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Journal Critical TITLE WITH Incidence of and Risk Factors for Sudden Cardiac Death in Children With Dilated Cardiomyopathy
Citation preview
Journal Reading
Argadia Y.
Pembimbing :dr. Sri Lilijanti, SpA(K)
Dilated Cardiomyopathy
Disease of heart muscle :- Ventricular chamber enlargement (Dilated)
- Contractile dysfunction
CAUSEGenetic, Secondary to other cardiovascular
disease, infection, Toxic
Dilated CardiomyopathyIn Children
• Sudden Cardiac Death (SCD) is common in DCM adult
• Cause of death CHF• NEW treatment of
Implantable cardioverter-defibrillators (ICDs)
AHA recommended in DCM Adult with (LVEF) <35%
ADULT
Incidence : 0.57 cases / 100,000 childrenPoor Prognoses40% Need cardiac transplantation
(Towbin, et.al., 2006)
• 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 cardioverter-defibrillators (ICDs) option
Dilated CardiomyopathyIn Children
• To know the Sudden Cardiac Death (SCD) incidence in DCM children (larger multicenter cohort)
• To know the risk factor for SCD in DCM children
Objectives
METHOD
• Multicenter Study – PCMR (Pediatric Cardiomyopathy
Registry), North America
• Design– Retrospective if DCM diagnosed from
1990-1995– Cohort study if DCM diagnosed >1995
Criteria (at least 1) :
• Echocardiographic criteria for DCM left ventricular [LV] dilation
[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
• Unexpected death
• Occurring <1 h after the onset of a symptomatic cardiac event
Sudden Cardiac Death
Dilated Cardiomyopathy
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
• 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
DATA ANALYSIS– Descriptive Statistic risk factor
– Classification and Regression tree [CART]• To identify the high risk group
RESULT
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 Years16.7 Years
SUBJECTCHARACTERISTIC
Cause of DCM on Children
1286
255
13610 78 38
Idiopathic
Myocarditis
Neuromuscular
Malformation Syndrome
Familial Isolated Cardiomyopathy
Inborn Error of Metabolism
SCD(35)12%
Non-SCD
(189)68%
Unknow(56)20%
Death; 280; 16%
Alive; 1523; 84%
SCD = Suddent Cardiac Death
Mortality In ChildrenDCM
SCD All Other p SCD All Other pAge at diagnosis 4.7 ± 5.6 5.3 ± 6.1 0.738 Beta-blocker 0.09Male 54.3 53.5 0.88 Yes 2.9 7 Race / ethnicity 0.649 No 40 48 White 62.9 55.3 Unknow 57.1 45 Black 22.9 21 LV end-diastolic dimension Z-Score 4.2 ± 2.3 4.3 ± 2.4 0.928 Hispanic 11.4 16.9 LV end-systolic dimension Z-Score 5.9 ± 2.2 6.0 ± 2.5 0.883 Other 2.9 6.8 LV fractional shortening Z-Score -8.8 ± 2.5 -8.5 ± 3.4 Idiopathic 77.1 71.6 0.279 LV end-diastolic posterior wall thickness Z-
Score-1.1 ± 2.8 -0.5 ± 2.0 0.047
CHF at Diagnosis 86.7 72.6 0.031 NYHA functional class IV 0.139 LV end-diastolic septal wall thickness Z-
Score-1.1 ± 1.1 -0.8 ± 1.5 0.123
Yes 34.3 23.8 No 20 19.6 LV mass Z-Score 2.0 ± 2.7 2.3 ± 2.8 0.766 Unknow 45.7 56.5 LVEF Z-Score -6.9 ± 2.5 6.0 ± 2.4 0.123Familiy history of cardiomyopaty 0.551 Raw LVEF 23.3 ± 14.6 28.5 ± 13.7 0.073 yes 11.4 12.1 LVEF <35% 8 (80.0) 330 (67.8) 0.213 no 61.4 44.9 Raw LV fractional shortening (%) 15.3 ± 6.2 16.0 ± 8.3 0.298 unknow 37.1 43 LV fractional shortening <18% 27 (77.1) 1249 (73.0) 0.282Anticongestive therapy 0.905 Log (ratio of LV posterior wall thickness
end-diastolic dimension)-2.24 ± 0.38 -2.13 ± 0.32 0.016
Yes 5.7 6 No 54.3 52.7 Moderate to severe tricuspid regurgitation 0.078 Unknow 40 41.3 Yes 8.6 3.7 Antiarrhythmic therapy 0.025 No 14.3 26.3 Yes 20.0 12.1 Unknow 77.1 69.9 No 22.9 41.4 Moderate to severe mitral regurgitation 0.176 Unknow 57.1 46.5 Yes 14.3 9.3 ACE Inhibitor 0.023 No 11.4 20.9 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.
SCD All Other p SCD All Other pAge at diagnosis 4.7 ± 5.6 5.3 ± 6.1 0.738 Beta-blocker 0.09Male 54.3 53.5 0.88 Yes 2.9 7 Race / ethnicity 0.649 No 40 48 White 62.9 55.3 Unknow 57.1 45 Black 22.9 21 LV end-diastolic dimension Z-Score 4.2 ± 2.3 4.3 ± 2.4 0.928 Hispanic 11.4 16.9 LV end-systolic dimension Z-Score 5.9 ± 2.2 6.0 ± 2.5 0.883 Other 2.9 6.8 LV fractional shortening Z-Score -8.8 ± 2.5 -8.5 ± 3.4 Idiopathic 77.1 71.6 0.279 LV end-diastolic posterior wall thickness Z-
Score-1.1 ± 2.8 -0.5 ± 2.0 0.047
CHF at Diagnosis 86.7 72.6 0.031 NYHA functional class IV 0.139 LV end-diastolic septal wall thickness Z-
Score-1.1 ± 1.1 -0.8 ± 1.5 0.123
Yes 34.3 23.8 No 20 19.6 LV mass Z-Score 2.0 ± 2.7 2.3 ± 2.8 0.766 Unknow 45.7 56.5 LVEF Z-Score -6.9 ± 2.5 6.0 ± 2.4 0.123Familiy history of cardiomyopaty 0.551 Raw LVEF 23.3 ± 14.6 28.5 ± 13.7 0.073 yes 11.4 12.1 LVEF <35% 8 (80.0) 330 (67.8) 0.213 no 61.4 44.9 Raw LV fractional shortening (%) 15.3 ± 6.2 16.0 ± 8.3 0.298 unknow 37.1 43 LV fractional shortening <18% 27 (77.1) 1249 (73.0) 0.282Anticongestive therapy 0.375 Log (ratio of LV posterior wall thickness
end-diastolic dimension)-2.24 ± 0.38 -2.13 ± 0.32 0.016
Yes 5.7 6 No 54.3 52.7 Moderate to severe tricuspid regurgitation 0.078 Unknow 40 41.3 Yes 8.6 3.7 Antiarrhythmic therapy 0.025 No 14.3 26.3 Yes 20.0 12.1 Unknow 77.1 69.9 No 22.9 41.4 Moderate to severe mitral regurgitation 0.176 Unknow 57.1 46.5 Yes 14.3 9.3 ACE Inhibitor 0.023 No 11.4 20.9 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.
Transplantation
Non-SCD
SCD
DCM Survival
RISKSuddent Cardiac Death
FACTORPrediction From The Time of DCM Was Diagnosed
Associated No-Associated
• CHF at DCM diagnosis• Antiarrhythmic therapy• Lower Log (ratio of LV posterior wall
thickness end-diastolic dimension)• LV posterior wall thickness z-score
• Race• Sex• Cause of DCM• Family history of SCD or
cardiomyopaty• NYHA class• Anticongestive or B-blocker• Other echocardiograph finding
Multivariable CART Analysis
Prediction From The Time of DCM Was Diagnosed
HIGH RISK GROUP1. LV end-diastolic posterior wall
thickness z-score <-1.72. 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%.
Multivariable CART Analysis
Prediction From The Last Available Follow-up (64%)
HIGH RISK GROUPMeet 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%.
DISCUSSION
5 year Cumulative Incidence of SCD in children with DCM =
2.4 %
SCD INCIDENCE IN DCM CHILDREN
5 year Cumulative Incidence of SCD in
children with DCM = 2.4 %
SCD INCIDENCE IN DCM CHILDREN
CompareNo 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%)
INCHILDREN
DCM • SCD has lower incidence than adult• Death caused by progressive CHF is more
common• Children had fewer “ventricular arrhythmia”
5 year Cumulative Incidence of SCD in
children with DCM = 2.4 %
SCD INCIDENCE IN DCM CHILDREN
CompareNo 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%)
INCHILDREN
DCM • SCD has lower incidence than adult• Death caused by progressive CHF is more
common• Children had fewer “ventricular arrhythmia”
DIFFERENT NATURAL HISTORY THAN ADULT
DCM with SSD risk faktorConsidering for had ICDIMPLANTABLE CARDIOVERTER-DEFIBRILLATORS
(Criteria for ICD?)
First Diagnosed Last Available Follow-up
Prediction at Diagnosed Decision for ICD
DCM with SSD risk faktorConsidering for had ICDIMPLANTABLE CARDIOVERTER-DEFIBRILLATORS
(Criteria for ICD?)
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 wouldreceive an ICD at the time of diagnosis of cardiomyopathy
44% of subjects might receive an ICD
ICD For HighRisk Group of DCM ChildrenNot Yet Recommeded
Dubin et al (2003) :• incidence of inappropriate ICD discharge was 25%
Need further study for ICD safety
CRITICAL APPRAISAL
PICO
PICO
Children With Dilated Cardiomyopathy
High Risk Factor
No High Risk Factor
Sudden Cardiac Death
Are the results of this prognosis study valid?
1,083 defined sample
VA
LID
Defined and Representative
Sample
Long Follow-Up
Blind Outcome Criteria
Adjustment Important Factor
18 years folow-up
Sudden Cardiac Death(possible unblind)
2 subgroup criteria :- At diagnosed- Last Folow-up
Are the valid results of this prognosis study important?
SSD Cumulative Incidence Rate1-year 1.3%
3-year 2.0%
5-year 2.4%
(95% CI: 1.7% to 3.4%)
High Risk Group for SSD in DCM Children
At diagnosed
24 % had SSD
57% sensitivity and 78% specificity
Positive predictive value 5%, negative predictive value 99%
At last follow-up
44 % had SSD
86% sensitivity and 57% specificity
Positive predictive value 4%, negative predictive value 99%
Are the valid results of this prognosis study important?
High RiskN = 415
Non high Risk
N = 1332
SCD 20 15
Non-SCD 395 1317
OD RATIO 20 x 1317395 x 15
= 4.44
Can you apply this valid, important evidence about prognosis in caring for your patient?
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%
VALID IMPORTANT APLICABLE
CONCLUSION• SUDDEN CARDIAC DEATH IN CHILDREN WITH DILATED
CARDIOMYOPATHY HAD 5-YEAR CUMULATIVE INCIDENCE OF 2.4%
• DILATED CARDIOMYOPATHY CHILDREN WITH CRITERIA OF :First Diagnosed Last Available Follow-
up
1. LV end-diastolic posterior wall thickness z-score <-1.72. 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
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
HAD HIGH RISK OF SUDDEN CARDIAC DEATH
Recomendation• DILATED CARDIOMYOPATHY CHILDREN SHOULD BE DETECTED
FOR HIGH RISK GROUP• A HIGH RISK DILATED CARDIOMYOPATHY CHILDREN MAY
NEED IMPLANTABLE CARDIAC DEFIBRILATOR
THANK YOU
Implantable Cardiac Defibrillator