Disease of heart muscle :
- Ventricular chamber enlargement (Dilated)
- Contractile dysfunction

CAUSE
Genetic, Secondary ...
Incidence : 0.57 cases / 100,000 children
Poor Prognoses
40%  Need cardiac transplantation
(Towbin, et.al., 2006)

ADULT
...
Objectives
• To know the Sudden Cardiac Death (SCD)
incidence in DCM children
(larger multicenter cohort)
• To know the ri...
• Multicenter Study
– PCMR (Pediatric Cardiomyopathy
Registry), North America

• Design
– Retrospective if DCM diagnosed f...
Criteria (at least 1) :
Dilated
Cardiomyopathy • Echocardiographic criteria
ventricular [LV] dilation

for DCM left

[i.e....
EXCLUSION CRITERIA
•

specific secondary causes of myocardial abnormalities
• Congenital heart disease
• Endocrine disorde...
• Risk Factor :
– Demographic information, clinical evidence
of CHF, New York Heart Association
functional class, family h...
DATA ANALYSIS
– Descriptive Statistic  risk factor
– Classification and Regression tree
[CART]
• To identify the high ris...
RESULT
CHARACTERISTIC
Time

1990-2009

DCM case

1,803 cases

Mean age at diagnosis

5.3 ± 6.1 Years

Mean LV EDD z-score

4.3 ± ...
Cause of DCM on Children
136

10 78

38

Idiopathic

255
Myocarditis
Neuromuscular
Malformation Syndrome

1286

Familial I...
DCM

Mortality In Children
Alive
1523
84%
Death
280
16%

SCD = Suddent Cardiac Death

Unknow
(56)
20%
Non-SCD
(189)
68%

S...
SCD

All Other

p

SCD

All Other

4.7 ± 5.6
5.3 ± 6.1
0.738
Age at diagnosis
Beta-blocker
54.3
53.5
0.88
Yes
2.9
7
Male
0...
SCD

All Other

p

SCD

All Other

4.7 ± 5.6
5.3 ± 6.1
0.738
Age at diagnosis
Beta-blocker
54.3
53.5
0.88
Yes
2.9
7
Male
0...
DCM Survival

Transplantation
Non-SCD
SCD
RISK

FACTOR

Associated
• CHF at DCM diagnosis
• Antiarrhythmic therapy
• Lower Log (ratio of LV posterior wall
thickness...
Prediction From The Time of DCM Was
Diagnosed

HIGH RISK GROUP
1. LV end-diastolic posterior wall
thickness z-score <-1.7
...
Prediction From The Last Available
Follow-up (64%)

HIGH RISK GROUP
Meet 3 following criteria
• LV end-systolic dimension ...
DISCUSSION
5 year Cumulative Incidence of SCD in
children with DCM =

2.4 %
5 year Cumulative Incidence of SCD in
children with DCM = 2.4

%

Compare
No

Author

Years

Result

1

Dimas vv, et al

2...
5 year Cumulative Incidence of SCD in
children with DCM = 2.4

%

Compare
No

Author

Years

Result

1

Dimas vv, et al

2...
First Diagnosed
Prediction at Diagnosed

Last Available Follow-up
Decision for ICD
First Diagnosed

Last Available Follow-up

Prediction at Diagnosed

Decision for ICD

1. LV end-diastolic posterior wall
t...
Not Yet Recommeded

Dubin et al (2003) :
• incidence of inappropriate ICD discharge was 25%

Need further study for ICD sa...
Children With Dilated Cardiomyopathy
High Risk Factor
No High Risk Factor

Sudden Cardiac Death
VALID

Defined and
Representative Sample

Long Follow-Up

Blind Outcome Criteria

Adjustment Important
Factor
1-year

1.3%

3-year

2.0%

5-year

2.4%

(95% CI: 1.7% to 3.4%)

24 % had SSD
57% sensitivity and 78% specificity

Positi...
High Risk
N = 415
SCD
Non-SCD

20
395

OD RATIO
20 x 1317 = 4.44
395 x 15

Non high
Risk
N = 1332
15
1317
Mean age at diagnosis

5.3 ± 6.1 Years

Mean LV EDD z-score

4.3 ± 2,7

LV Fractional Shortening

16 ± 9 %

LFEF

28 ± 14%
First Diagnosed
1.
2.

LV end-diastolic posterior wall thickness z-score <-1.7
Group with :
•
LV end-diastolic posterior w...
JOURNAL : Incidence of and Risk Factors for Sudden Cardiac Death in Children With Dilated Cardiomyopathy
JOURNAL : Incidence of and Risk Factors for Sudden Cardiac Death in Children With Dilated Cardiomyopathy
JOURNAL : Incidence of and Risk Factors for Sudden Cardiac Death in Children With Dilated Cardiomyopathy
JOURNAL : Incidence of and Risk Factors for Sudden Cardiac Death in Children With Dilated Cardiomyopathy
JOURNAL : Incidence of and Risk Factors for Sudden Cardiac Death in Children With Dilated Cardiomyopathy
JOURNAL : Incidence of and Risk Factors for Sudden Cardiac Death in Children With Dilated Cardiomyopathy
JOURNAL : Incidence of and Risk Factors for Sudden Cardiac Death in Children With Dilated Cardiomyopathy
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JOURNAL : Incidence of and Risk Factors for Sudden Cardiac Death in Children With Dilated Cardiomyopathy

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Incidence of and Risk Factors for Sudden Cardiac Death in Children With Dilated Cardiomyopathy

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JOURNAL : Incidence of and Risk Factors for Sudden Cardiac Death in Children With Dilated Cardiomyopathy

  1. 1. Disease of heart muscle : - Ventricular chamber enlargement (Dilated) - Contractile dysfunction CAUSE Genetic, Secondary to other cardiovascular disease, infection, Toxic
  2. 2. Incidence : 0.57 cases / 100,000 children Poor Prognoses 40%  Need cardiac transplantation (Towbin, et.al., 2006) ADULT • Sudden Cardiac Death (SCD) is common in DCM adult • Cause of death  CHF • NEW treatment of Implantable cardioverterdefibrillators (ICDs)  AHA recommended in DCM Adult with (LVEF) <35% • Lack information of DCM incidence and SCD in LARGE population • Lack information of SCD risk factor • Need Information about high-risk child  criteria for Implantable cardioverterdefibrillators (ICDs) option
  3. 3. Objectives • To know the Sudden Cardiac Death (SCD) incidence in DCM children (larger multicenter cohort) • To know the risk factor for SCD in DCM children
  4. 4. • Multicenter Study – PCMR (Pediatric Cardiomyopathy Registry), North America • Design – Retrospective if DCM diagnosed from 1990-1995 – Cohort study if DCM diagnosed >1995
  5. 5. Criteria (at least 1) : Dilated Cardiomyopathy • Echocardiographic criteria ventricular [LV] dilation for DCM left [i.e., LV end-diastolic dimension [EDD] > 2SD] depressed LV systolic function [LV fractional shortening or LVEF >2 SD • Pathologic findings consistent with DCM at autopsy or by endomyocardial biopsy; • Other clinical evidence of DCM provided by the cardiologist Sudden Cardiac Death • Unexpected death • Occurring <1 h after the onset of a symptomatic cardiac event
  6. 6. EXCLUSION CRITERIA • specific secondary causes of myocardial abnormalities • Congenital heart disease • Endocrine disorders known to cause myocardial damage • chemotherapy or pharmacology-associated cardiotoxicity • chronic arrhythmia, • pulmonary parenchymal or • Vascular disease, • Immunologic disease
  7. 7. • Risk Factor : – Demographic information, clinical evidence of CHF, New York Heart Association functional class, family history of cardiomyopathy, medication classes, and other therapies – Echocardiographic measurements • • • • • • LV EDD (Left Ventricular End-Diastolic Dimension) LV end-systolic dimension, LV fractional shortening, LV septal and LV posterior wall thicknesses, LV mass, Tricuspid or mitral regurgitation
  8. 8. DATA ANALYSIS – Descriptive Statistic  risk factor – Classification and Regression tree [CART] • To identify the high risk group
  9. 9. RESULT
  10. 10. CHARACTERISTIC Time 1990-2009 DCM case 1,803 cases Mean age at diagnosis 5.3 ± 6.1 Years Mean LV EDD z-score 4.3 ± 2,7 LV Fractional Shortening 16 ± 9 % LFEF 28 ± 14% Median Follow up (Patient With no Death or Transplatation Event) Max 2.6 Years 16.7 Years
  11. 11. Cause of DCM on Children 136 10 78 38 Idiopathic 255 Myocarditis Neuromuscular Malformation Syndrome 1286 Familial Isolated Cardiomyopathy Inborn Error of Metabolism
  12. 12. DCM Mortality In Children Alive 1523 84% Death 280 16% SCD = Suddent Cardiac Death Unknow (56) 20% Non-SCD (189) 68% SCD (35) 12%
  13. 13. SCD All Other p SCD All Other 4.7 ± 5.6 5.3 ± 6.1 0.738 Age at diagnosis Beta-blocker 54.3 53.5 0.88 Yes 2.9 7 Male 0.649 No 40 48 Race / ethnicity White 62.9 55.3 Unknow 57.1 45 Black 22.9 21 4.2 ± 2.3 4.3 ± 2.4 LV end-diastolic dimension Z-Score Hispanic 11.4 16.9 5.9 ± 2.2 6.0 ± 2.5 LV end-systolic dimension Z-Score Other 2.9 6.8 -8.8 ± 2.5 -8.5 ± 3.4 LV fractional shortening Z-Score 77.1 71.6 0.279 Idiopathic LV end-diastolic posterior wall thickness Z-1.1 ± 2.8 -0.5 ± 2.0 Score 86.7 72.6 0.031 CHF at Diagnosis 0.139 NYHA functional class IV LV end-diastolic septal wall thickness Z-1.1 ± 1.1 -0.8 ± 1.5 Score Yes 34.3 23.8 No 20 19.6 2.0 ± 2.7 2.3 ± 2.8 LV mass Z-Score Unknow 45.7 56.5 -6.9 ± 2.5 6.0 ± 2.4 LVEF Z-Score 0.551 23.3 ± 14.6 28.5 ± 13.7 Familiy history of cardiomyopaty Raw LVEF yes 11.4 12.1 8 (80.0) 330 (67.8) LVEF <35% no 61.4 44.9 15.3 ± 6.2 16.0 ± 8.3 Raw LV fractional shortening (%) unknow 37.1 43 27 (77.1) 1249 (73.0) LV fractional shortening <18% 0.905 Anticongestive therapy Log (ratio of LV posterior wall thickness -2.24 ± 0.38 -2.13 ± 0.32 end-diastolic dimension) Yes 5.7 6 No 54.3 52.7 Moderate to severe tricuspid regurgitation Unknow 40 41.3 Yes 8.6 3.7 0.025 No 14.3 26.3 Antiarrhythmic therapy Yes 20.0 12.1 Unknow 77.1 69.9 No 22.9 41.4 Moderate to severe mitral regurgitation Unknow 57.1 46.5 Yes 14.3 9.3 0.023 No 11.4 20.9 ACE Inhibitor Yes 20 38.3 Unknow 74.3 69.9 No 22.9 18.5 Unknow 57.1 43.2 ACE : angiotensin-converting enzyme; CHF : congestive heart failure; CI : confidence interval; LV : left ventricular; LVEF : left ventricular ejection fraction; NYHA : New York Heart Association; SCD : sudden cardiac death. p 0.09 0.928 0.883 0.047 0.123 0.766 0.123 0.073 0.213 0.298 0.282 0.016 0.078 0.176
  14. 14. SCD All Other p SCD All Other 4.7 ± 5.6 5.3 ± 6.1 0.738 Age at diagnosis Beta-blocker 54.3 53.5 0.88 Yes 2.9 7 Male 0.649 No 40 48 Race / ethnicity White 62.9 55.3 Unknow 57.1 45 Black 22.9 21 4.2 ± 2.3 4.3 ± 2.4 LV end-diastolic dimension Z-Score Hispanic 11.4 16.9 5.9 ± 2.2 6.0 ± 2.5 LV end-systolic dimension Z-Score Other 2.9 6.8 -8.8 ± 2.5 -8.5 ± 3.4 LV fractional shortening Z-Score 77.1 71.6 0.279 Idiopathic LV end-diastolic posterior wall thickness Z-1.1 ± 2.8 -0.5 ± 2.0 Score 86.7 72.6 0.031 CHF at Diagnosis 0.139 NYHA functional class IV LV end-diastolic septal wall thickness Z-1.1 ± 1.1 -0.8 ± 1.5 Score Yes 34.3 23.8 No 20 19.6 2.0 ± 2.7 2.3 ± 2.8 LV mass Z-Score Unknow 45.7 56.5 -6.9 ± 2.5 6.0 ± 2.4 LVEF Z-Score 0.551 23.3 ± 14.6 28.5 ± 13.7 Familiy history of cardiomyopaty Raw LVEF yes 11.4 12.1 8 (80.0) 330 (67.8) LVEF <35% no 61.4 44.9 15.3 ± 6.2 16.0 ± 8.3 Raw LV fractional shortening (%) unknow 37.1 43 27 (77.1) 1249 (73.0) LV fractional shortening <18% 0.375 Anticongestive therapy Log (ratio of LV posterior wall thickness -2.24 ± 0.38 -2.13 ± 0.32 end-diastolic dimension) Yes 5.7 6 No 54.3 52.7 Moderate to severe tricuspid regurgitation Unknow 40 41.3 Yes 8.6 3.7 0.025 No 14.3 26.3 Antiarrhythmic therapy Yes 20.0 12.1 Unknow 77.1 69.9 No 22.9 41.4 Moderate to severe mitral regurgitation Unknow 57.1 46.5 Yes 14.3 9.3 0.023 No 11.4 20.9 ACE Inhibitor Yes 20 38.3 Unknow 74.3 69.9 No 22.9 18.5 Unknow 57.1 43.2 ACE : angiotensin-converting enzyme; CHF : congestive heart failure; CI : confidence interval; LV : left ventricular; LVEF : left ventricular ejection fraction; NYHA : New York Heart Association; SCD : sudden cardiac death. p 0.09 0.928 0.883 0.047 0.123 0.766 0.123 0.073 0.213 0.298 0.282 0.016 0.078 0.176
  15. 15. DCM Survival Transplantation Non-SCD SCD
  16. 16. RISK FACTOR Associated • CHF at DCM diagnosis • Antiarrhythmic therapy • Lower Log (ratio of LV posterior wall thickness end-diastolic dimension) • LV posterior wall thickness z-score No-Associated • • • • Race Sex Cause of DCM Family history of SCD or cardiomyopaty • NYHA class • Anticongestive or B-blocker • Other echocardiograph finding
  17. 17. Prediction From The Time of DCM Was Diagnosed HIGH RISK GROUP 1. LV end-diastolic posterior wall thickness z-score <-1.7 2. Group with : • LV end-diastolic posterior wall thickness z-score >-1.7 • Age at diagnosis < 13.1 yo • Septal thickness z-score 0.8, • Using antiarrhythmic therapy within a month of presentation with DCM 57% sensitivity and 78% specificity. Positive predictive value (percentage of % SCD among those identified as high risk) = 5%, Negative predictive value (percentage of non-SCD among those identified as lower risk) = 99%.
  18. 18. Prediction From The Last Available Follow-up (64%) HIGH RISK GROUP Meet 3 following criteria • LV end-systolic dimension z-score >2.6; • DCM diagnosis at age younger than 14.3 years; • LVPWT:EDD ratio <0.14 86% sensitivity and 57% specificity. Positive predictive value (percentage of % SCD among those identified as high risk) = 4%, Negative predictive value (percentage of non-SCD among those identified as lower risk) = 99%.
  19. 19. DISCUSSION
  20. 20. 5 year Cumulative Incidence of SCD in children with DCM = 2.4 %
  21. 21. 5 year Cumulative Incidence of SCD in children with DCM = 2.4 % Compare No Author Years Result 1 Dimas vv, et al 2009 2 Rhee, et al., 2007 Incidence SCD in DCM children and Congenital Heart Disease was 3 Shekha K., et al 2005 [ADULT] SCD in DCM adult was 4 Kadish, et al. 2004 [ADULT] SCD occurred in DCM IN CHILDREN DCM children, died suddenly of 458 adults with DCM (with LVEF 36%) • SCD has lower incidence than adult • Death caused by progressive CHF is more common • Children had fewer “ventricular arrhythmia”
  22. 22. 5 year Cumulative Incidence of SCD in children with DCM = 2.4 % Compare No Author Years Result 1 Dimas vv, et al 2009 1% DCM children, died suddenly 2 Rhee, et al., 2007 Incidence SCD in DCM children and Congenital Heart Disease was 1.3 3 Shekha K., et al 2005 [ADULT] SCD in DCM adult was low 4 Kadish, et al. 2004 [ADULT] SCD occurred in 7.4% of 458 adults with DCM (with LVEF 36%) DCM IN CHILDREN % • SCD has lower incidence than adult • Death caused by progressive CHF is more common • Children had fewer “ventricular arrhythmia” DIFFERENT NATURAL HISTORY THAN ADULT
  23. 23. First Diagnosed Prediction at Diagnosed Last Available Follow-up Decision for ICD
  24. 24. First Diagnosed Last Available Follow-up Prediction at Diagnosed Decision for ICD 1. LV end-diastolic posterior wall thickness z-score <-1.7 2. Group with : • LV end-diastolic posterior wall thickness z-score >-1.7 • Age at diagnosis < 13.1 yo • Septal thickness z-score 0.8, • Using antiarrhythmic therapy within a month of presentation with DCM Meet 3 following criteria • LV end-systolic dimension z-score >2.6; • DCM diagnosis at age younger than 14.3 years; • LVPWT:EDD ratio <0.14 24% of children with DCM would receive an ICD at the time of diagnosis of cardiomyopathy 44% of subjects might receive an ICD
  25. 25. Not Yet Recommeded Dubin et al (2003) : • incidence of inappropriate ICD discharge was 25% Need further study for ICD safety
  26. 26. Children With Dilated Cardiomyopathy High Risk Factor No High Risk Factor Sudden Cardiac Death
  27. 27. VALID Defined and Representative Sample Long Follow-Up Blind Outcome Criteria Adjustment Important Factor
  28. 28. 1-year 1.3% 3-year 2.0% 5-year 2.4% (95% CI: 1.7% to 3.4%) 24 % had SSD 57% sensitivity and 78% specificity Positive predictive value 5%, negative predictive value 99% 44 % had SSD 86% sensitivity and 57% specificity Positive predictive value 4%, negative predictive value 99%
  29. 29. High Risk N = 415 SCD Non-SCD 20 395 OD RATIO 20 x 1317 = 4.44 395 x 15 Non high Risk N = 1332 15 1317
  30. 30. Mean age at diagnosis 5.3 ± 6.1 Years Mean LV EDD z-score 4.3 ± 2,7 LV Fractional Shortening 16 ± 9 % LFEF 28 ± 14%
  31. 31. First Diagnosed 1. 2. LV end-diastolic posterior wall thickness z-score <-1.7 Group with : • LV end-diastolic posterior wall thickness z-score >-1.7 • Age at diagnosis < 13.1 years old • Septal thickness z-score 0.8, • Using antiarrhythmic therapy within a month of presentation with DCM Last Available Follow-up Meet 3 following criteria • LV end-systolic dimension zscore >2.6; • DCM diagnosis at age younger than 14.3 years; • LVPWT:EDD ratio <0.14

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