Abstract
Population-based studies have provided insight into the natural history of adult cardiomyopathy (CM), but comparable information for affected children is lacking. All Australian children diagnosed with primary CM at age 0-10 years between 1.1.1987 and 12.31.1996 were enrolled in a longitudinal cohort study. A single cardiologist and pathologist reviewed all cardiac investigations and histopathological material, respectively. During the study period, the overall incidence of CM was 1.24/105 person-years at risk. Indigenous children had a higher incidence of DCM (relative risk 2.8) and a higher rate of presenting death (16.7% vs. 2.6%). The 5-year freedom from death or transplantation was 63% for children with DCM (n = 184), 83% for those with HCM (n = 80), 53% for those with left ventricular non-compaction (LVNC) (n = 29) and 38% for those with RCM (n = 8). In subjects with DCM, positive viral identification and/or lymphocytic myocarditis were found in 30/44 (68.2%) cases with available early histology and 8/9 cases presenting with sudden death. Risk factors for death/transplantation comprised presenting age > 5 years, familial DCM, lower initial fractional shortening Z score and failure to increase fractional shortening Z score during follow-up. In subjects with HCM, an underlying syndromal, genetic or metabolic condition was identified in 46 (57.5%) of subjects. Risk factors for death/transplantation included concentric LV hypertrophy, presenting age < 1 year, lower initial fractional shortening Z score and increasing LV posterior wall Z score. Lymphocytic myocarditis and LVNC are important causes of childhood CM. This population-based study identifies pediatric subjects at risk of adverse events.
| Original language | English (US) |
|---|---|
| Pages (from-to) | 17-24 |
| Number of pages | 8 |
| Journal | Progress in Pediatric Cardiology |
| Volume | 23 |
| Issue number | 1-2 |
| DOIs | |
| State | Published - Sep 2007 |
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Keywords
- Cardiomyopathy
- Left ventricular non-compaction
- Myocarditis
- Sudden death
- Transplantation
ASJC Scopus subject areas
- Cardiology and Cardiovascular Medicine
Cite this
Pediatric cardiomyopathy : The Australian experience. / Weintraub, Robert G.; Nugent, Alan W.; Daubeney, Piers E F.
In: Progress in Pediatric Cardiology, Vol. 23, No. 1-2, 09.2007, p. 17-24.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Pediatric cardiomyopathy
T2 - The Australian experience
AU - Weintraub, Robert G.
AU - Nugent, Alan W.
AU - Daubeney, Piers E F
PY - 2007/9
Y1 - 2007/9
N2 - Population-based studies have provided insight into the natural history of adult cardiomyopathy (CM), but comparable information for affected children is lacking. All Australian children diagnosed with primary CM at age 0-10 years between 1.1.1987 and 12.31.1996 were enrolled in a longitudinal cohort study. A single cardiologist and pathologist reviewed all cardiac investigations and histopathological material, respectively. During the study period, the overall incidence of CM was 1.24/105 person-years at risk. Indigenous children had a higher incidence of DCM (relative risk 2.8) and a higher rate of presenting death (16.7% vs. 2.6%). The 5-year freedom from death or transplantation was 63% for children with DCM (n = 184), 83% for those with HCM (n = 80), 53% for those with left ventricular non-compaction (LVNC) (n = 29) and 38% for those with RCM (n = 8). In subjects with DCM, positive viral identification and/or lymphocytic myocarditis were found in 30/44 (68.2%) cases with available early histology and 8/9 cases presenting with sudden death. Risk factors for death/transplantation comprised presenting age > 5 years, familial DCM, lower initial fractional shortening Z score and failure to increase fractional shortening Z score during follow-up. In subjects with HCM, an underlying syndromal, genetic or metabolic condition was identified in 46 (57.5%) of subjects. Risk factors for death/transplantation included concentric LV hypertrophy, presenting age < 1 year, lower initial fractional shortening Z score and increasing LV posterior wall Z score. Lymphocytic myocarditis and LVNC are important causes of childhood CM. This population-based study identifies pediatric subjects at risk of adverse events.
AB - Population-based studies have provided insight into the natural history of adult cardiomyopathy (CM), but comparable information for affected children is lacking. All Australian children diagnosed with primary CM at age 0-10 years between 1.1.1987 and 12.31.1996 were enrolled in a longitudinal cohort study. A single cardiologist and pathologist reviewed all cardiac investigations and histopathological material, respectively. During the study period, the overall incidence of CM was 1.24/105 person-years at risk. Indigenous children had a higher incidence of DCM (relative risk 2.8) and a higher rate of presenting death (16.7% vs. 2.6%). The 5-year freedom from death or transplantation was 63% for children with DCM (n = 184), 83% for those with HCM (n = 80), 53% for those with left ventricular non-compaction (LVNC) (n = 29) and 38% for those with RCM (n = 8). In subjects with DCM, positive viral identification and/or lymphocytic myocarditis were found in 30/44 (68.2%) cases with available early histology and 8/9 cases presenting with sudden death. Risk factors for death/transplantation comprised presenting age > 5 years, familial DCM, lower initial fractional shortening Z score and failure to increase fractional shortening Z score during follow-up. In subjects with HCM, an underlying syndromal, genetic or metabolic condition was identified in 46 (57.5%) of subjects. Risk factors for death/transplantation included concentric LV hypertrophy, presenting age < 1 year, lower initial fractional shortening Z score and increasing LV posterior wall Z score. Lymphocytic myocarditis and LVNC are important causes of childhood CM. This population-based study identifies pediatric subjects at risk of adverse events.
KW - Cardiomyopathy
KW - Left ventricular non-compaction
KW - Myocarditis
KW - Sudden death
KW - Transplantation
UR - http://www.scopus.com/inward/record.url?scp=34547417531&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=34547417531&partnerID=8YFLogxK
U2 - 10.1016/j.ppedcard.2007.05.006
DO - 10.1016/j.ppedcard.2007.05.006
M3 - Article
VL - 23
SP - 17
EP - 24
JO - Progress in Pediatric Cardiology
JF - Progress in Pediatric Cardiology
SN - 1058-9813
IS - 1-2
ER -