 / 0.001 / [7]
Myomectomy, incidence of HB after / 0.015 / 0.0075 / 0.03 / [7]
Myomectomy, operative mortality / 0.02 / 0.01 / 0.04 / [12,18]
Effect of diagnosis and treatment on mortality of undiagnosed hypertrophic cardiomyopathy versus diagnosed LR / 0.67 / 0.33 / 1 / Estimated
Long QT Syndrome
Probability of death, per year
LR / 0.001 / 0.0005 / 0.002 / [4,19,20]
HR, on beta-blocker or Mexilitine / 0.01 / 0.0012 / 0.02 / [19-21]
After ICD / 0.002 / 0.001 / 0.004 / [19]
After LCSD / 0.01 / 0.005 / 0.02 / [19]
Undiagnosed / 0.013 / 0.007 / 0.05 / Estimated
Other disease parameters
LQTS prevalence / 0.0004 / 0.0002 / 0.0008 / [19]
ECG/H&P, probability start HR / 0.25 / 0 / 0.25 / [5]
H&P, probability start HR / 0.75 / 0.5 / 1 / [5]
Diagnosed, probability of genetic testing / 1 / 0.9 / 1 / [15]
LR, probability of progressing to HR / 0.027 / 0.0135 / 0.054 / [4]
LR probability of beta-blockers or mexiletine / 1 / 0.5 / 1 / Estimated
HR, probability of ICD placement with continued beta-blockers / year / 0.00234 / 0.001 / 0.005 / [4]
HR, probability of ICD placement without beta-blockers / year / 0.00114 / 0.00062 / 0.00228 / [1,4]
HR, probability of LCSD / year / 0.00045 / 0.00023 / 0.0009 / [4]
Undiagnosed, probability diagnosed / year / 0.01 / 0.01 / 0.03 / [19]
Costs ($)
Cardioversion (in- & out- patient average) / 1,829 / 915 / 2,744 / [22]
Clinic visit, initial / 147 / 74 / 221 / [22]
Clinic visit, follow-up / 81 / 40 / 121 / [22]
Electrocardiogram / 33 / 16 / 50 / [22]
Echocardiogram, initial / 599 / 300 / 900 / [22]
Echocardiogram, follow-up / 272 / 136 / 408 / [22]
Exercise stress test / 254 / 126 / 381 / [22]
Holter / 189 / 94 / 283 / [22]
Genetic testing / 2,500 / 1,250 / 5,000 / Estimated
Hospitalization, HF / 3,625 / 1,812 / 5,437 / [22]
ICD insertion / 27,783 / 13,891 / 55,565 / [22]
ICD or Pacemaker revision, every 5 years / 24,894 / 12,447 / 49,788 / [22]
Blood test (coagulation factors) / 23 / 11 / 34 / [22]
LCSD / 13,158 / 6,577 / 19,733 / [22]
Myomectomy / 39,370 / 19,692 / 59,075 / [22]
Pacemaker check, office / 50 / 25 / 75 / [22]
Pacemaker check, by phone / 44 / 22 / 66 / [22]
Pacemaker insertion / 27,783 / 13,891 / 55,565 / [22]
Transplantation / 65,359 / 32,652 / 97,955 / [22]
Medications
Amiodarone (monthly) / 155 / 77 / 232 / [23]
Beta-blockers (monthly) / 144 / 71 / 215 / [23]
Coumadin (monthly) / 39 / 20 / 59 / [23]
Verapamil (monthly) / 48 / 24 / 72 / [23]
AF=Atrial fibrillation; ECG/H&P=Screening arm with electrocardiogram and history/physical examination; ES=End-stage/listed for transplant; HCM=Hypertrophic cardiomyopathy; HB=Heart block; HF=Heart failure; H&P=Screening arm with only history and physical; LCSD=Left cervical sympathetic denervation; LQTS=Long QT syndrome; LR=Lower risk; HR=Higher risk; ICD=Implantable cardioverter defibrillator
References for Supplemental Table
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22(2010. Available at Accessed June 15, 2010 ) Centers for Medicare and Medicaid Services.
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