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What is New in Risk Stratification?
Martin S. Maron, MD
Medical Director, HCM Center
Tufts Medical Center Boston, MA
Chani...
Two New Issues in Risk
Assessment in HCM…….
• Contrast-enhanced CMR
• ESC Risk Score
Highest
Intermediate
Lowest
ICD
Strongest Risk Factors:
Cardiac arrest/Sus. VT.
Familial Hx of SD
Syncope
Multiple-repetit...
Three
Risk
Factors
(2%)
Two Risk Factors
(10%)
One Risk Factor
(33%)
Zero Risk Factors
(55%)
~50% of Clinically
Identified...
LA
LA
VS
RV
LV VS
A B C
D E F
Foci For Ventricular Arrhythmias?
RV
LV
VS
Holter NSVT and Presence of LGE
0
5
10
15
20
25
30
%ofHCMPatientswithNSVT
LGE (+)
LGE (-)
p<0.001
Adabag et al. Rubinstein...
LGE for Prognosis in HCM
Multicenter Study
Tufts Medical Center, Boston, MA
Minneapolis Heart Institute
Toronto General Ho...
L
G
E
LGELGE
Follow-up (years)
FreedomfromSuddenDeath
LGE (-)
LGE < 10%
LGE 10-19%
LGE ≥20%
p=0.02
Relation Between Sudden...
% LGE Adjusted HR Est. 5-year Event
Rate(%)
0% 1.0 2.5
5% 1.3 3.2
10% 1.6 4.0
15% 2.0 5.0
20% 2.6 6.3
25% 3.2 8.0
30% 4.2 ...
Improvement of Sudden Death Prediction
with Addition of %LGE to Risk Model
SuddenDeathEventRate
Highest
Intermediate
Lowest
ICD
Strongest 10 Risk Factors:
Familial Hx of SD
Syncope
Multiple-repetitive NSVT
BP — exerci...
Two New Issues in Risk
Assessment in HCM…….
• Contrast-enhanced CMR
• ESC Risk Score
The ESC-HCM-Sudden death prediction formula is as follows:
Probability SCD at 5 years = 1 – 0.998 exp (Prognostic index);
...
MISSING FROM ESC RISK MODEL:
• CMR and LGE
• LV apical aneurysm
• End stage (EF <50%)
QUESTIONABLE ADDITIONS TO ESC RISK M...
Study Population
Minneapolis Heart Institute; Tufts Medical Center
1649 consecutive HCM patients ≥ 16 years
(1992-2014)
• ...
%PatientsWith/Without
ICDIntervention/SuddenDeath
Appropriate
ICD
Intervention
No Appropriate
ICD
Intervention
ESC Risk Sc...
ESC Prognostic Score…
In a genetic heart disease with enormous
heterogeneity, a strategy in which risk stratification
is r...
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What’s new in risk stratification

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What’s new in risk stratification

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What’s new in risk stratification

  1. 1. What is New in Risk Stratification? Martin S. Maron, MD Medical Director, HCM Center Tufts Medical Center Boston, MA Chanin T. Mast HCM Center, Morristown Medical Center Disclosure: Gadolinium is FDA off-label use for CV imaging
  2. 2. Two New Issues in Risk Assessment in HCM……. • Contrast-enhanced CMR • ESC Risk Score
  3. 3. Highest Intermediate Lowest ICD Strongest Risk Factors: Cardiac arrest/Sus. VT. Familial Hx of SD Syncope Multiple-repetitive NSVT BP — exercise Massive LVH ≥30 mm
  4. 4. Three Risk Factors (2%) Two Risk Factors (10%) One Risk Factor (33%) Zero Risk Factors (55%) ~50% of Clinically Identified HCM Pts At Increased Risk For Sudden Death Challenges of Risk Stratification in HCM ~40% of HCM Sudden Deaths 0.5%/year The “Grey Area” of Risk Stratification
  5. 5. LA LA VS RV LV VS A B C D E F
  6. 6. Foci For Ventricular Arrhythmias? RV LV VS
  7. 7. Holter NSVT and Presence of LGE 0 5 10 15 20 25 30 %ofHCMPatientswithNSVT LGE (+) LGE (-) p<0.001 Adabag et al. Rubinstein et al. (Mayo)n=177 n=220
  8. 8. LGE for Prognosis in HCM Multicenter Study Tufts Medical Center, Boston, MA Minneapolis Heart Institute Toronto General Hospital, Canada Azzendia Carregia, Florence, Italy Bologna, Italy Pisa, Italy Rome, Italy PERFUSE CMR Core Laboratory 1,293 HCM Patients SCD Event3.5±1.7
  9. 9. L G E LGELGE Follow-up (years) FreedomfromSuddenDeath LGE (-) LGE < 10% LGE 10-19% LGE ≥20% p=0.02 Relation Between Sudden Death and Extent of LGE in 1293 HCM Patients
  10. 10. % LGE Adjusted HR Est. 5-year Event Rate(%) 0% 1.0 2.5 5% 1.3 3.2 10% 1.6 4.0 15% 2.0 5.0 20% 2.6 6.3 25% 3.2 8.0 30% 4.2 10.0 40% 6.7 15.5 Sudden Death Event Rates in HCM Patients Without Conventional Risk Factors
  11. 11. Improvement of Sudden Death Prediction with Addition of %LGE to Risk Model SuddenDeathEventRate
  12. 12. Highest Intermediate Lowest ICD Strongest 10 Risk Factors: Familial Hx of SD Syncope Multiple-repetitive NSVT BP — exercise Massive LVH ≥30 mm CMR LGE ≥15% NO LGE Chan R, Maron MS et al. in Review +
  13. 13. Two New Issues in Risk Assessment in HCM……. • Contrast-enhanced CMR • ESC Risk Score
  14. 14. The ESC-HCM-Sudden death prediction formula is as follows: Probability SCD at 5 years = 1 – 0.998 exp (Prognostic index); where Prognostic index = [0.15939858 x maximal LV wall thickness (mm)] – [0.00294271 x LV maximal wall thickness2 (mm2)] + [0.0259082 x left atrial diameter (mm)] + [0.00446131 x maximal (rest/Valsalva) LV outflow tract gradient (mm Hg)] + [0.4583082 x family history SCD] + [0.82639195 x NSVT] + [0.71650361 x unexplained syncope] – [0.01799934 x age at clinical evaluation (years)]. Low Risk <4%/yr. High Risk ≥6%/yr. Inter. Risk ≥4-6%/yr. 5-year SD Risk Generally Not Indicated May Be Considered Should be ConsideredICD
  15. 15. MISSING FROM ESC RISK MODEL: • CMR and LGE • LV apical aneurysm • End stage (EF <50%) QUESTIONABLE ADDITIONS TO ESC RISK MODEL: • Left atrial size • LV outflow gradient • Remote syncope Mixed /no relation to SD risk No Validation in Independent HCM Population…
  16. 16. Study Population Minneapolis Heart Institute; Tufts Medical Center 1649 consecutive HCM patients ≥ 16 years (1992-2014) • Age: 47 ± 17 years • Risk stratified by: ACC/AHA 2011; ACC/ESC 2003 • Sudden death events: 35 • Primary prevention appropriate ICD interventions: 46
  17. 17. %PatientsWith/Without ICDIntervention/SuddenDeath Appropriate ICD Intervention No Appropriate ICD Intervention ESC Risk Score <4%<4% 4-6%4-6% >6%>6% Risk/5y Risk/5y <4% 4-6%>6% Risk/5y Sudden Death Assessment of ESC Sudden Death Risk Score (n = 1649) 60% 26% 63% 9% 70 patients left vulnerable to SD without protection from ICD… using ESC risk score
  18. 18. ESC Prognostic Score… In a genetic heart disease with enormous heterogeneity, a strategy in which risk stratification is restricted to a rigid automated mathematical formula will, by its very nature, make it difficult to implement a measure of individualized physician judgment in the framework of shared decision-making with fully informed patients

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