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Suzanne M. Gilboa, Jason L. Salemi, Wendy N. Nembhard, David E.

Fixler and Adolfo Correa
United States, 1999 to 2006
Mortality Resulting From Congenital Heart Disease Among Children and Adults in the
Print ISSN: 0009-7322. Online ISSN: 1524-4539
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is published by the American Heart Association, 7272 Greenville Avenue, Dallas, TX 75231 Circulation
doi: 10.1161/CIRCULATIONAHA.110.947002
2010;122:2254-2263; originally published online November 22, 2010; Circulation. 
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Congenital Heart Disease
Mortality Resulting From Congenital Heart Disease Among
Children and Adults in the United States, 1999 to 2006
Suzanne M. Gilboa, PhD; Jason L. Salemi, MPH; Wendy N. Nembhard, PhD;
David E. Fixler, MD; Adolfo Correa, MD, PhD
Background—Previous reports suggest that mortality resulting from congenital heart disease (CHD) among infants and
young children has been decreasing. There is little population-based information on CHD mortality trends and patterns
among older children and adults.
Methods and Results—We used data from death certificates filed in the United States from 1999 to 2006 to calculate
annual CHD mortality by age at death, race-ethnicity, and sex. To calculate mortality rates for individuals Ն1 year of
age, population counts from the US Census were used in the denominator; for infant mortality, live birth counts were
used. From 1999 to 2006, there were 41 494 CHD-related deaths and 27 960 deaths resulting from CHD (age-
standardized mortality rates, 1.78 and 1.20 per 100 000, respectively). During this period, mortality resulting from CHD
declined 24.1% overall. Mortality resulting from CHD significantly declined among all race-ethnicities studied. However,
disparities persisted; overall and among infants, mortality resulting from CHD was consistently higher among non-Hispanic
blacks compared with non-Hispanic whites. Infant mortality accounted for 48.1% of all mortality resulting from CHD; among
those who survived the first year of life, 76.1% of deaths occurred during adulthood (Ն18 years of age).
Conclusions—CHD mortality continued to decline among both children and adults; however, differences between
race-ethnicities persisted. A large proportion of CHD-related mortality occurred during infancy, although significant
CHD mortality occurred during adulthood, indicating the need for adult CHD specialty management. (Circulation.
2010;122:2254-2263.)
Key Words: epidemiology

heart defects, congenital

mortality

race

vital statistics
A
mong infants and young children, congenital heart
disease (CHD) is responsible for the largest proportion,
30% to 50%, of mortality caused by birth defects.
1–4
Mortal-
ity resulting from CHD during infancy and childhood report-
edly is decreasing,
5
and the prevalence of CHD among adults
is increasing.
6,7
Until recently, limited population-based data
were available on CHD-related mortality through adult-
hood.
8,9
Documentation of survival into middle age or beyond
is typically available only from clinical or surgical series.
Survival analyses based on birth defects surveillance system
records linked to data from death certificates have been useful
in understanding infant and child mortality but generally have
not examined mortality into adulthood or trends in CHD-
related mortality over time.
10–14
Such information is needed
as a baseline for planning for and evaluating interventions to
decrease losses to specialty care follow-up among children
and adults with CHD
15
and for ongoing monitoring of CHD
mortality among adults.
Editorial see p 2231
Clinical Perspective on p 2263
The aims of the present study were to examine recent
temporal trends in mortality resulting from CHD from 1999
to 2006, to explore differences in CHD mortality by race-
ethnicity (non-Hispanic [NH] whites, NH blacks or African
Americans [referred to as NH blacks], Hispanics, and other
NH race-ethnicities), and to determine whether CHD mortal-
ity has declined to the same extent among all race-ethnicities.
Methods
Multiple cause of death (MCOD) public-use data files are released
annually by the National Center for Health Statistics. These files
were derived from compiled data collected from all death certificates
issued in the United States for deaths occurring in the United States.
Deaths occurring outside the country in US citizens and members of
the US Armed Forces were excluded. The data files included
demographic and geographic information on all decedents, Interna-
Received February 17, 2010; accepted August 17, 2010.
From the National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention, Atlanta, Ga (S.M.G., A.C.);
Department of Epidemiology and Biostatistics, College of Public Health, University of South Florida, Tampa (J.L.S., W.N.N.); and Division of
Cardiology, Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas (D.E.F.).
The findings and conclusions in this report are those of the authors and do not necessarily represent the official position of the Centers for Disease
Control and Prevention.
The online-only Data Supplement is available with this article at http://circ.ahajournals.org/cgi/content/full/CIRCULATIONAHA.110.947002/DC1.
Correspondence to Suzanne M. Gilboa, PhD, National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and
Prevention, Mail Stop E-86, 1600 Clifton Rd, Atlanta, GA 30333. E-mail sgilboa@cdc.gov
© 2010 American Heart Association, Inc.
Circulation is available at http://circ.ahajournals.org DOI: 10.1161/CIRCULATIONAHA.110.947002
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tional Classification of Disease (ICD) codes for the underlying cause
of death (UCOD), and up to 20 conditions listed as contributing
causes of death.
For these analyses, we used the MCOD data files from 1999 to
2006, which include ICD 10th revision (ICD-10)
16,17
codes (Q20 to
Q26) to identify the underlying and contributing causes of CHD
mortality (Appendix I in the online-only Data Supplement). Deaths
resulting from CHD were defined as those with a CHD listed as the
UCOD; CHD-related deaths had a CHD listed as an underlying or
contributing cause of death. Although the majority of analyses
reported here are based on mortality resulting from CHD, we also
report selected information on CHD-related mortality. The denomi-
nators for mortality rates for those Ն1 year of age were the US
bridged-race postcensal population estimates for each year of inter-
est.
18
For infant mortality rates, live birth data from the National
Center for Health Statistics were used in the denominators.
19
Annual age-specific death rates for any CHD and specific CHD
diagnoses were calculated per 100 000 population among the fol-
lowing age groups: Ͻ1, 1 to 4, 5 to 17, 18 to 34, 35 to 49, 50 to 64,
and Ն65 years of age, stratified by sex and race-ethnicity (NH white,
NH black, Hispanic, other NH race-ethnicity [ie, Alaska Native,
American Indian or Native American, Asian, Native Hawaiian, or
other Pacific Islander]). To account for the different age composi-
tions among these subpopulations, we adjusted the overall, race-
ethnicity–specific, and sex-specific mortality rates using direct
standardization by applying the age-specific mortality rates to the US
standard population for the year 2000.
20
We calculated the overall
percentage of change in the mortality rates over the time period of
interest by subtracting the rate in 1999 from the rate in 2006 and
dividing by the rate in 1999. To calculate the average annual
percentage of change and to test the hypothesis that this change was
equal to zero, a weighted least squares regression model was fit to
the natural logarithm of the rate, with the calendar year of death used
as the independent variable. To quantify differences by race-
ethnicity, we calculated mortality rate ratios and their accompanying
95% confidence intervals comparing NH blacks and Hispanics with
NH whites. We also calculated mortality rate ratios comparing age
groups and sexes. Finally, we calculated the median age at death by
CHD cause of death, by demographic characteristics, and over time.
Results
Overall Mortality
In the United States from 1999 to 2006, there were 19.4
million deaths. CHD-related deaths accounted for 0.21% of
all mortality (nϭ41 494); of these, CHD was listed as the
UCOD in 27 960 deaths (67.4% of CHD-related deaths). The
age-standardized mortality rates were 1.78 and 1.20 per
100 000 for CHD-related mortality and mortality resulting
from CHD, respectively (Table 1). Mortality resulting from
CHD was highest among NH blacks compared with other
race-ethnicities. Mortality caused by CHD was also higher
among male than female individuals (Table 1). Unspecified
CHD (34.1%) and other specified CHD (18.8%) contributed
the most to overall CHD mortality, although among specific
diagnoses, hypoplastic left heart syndrome (HLHS) was the
greatest contributor (10.9%; Table 1).
Mortality resulting from CHD declined 24.1% overall and
3.6% annually during the period of 1999 to 2006 (PϽ0.01;
Table 3). Mortality declined among both male and female
individuals (Table 3) and among all 4 race-ethnicities studied
(Figure 1 and Table 3). However, despite these declines, the
overall mortality among NH blacks was consistently higher
than among NH whites and was higher among NH whites
than among Hispanics (Table 2; Figure 1 and Appendix II in
the online-only Data Supplement).
Age-Specific Mortality
We observed a reverse J-shaped pattern of age-specific
mortality resulting from CHD (Table 1 and Figure 2). CHD
mortality was highest among infants and lowest among
children 5 to17 years of age (Table 1). There was a 34%
increase in mortality among adults 18 to 34 years of age
compared with children 5 to17 years of age (mortality rate
ratioϭ1.34; 95% confidence intervalϭ1.27 to 1.43; calcula-
tion not shown). Mortality resulting from CHD was un-
changed among adults 18 to 64 years of age, with a marked
increase among individuals Ն65 years of age.
Infant Mortality
Infant mortality accounted for 48.1% (13 449 of 27 960) of
all mortality caused by CHD (Table 1). Throughout the study
period, infant mortality resulting from CHD was higher
among male than among female individuals (Table 3). Infant
mortality caused by CHD decreased by 17.3% (PϽ0.01)
overall and 2.8% annually during the study period and
decreased significantly among NH whites and Hispanics
(Table 3). NH blacks had consistently higher mortality
resulting from CHD than NH whites (Appendix II in the
online-only Data Supplement). There was no evidence of a
disparity between Hispanics and NH whites (Appendix II in
the online-only Data Supplement).
Mortality Among Children
Among children 1 to 4 years of age, mortality caused by CHD
decreased 21.0% overall and 2.8% annually (Pϭ0.05; Table
3). However, no race-ethnicity studied experienced a statis-
tically significant change in mortality. Except for 2002 and
2005, there were significant differences between NH blacks
and NH whites in mortality resulting from CHD among
children in this age group (Appendix II in the online-only
Data Supplement). There was little evidence of a disparity
between Hispanics and NH whites.
Among children 5 to17 years of age, mortality caused by
CHD declined 36.1% overall and 4.8% annually during the
study period (Pϭ0.02). The magnitudes of the CHD mortality
decrease among the different race-ethnicities were very sim-
ilar, yet only NH whites experienced a statistically significant
decrease; the decrease among NH blacks was of borderline
significance (Pϭ0.06). CHD mortality decreased signifi-
cantly among male but not among female individuals (Table
3). NH blacks had consistently higher mortality resulting
from CHD than NH whites; there were no differences
between Hispanics and NH whites (Appendix II in the
online-only Data Supplement).
Overall, mortality among children 1 to 17 years of age
accounted for 12.4% of all mortality caused by CHD (3471 of
27 960) and for 23.9% of mortality resulting from CHD
among children who survived the first year of life (3471 of
14 511). Therefore, mortality among adults Ն18 years of age
accounted for 76.1% of mortality resulting from CHD among
individuals who survived infancy.
Mortality Among Adults
From 1999 to 2006, mortality resulting from CHD decreased
21.4% overall and 3.0% annually (Pϭ0.01) among adults 18
to 34 years of age. This decrease was driven by a significant
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decline among NH whites. CHD mortality decreased signif-
icantly among women but not among males (Table 3).
Although CHD mortality in this age group was consistently
higher among NH blacks than NH whites throughout the
study period, the disparity was statistically significant only in
2003 and 2005. Hispanics had consistently lower mortality
resulting from CHD than NH whites (Appendix II in the
online-only Data Supplement).
Among adults 35 to 49 years of age, mortality resulting
from CHD decreased 24.6% overall and 3.5% annually
(Pϭ0.02) and decreased significantly among both NH whites
and NH blacks. CHD mortality also decreased significantly
among men (Table 3). There is no evidence of a difference in
CHD mortality between NH blacks and NH whites. Similar to
the pattern among adults 18 to 34 years of age, Hispanics 35
to 49 years of age had lower mortality resulting from CHD
than NH whites of the same age (Appendix II in the
online-only Data Supplement).
Among adults 50 to 64 years of age, mortality caused by
CHD declined 27.7% overall and 4.4% annually (PϽ0.01)
during the study period. Among subgroups, only NH whites
experienced a significant decline, and mortality decreased
significantly among both men and women (Table 3). Overall
and during 1999 to 2001, Hispanics had significantly lower
mortality resulting from CHD than NH white adults, but
beginning in 2002, their mortalities resulting from CHD were
statistically equivalent (Appendix II in the online-only Data
Supplement).
Among adults Ն65 years of age, mortality caused by CHD
declined significantly during the study period (42.7% overall,
Table 1. Overall CHD-Related Mortality and Overall and Age-Specific (Ages 17 and Younger) Mortality Resulting From CHD, United
States, 1999–2006
CHD*
Mortality Related
to CHD, All Ages
Mortality Resulting From CHD
Age at Death, y
All Ages 0–Ͻ1 1–4 5–17
n Rate† n Rate† n Rate‡ n Rate§ n Rate§
Any CHD 41 494 1.78 27 960 1.20 13 449 41.46 1729 1.38 1742 0.41
Race-ethnicity
NH white 26 504 1.76 17 731 1.19 7300 39.37 867 1.18 993 0.38
NH black 6933 2.19 4739 1.49 2600 55.11 382 1.98 413 0.62
Hispanic 6449 1.53 4408 1.04 2952 40.89 375 1.44 255 0.34
Other NH 1608 1.27 1082 0.85 597 30.45 105 1.57 81 0.37
Sex
Male 21 848 1.89 15 020 1.29 7398 44.56 960 1.49 1040 0.48
Female 19 646 1.67 12 940 1.10 6051 38.21 769 1.25 702 0.34
Anomalous pulmonary venous
connection
592 0.03 300 0.01 268 0.83 20 0.02 4 0.00
Aortic valve anomalies 700 0.03 460 0.02 247 0.76 12 0.01 23 0.01
Atrial septal defect 3931 0.17 2098 0.09 167 0.51 24 0.02 35 0.01
Atrioventricular septal defect 959 0.04 466 0.02 278 0.86 58 0.05 27 0.01
Coarctation of the aorta 1189 0.05 428 0.02 263 0.81 7 0.01 24 0.01
Common truncus 522 0.02 405 0.02 314 0.97 19 0.02 23 0.01
Common ventricle 320 0.01 215 0.01 122 0.38 27 0.02 25 0.01
Ebstein anomaly 505 0.02 393 0.02 226 0.70 12 0.01 26 0.01
HLHS 3657 0.16 3043 0.13 2781 8.57 180 0.14 65 0.02
Patent ductus arteriosus 2206 0.09 507 0.02 424 1.31 6 0.00 7 0.00
Pulmonary artery atresia/stenosis 1756 0.08 688 0.03 395 1.22 82 0.07 45 0.01
Pulmonary valve anomalies 170 0.01 69 0.00 52 0.16 5 0.00 1 0.00
Tetralogy of Fallot 2214 0.10 1472 0.06 569 1.75 157 0.12 115 0.03
Transposition of the great arteries 1469 0.06 1006 0.04 623 1.92 53 0.04 75 0.02
Tricuspid valve anomalies 317 0.01 231 0.01 89 0.27 17 0.01 27 0.01
Ventricular septal defect 3422 0.15 1394 0.06 342 1.05 44 0.03 60 0.01
Other specified CHD 7220 0.31 5242 0.23 1104 3.40 149 0.12 390 0.09
Unspecified CHD 13 274 0.57 9543 0.41 5185 15.98 857 0.68 770 0.18
*See Appendix I in the online-only Data Supplement for ICD-10 codes for CHD categories.
†Age-standardized mortality rates.
‡Infant mortality rate calculated per 100 000 live births.
§Mortality rates among individuals Ͼ1 year of age calculated per 100 000 population of that age.
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7.7% annually; PϽ0.01). Significant declines were seen for
NH whites and Hispanics; the magnitude of the mortality
decrease among NH blacks was comparable but had border-
line statistical significance (Pϭ0.06). Mortality also declined
for both men and women (Table 3). Consistently over time,
NH blacks Ն65 years of age experienced less mortality
caused by CHD than NH whites; a similar pattern was seen
when Hispanics with NH whites were compared (Appendix II
in the online-only Data Supplement). Mortality resulting from
CHD was greater for male than female individuals among
those Ͻ65 years of age, but among adults Ն65 years of age,
CHD mortality was lower for men (mortality rate ratioϭ0.84;
95% confidence intervalϭ0.81 to 0.87) (data not shown).
Median Age at Death
Median age at death for all CHD combined was 1 year
(Figure 3); this did not change over the study period, nor did
it vary by sex (data not shown). The median age at death,
however, varied substantially by CHD diagnosis: from Ͻ1
month (eg, patent ductus arteriosus, 11 days; HLHS, 14 days;
and common truncus, 30 days) to Ͼ40 years (ventricular
septal defect, 44 years; and atrial septal defect, 72 years;
Figure 3). For most diagnoses, there was no change in the
median age at death over the study period. For other, less
common phenotypes, the median age at death did vary over
time, although there were no distinctive patterns of increasing
or decreasing median age at death. Finally, the median age at
death varied by race-ethnicity (data not shown).
Discussion
The patterns of CHD mortality in the United States from 1999
through 2006 described here provide an update to previous
studies.
5,8,21
Consistent with earlier literature, our study
showed significant declines in both overall and infant mor-
tality resulting from CHD. In addition, we confirmed excess
CHD mortality among NH blacks compared with NH whites,
overall and during infancy and early childhood. Nearly half of
all mortality caused by CHD occurred during infancy; among
those who survive the first year of life, Ϸ76% of mortality
resulting from CHD occurs in adulthood. Compared with all
Table 2. Age-Specific (Ages 18 and Older) Mortality Resulting from CHD, United States, 1999–2006
CHD
Age at Death, y
18–34 35–49 50–64 Ն65
n Rate§ n Rate§ n Rate* n Rate§
Any CHD 3014 0.55 2880 0.55 1984 0.54 3162 1.10
Race-ethnicity
NH white 1982 0.58 2197 0.59 1611 0.56 2781 1.17
NH black 558 0.75 390 0.61 206 0.55 190 0.80
Hispanic 365 0.38 218 0.35 119 0.41 124 0.77
Other NH 109 0.32 75 0.26 48 0.28 67 0.71
Sex
Male 1864 0.67 1551 0.59 1025 0.57 1182 0.99
Female 1150 0.43 1329 0.50 959 0.50 1980 1.18
Anomalous pulmonary venous
connection
0 0.00 1 0.00 2 0.00 5 0.00
Aortic valve anomalies 40 0.01 43 0.01 27 0.01 68 0.02
Atrial septal defect 107 0.02 209 0.04 307 0.08 1249 0.44
Atrioventricular septal defect 46 0.01 34 0.01 20 0.01 3 0.00
Coarctation of the aorta 44 0.01 40 0.01 29 0.01 21 0.01
Common truncus 23 0.00 17 0.00 4 0.00 5 0.00
Common ventricle 25 0.00 13 0.00 3 0.00 0 0.00
Ebstein anomaly 32 0.01 33 0.01 25 0.01 39 0.01
HLHS 12 0.00 3 0.00 0 0.00 2 0.00
Patent ductus arteriosus 14 0.00 14 0.00 8 0.00 34 0.01
Pulmonary artery atresia/stenosis 36 0.01 27 0.01 26 0.01 77 0.03
Pulmonary valve anomalies 5 0.00 0 0.00 4 0.00 2 0.00
Tetralogy of Fallot 219 0.04 217 0.04 131 0.04 64 0.02
Transposition of the great arteries 159 0.03 50 0.01 29 0.01 17 0.01
Tricuspid valve anomalies 61 0.01 30 0.01 7 0.00 0 0.00
Ventricular septal defect 125 0.02 202 0.04 185 0.05 436 0.15
Other specified CHD 874 0.16 1157 0.22 753 0.20 815 0.28
Unspecified CHD 1192 0.22 790 0.15 424 0.11 325 0.11
*Mortality rates among individuals Ͼ1 year of age calculated per 100 000 population of that age.
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Table 3. Annual Mortality Rates* Resulting From CHD by Age Group, Race-Ethnicity, and Sex, United States, 1999–2006
Year of Death Change, %
P§ 1999 2000 2001 2002 2003 2004 2005 2006 Overall† Per Year‡
All ages࿣
Overall 1.37 1.31 1.25 1.24 1.18 1.12 1.11 1.04 Ϫ24.1 Ϫ3.6 Ͻ0.01
Race-ethnicity
NH white 1.36 1.28 1.22 1.21 1.17 1.13 1.11 1.01 Ϫ25.7 Ϫ4.0 Ͻ0.01
NH black 1.67 1.60 1.57 1.50 1.46 1.41 1.31 1.41 Ϫ15.6 Ϫ3.2 Ͻ0.01
Hispanic 1.20 1.16 1.04 1.14 0.99 0.96 1.00 0.86 Ϫ28.3 Ϫ3.1 Ͻ0.01
Other NH 0.88 0.97 0.85 0.96 0.92 0.71 0.77 0.75 Ϫ14.8 Ϫ3.4 0.03
Sex
Male 1.46 1.41 1.35 1.33 1.26 1.22 1.19 1.16 Ϫ20.5 Ϫ3.2 Ͻ0.01
Female 1.27 1.21 1.14 1.15 1.10 1.03 1.02 0.92 Ϫ27.6 Ϫ4.2 Ͻ0.01
Age at death, Ͻ1 y
Overall 45.56 45.07 42.76 42.36 40.54 39.50 38.61 37.69 Ϫ17.3 Ϫ2.8 Ͻ0.01
Race-ethnicity
NH white 44.31 42.53 39.54 38.38 38.32 38.92 37.54 35.21 Ϫ20.5 Ϫ2.6 Ͻ0.01
NH black 57.04 57.75 56.96 58.10 52.94 56.31 47.95 53.77 Ϫ5.7 Ϫ1.8 0.07
Hispanic 45.08 44.98 44.84 44.37 39.99 34.65 38.91 36.82 Ϫ18.3 Ϫ3.6 0.01
Other NH 29.20 38.25 31.32 35.33 34.63 24.82 25.81 25.52 Ϫ12.6 Ϫ3.9 0.10
Sex
Male 48.99 46.81 46.25 45.43 44.86 42.07 41.38 41.10 Ϫ16.1 Ϫ2.5 Ͻ0.01
Female 41.96 43.25 39.11 39.15 36.01 36.80 35.70 34.11 Ϫ18.7 Ϫ3.1 Ͻ0.01
Age at death, 1–4 y
Overall 1.45 1.42 1.40 1.38 1.60 1.35 1.25 1.15 Ϫ21.0 Ϫ2.8 0.05
Race-ethnicity
NH white 1.14 1.07 1.23 1.34 1.33 1.11 1.24 0.95 Ϫ16.6 Ϫ1.3 0.51
NH black 2.43 2.34 2.05 1.42 2.55 1.82 1.52 1.72 29.0 Ϫ4.9 0.16
Hispanic 1.61 1.79 1.29 1.46 1.70 1.56 1.00 1.21 Ϫ25.1 Ϫ5.2 0.08
Other NH 1.67 1.39 1.89 1.48 1.44 1.74 1.67 1.31 Ϫ22.0 Ϫ1.3 0.53
Sex
Male 1.64 1.52 1.49 1.65 1.62 1.53 1.29 1.23 Ϫ25.5 Ϫ3.4 0.03
Female 1.25 1.32 1.31 1.11 1.59 1.17 1.21 1.07 Ϫ14.8 Ϫ1.9 0.31
Age at death, 5–17 y
Overall 0.47 0.46 0.41 0.43 0.40 0.41 0.41 0.3 Ϫ36.1 Ϫ4.8 0.02
Race-ethnicity
NH white 0.43 0.43 0.38 0.41 0.37 0.37 0.33 0.3 Ϫ30.3 Ϫ4.8 Ͻ0.01
NH black 0.69 0.71 0.66 0.58 0.57 0.65 0.69 0.44 Ϫ37.3 Ϫ4.8 0.06
Hispanic 0.42 0.32 0.29 0.40 0.34 0.37 0.40 0.21 Ϫ51.3 Ϫ5.2 0.19
Other NH 0.42 0.49 0.33 0.29 0.40 0.32 0.46 0.25 Ϫ41.5 Ϫ5.1 0.17
Sex
Male 0.58 0.53 0.51 0.49 0.46 0.47 0.45 0.33 Ϫ43.3 Ϫ6.3 Ͻ0.01
Female 0.35 0.39 0.30 0.36 0.33 0.36 0.36 0.27 Ϫ23.2 Ϫ2.3 0.27
Age at death, 18–34 y
Overall 0.62 0.59 0.58 0.58 0.49 0.52 0.55 0.49 Ϫ21.4 Ϫ3.0 0.01
Race-ethnicity
NH white 0.66 0.66 0.62 0.62 0.50 0.56 0.56 0.47 Ϫ28.4 Ϫ4.3 0.01
NH black 0.71 0.74 0.82 0.76 0.73 0.74 0.78 0.73 2.5 0.1 0.85
Hispanic 0.45 0.34 0.33 0.41 0.32 0.33 0.44 0.38 Ϫ15.2 Ϫ0.2 0.92
Other NH 0.47 0.27 0.34 0.24 0.30 0.25 0.30 0.43 Ϫ8.1 Ϫ1.0 0.82
(Continued)
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causes of mortality, for which this latter statistic is nearly
99%, mortality resulting from CHD disproportionately affects
children. A previous report showed that the median age of
death caused by CHD increased from 6 to 12 months between
1979 and 1997.
5
During the present study period of 1999 to
2006, we found the median age at death for all CHD
unchanged at 12 months. However, the median age at death
varied widely by CHD diagnosis, ranging from Ͻ1 month to
Ͼ70 years.
Our results extend previous findings in several important
ways. We reported that children of all ages have experienced
a significant decline in CHD mortality. In addition, adults in
all age groups have experienced a significant decline in
mortality resulting from CHD. Overall, infant and child
mortality resulting from CHD was higher among NH blacks
than among NH whites, and this difference persisted over
time. The difference between Hispanics and NH whites was
less pronounced, was inconsistent across age groups, and
tended to show greater mortality resulting from CHD among
NH whites than among Hispanics. Interestingly, among
adults Ն65 years of age, we observed lower mortality caused
by CHD among NH blacks compared with NH whites. This
Table 3. Continued
Year of Death Change, %
P§ 1999 2000 2001 2002 2003 2004 2005 2006 Overall† Per Year‡
Sex
Male 0.68 0.72 0.70 0.72 0.59 0.63 0.67 0.65 Ϫ4.3 Ϫ1.4 0.21
Female 0.56 0.46 0.46 0.42 0.38 0.41 0.43 0.32 Ϫ43.5 Ϫ5.7 0.01
Age at death, 35–49 y
Overall 0.64 0.56 0.57 0.58 0.56 0.46 0.53 0.48 Ϫ24.6 Ϫ3.5 0.02
Race-ethnicity
NH white 0.69 0.59 0.60 0.63 0.63 0.50 0.57 0.53 Ϫ22 Ϫ2.9 0.05
NH black 0.70 0.72 0.64 0.61 0.66 0.48 0.62 0.45 Ϫ35 Ϫ5.7 0.01
Hispanic 0.39 0.40 0.38 0.34 0.22 0.30 0.40 0.35 Ϫ11.2 Ϫ3.2 0.42
Other NH 0.32 0.18 0.29 0.34 0.25 0.24 0.24 0.25 Ϫ20.2 Ϫ0.2 0.96
Sex
Male 0.68 0.63 0.61 0.59 0.60 0.53 0.56 0.54 Ϫ20.3 Ϫ3.0 Ͻ0.01
Female 0.60 0.50 0.53 0.56 0.52 0.38 0.50 0.42 Ϫ29.4 Ϫ4.1 0.07
Age at death, 50–64 y
Overall 0.65 0.59 0.57 0.55 0.52 0.52 0.47 0.47 Ϫ27.7 Ϫ4.4 Ͻ0.01
Race-ethnicity
NH white 0.67 0.64 0.59 0.57 0.54 0.57 0.49 0.47 Ϫ29.9 Ϫ4.6 Ͻ0.01
NH black 0.74 0.57 0.73 0.52 0.46 0.32 0.37 0.72 Ϫ2.8 Ϫ8.0 0.13
Hispanic 0.38 0.29 0.33 0.43 0.48 0.48 0.52 0.31 Ϫ17.1 3.2 0.38
Other NH 0.47 0.33 0.15 0.44 0.28 0.22 0.25 0.20 Ϫ58.6 Ϫ5.2 0.42
Sex
Male 0.72 0.61 0.58 0.56 0.51 0.57 0.53 0.54 Ϫ25.8 Ϫ3.4 0.03
Female 0.59 0.57 0.55 0.54 0.53 0.47 0.41 0.41 Ϫ29.8 Ϫ5.5 Ͻ0.01
Age at death, Ն65 y
Overall 1.45 1.30 1.18 1.20 1.09 0.94 0.86 0.83 Ϫ42.7 Ϫ7.7 Ͻ0.01
Race-ethnicity
NH white 1.50 1.35 1.27 1.24 1.17 1.00 0.95 0.87 Ϫ41.8 Ϫ7.3 Ͻ0.01
NH black 1.39 0.88 0.76 0.89 0.67 0.63 0.39 0.87 Ϫ37.7 Ϫ10.7 0.06
Hispanic 1.21 1.25 0.65 1.07 0.63 0.83 0.48 0.29 Ϫ75.8 Ϫ17.0 Ͻ0.01
Other NH 0.42 0.89 0.65 1.06 0.92 0.47 0.60 0.71 66.5 2.9 0.59
Sex
Male 1.25 1.35 1.08 0.99 0.87 0.83 0.77 0.83 Ϫ33.6 Ϫ7.4 Ͻ0.01
Female 1.59 1.26 1.25 1.34 1.25 1.03 0.93 0.83 Ϫ47.7 Ϫ7.8 Ͻ0.01
*Mortality rate calculated per 100 000 population in the age group.
†Overall percent change computed as follows: ͓(mortality rate in 2006Ϫmortality rate in 1999)/(mortality rate in 1999)͔ϫ100.
‡Average annual percent change computed from weighted least squares model regressing natural log of the mortality rate on the year of death.
§P for weighted least squares model regressing natural log of the mortality rate on the year of death.
࿣Mortality rates for “all ages” are age standardized. All other mortality rates are age specific.
¶Infant mortality rate calculated per 100 000 live births.
Gilboa et al Mortality From Congenital Heart Disease 2259
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mortality crossover
22,23
is seen for other causes of death;
compared with NH whites, NH blacks tend to have higher
mortality as infants and children and lower mortality as older
adults. Less severe CHD that does not cause early mortality
(but may be responsible for later mortality) may be diagnosed
less frequently in NH blacks (or Hispanics) than NH whites.
This might ultimately be reflected in differential misclassifi-
cation of UCOD by race-ethnicity. These analyses assumed
homogeneity within population subgroups and did not at-
tempt to separate out mortality patterns by place of birth (US
or foreign born), or among Hispanics, by country of origin.
Despite the analytic potential of MCOD,
24
analyses using
death certificate data have limitations,
25,26
and interpretation
of these results should be done with caution. We report on
results using the UCOD for most analyses. Unfortunately, the
UCOD was often nonspecific; 34% of all mortality resulting
from CHD was coded as unspecified CHD. Supplemental
analysis of the associated causes of death among this sub-
population showed I46.9 (cardiac arrest, unspecified) as the
most common associated cause of death. Other frequently
reported associated causes included I50.0 (congestive heart
failure), I49.9 (cardiac arrhythmia, unspecified), and P29.1
(neonatal cardiac dysrhythmia).
For the MCOD data files, the UCOD is selected using the
Automated Classification of Medical Entities, a computer
program developed by the National Center for Health Statis-
tics to standardize the assignment of the UCOD.
27
Despite the
development of rules and decision tables to identify “accept-
able” causal relationships between cause of death codes to
ultimately select the correct UCOD, the data are imperfect
and are limited by what was recorded on the death certificate
itself. Compared with the gold standard of autopsy, one study
showed that death certificates had a 52% sensitivity for
recording mortality caused by myocardial infarction.
26
This
Figure 1. Annual age-standardized mor-
tality resulting from CHD, by race-
ethnicity, United States, 1999–2006.
Figure 2. Age-specific mortality resulting
from CHD, by race-ethnicity, United
States, 1999–2006.
2260 Circulation November 30, 2010
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poor sensitivity may be related to the inadequate training of
physicians in the completion of death certificates; a study
investigating the ability of resident physicians to correctly
complete death certificates found that 45% of respondents
incorrectly listed the primary cause of death, and this error
was associated with previous experience completing death
certificates.
25
In our data, the 5 deaths reportedly resulting
from HLHS among adults Ն35 years of age (Table 1) might
represent coding errors; the first successful Norwood pallia-
tion of HLHS was completed in 1981.
28
Before this time,
HLHS was generally fatal during infancy.
Because CHD mortality is a function of both the underly-
ing prevalence of CHD and the case fatality associated with
them, it is impossible to know whether these patterns reflect
changes in one or both of these components. Recent data
indicate that the prevalence of CHD increased through the
1990s into the early 21st century,
29,30
probably in part as a
result of changes in diagnosis and ascertainment, most
notably the use of echocardiography in infants
31
and im-
proved ascertainment of milder lesions. In light of this, the
pattern of decreasing mortality among infants and young
children implies an improvement in case fatality, and not a
reduction in prevalence. In the case of HLHS, a severe lesion,
we observed a decrease in infant mortality (Appendix III in
the online-only Data Supplement) yet observed an increase in
mortality among children 1 to 4 years of age (data not
shown), suggesting delayed mortality. Other data show that
the prevalence of HLHS has been stable over time.
29
The classification of CHD causes of death in MCOD data
imposes additional uncertainty and the necessity for cautious
interpretation of these results. Although an improvement over
ICD-9 with the addition of Ϸ3000 categories for causes of
death,
32
ICD-10 is still a crude tool for classifying CHD
mortality. For research (National Birth Defects Prevention
Study)
33
and surveillance (Metropolitan Atlanta Congenital
Defects Program)
34,35
purposes, other CHD classification
systems have been implemented that have been able to take
advantage of verbatim and other information available
through the medical record (eg, method of diagnosis and
presence of complex cardiac defects, extracardiac defects, or
both), but they were impossible to overlay on the MCOD
data.
The overall reduction in mortality resulting from CHD
likely resulted from improved diagnostic capacities, surgical
techniques, and catheter-based treatments.
31
There is debate
as to whether prenatal diagnosis of CHD improves survival.
36
Some studies report that prenatal detection of CHD is
associated with decreased morbidity and mortality.
37–39
Pre-
natal diagnosis may also be associated with the termination of
fetuses affected by CHD, which could lead to spurious
findings of a decrease in CHD mortality. Although data from
population-based surveillance systems suggest that termina-
tion of fetuses with CHD is not responsible for a large
decrease in the live birth prevalence,
40
the frequency of
pregnancy termination varies widely by CHD diagnosis and
other conditions (eg, trisomies 13, 18, and 21).
41–44
In
Figure 3. Median age (in days) at death resulting from CHD, by UCOD, United States, 1999–2006.
Gilboa et al Mortality From Congenital Heart Disease 2261
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addition, there are known differences in pregnancy termina-
tion for chromosomal anomalies by race-ethnicity,
43,44
which,
if relevant to CHD, could contribute to some of the differ-
ences reported here.
CHD mortality continues to decline, and people with
CHD are living longer, making managing care into adult-
hood increasingly important.
45,46
In addition to the man-
agement of the cardiac sequelae of CHD (eg, hypertension,
cardiac arrhythmias, and endocarditis), effective care of
adults with CHD is likely to require attention to the
diagnosis and management of noncardiac organ dysfunc-
tion such as renal impairment
47
and abnormal glucose
metabolism,
47,48
as well as counseling on issues such as
contraception and pregnancy, potential genetic transmis-
sion of CHD, dental care, diet, optimal weight, exercise,
and physical activity.
46
Disclosures
None.
References
1. Petrini J, Damus K, Johnston RB Jr. An overview of infant mortality and
birth defects in the United States. Teratology. 1997;56:8–10.
2. Petrini J, Damus K, Russell R, Poschman K, Davidoff MJ, Mattison D.
Contribution of birth defects to infant mortality in the United States.
Teratology. 2002; 66(suppl 1):S3–S6.
3. Yang Q, Khoury MJ, Mannino D. Trends and patterns of mortality
associated with birth defects and genetic diseases in the United States,
1979–1992: an analysis of multiple-cause mortality data. Genet Epi-
demiol. 1997;14:493–505.
4. Yang Q, Chen H, Correa A, Devine O, Mathews TJ, Honein MA. Racial
differences in infant mortality attributable to birth defects in the United
States, 1989–2002. Birth Defects Res A Clin Mol Teratol. 2006;76:
706–713.
5. Boneva RS, Botto LD, Moore CA, Yang Q, Correa A, Erickson JD.
Mortality associated with congenital heart defects in the United States:
trends and racial disparities, 1979–1997. Circulation. 2001;103:
2376–2381.
6. Marelli AJ, Mackie AS, Ionescu-Ittu R, Rahme E, Pilote L. Congenital
heart disease in the general population: changing prevalence and age
distribution. Circulation. 2007;115:163–172.
7. Warnes CA, Liberthson R, Danielson GK, Dore A, Harris L, Hoffman JI,
Somerville J, Williams RG, Webb GD. Task Force 1: the changing profile
of congenital heart disease in adult life. J Am Coll Cardiol. 2001;37:
1170–1175.
8. Nembhard WN, Pathak EB, Schocken DD. Racial/ethnic disparities in
mortality related to congenital heart defects among children and adults in
the United States. Ethn Dis. 2008;18:442–449.
9. Pillutla P, Shetty KD, Foster E. Mortality associated with adult congenital
heart disease: trends in the US population from 1979 to 2005. Am Heart J.
2009;158:874–879.
10. Cleves MA, Ghaffar S, Zhao W, Mosley BS, Hobbs CA. First-year
survival of infants born with congenital heart defects in Arkansas
(1993–1998): a survival analysis using registry data. Birth Defects Res A
Clin Mol Teratol. 2003;67:662–668.
11. Nembhard WN, Waller DK, Sever LE, Canfield MA. Patterns of
first-year survival among infants with selected congenital anomalies in
Texas, 1995–1997. Teratology. 2001;64:267–275.
12. Agha MM, Williams JI, Marrett L, To T, Dodds L. Determinants of
survival in children with congenital abnormalities: a long-term
population-based cohort study. Birth Defects Res A Clin Mol Teratol.
2006;76:46–54.
13. Copeland GE, Kirby RS. Using birth defects registry data to evaluate
infant and childhood mortality associated with birth defects: an alter-
native to traditional mortality assessment using underlying cause of death
statistics. Birth Defects Res A Clin Mol Teratol. 2007;79:792–797.
14. Dadvand P, Rankin J, Shirley MD, Rushton S, Pless-Mulloli T.
Descriptive epidemiology of congenital heart disease in Northern
England. Paediatr Perinat Epidemiol. 2009;23:58–65.
15. Mackie AS, Ionescu-Ittu R, Therrien J, Pilote L, Abrahamowicz M,
Marelli AJ. Children and adults with congenital heart disease lost to
follow-up: who and when? Circulation. 2009;120:302–309.
16. World Health Organization. International Statistical Classification of
Diseases and Related Health Problems. 10th revision. Geneva, Swit-
zerland: World Health Organization; 1992.
17. National Center for Health Statistics. Vital statistics data available on
line: multiple cause-of-death public use data files. Available at: http://
www.cdc.gov/nchs/products/elec_prods/subject/mortmcd.htm. Accessed
January 11, 2010.
18. Ingram DD, Parker JD, Schenker N, Weed JA, Hamilton B, Arias E,
Madans JH. United States Census 2000 population with bridged race
categories. Vital Health Stat 2. 2003;135:1–55.
19. National Center for Health Statistics. Vital statistics data available online:
birthdatafiles. Availableat: http://www.cdc.gov/nchs/nvss/birth_methods.
htm. Accessed January 11, 2010.
20. Anderson RN, Rosenberg HM. Age standardization of death rates:
implementation of the year 2000 standard. Natl Vital Stat Rep. 1998;
47L1–16, 20.
21. Gillum RF. Epidemiology of congenital heart disease in the United States.
Am Heart J. 1994;127:919–927.
22. Eberstein IW, Nam CB, Heyman KM. Causes of death and mortality
crossovers by race. Biodemography Soc Biol. 2008;54:214–228.
23. Nam CB. Another look at mortality crossovers. Social Biol. 1995;42:
133–142.
24. Israel RA, Rosenberg HM, Curtin LR. Analytical potential for multiple
cause-of-death data. Am J Epidemiol. 1986;124:161–179.
25. Lakkireddy DR, Gowda MS, Murray CW, Basarakodu KR, Vacek JL.
Death certificate completion: how well are physicians trained and are
cardiovascular causes overstated? Am J Med. 2004;117:492–498.
26. Ravakhah K. Death certificates are not reliable: revivification of the
autopsy. South Med J. 2006;99:728–733.
27. Lu TH. Using ACME (Automatic Classification of Medical Entry)
software to monitor and improve the quality of cause of death statistics.
J Epidemiol Community Health. 2003;57:470–471.
28. Norwood WI, Pigott JD. Recent advances in cardiac surgery. Pediatr Clin
N Am. 1985;32:1117–1124.
29. Botto LD, Correa A, Erickson JD. Racial and temporal variations in the
prevalence of heart defects. Pediatrics. 2001;107:E32.
30. Correa A, Cragan JD, Kucik JE, Alverson CJ, Gilboa SM, Balakrishnan
R, Strickland MJ, Duke CW, O’Leary LA, Riehle-Colarusso T, Siffel C,
Gambrell D, Thompson D, Atkinson M, Chitra J. Reporting birth defects
surveillance data 1968–2003. Birth Defects Res A Clin Mol Teratol.
2007;79:65–186.
31. Hoffman JI, Kaplan S. The incidence of congenital heart disease. J Am
Coll Cardiol. 2002;39:1890–1900.
32. Anderson RN, Minino AM, Hoyert DL, Rosenberg HM. Comparability of
cause of death between ICD-9 and ICD-10: preliminary estimates. Natl
Vital Stat Rep. 2001;49:1–32.
33. Botto LD, Lin AE, Riehle-Colarusso T, Malik S, Correa A. Seeking
causes: classifying and evaluating congenital heart defects in etiologic
studies. Birth Defects Res A Clin Mol Teratol. 2007;79:714–727.
34. Riehle-Colarusso T, Strickland MJ, Reller MD, Mahle WT, Botto LD,
Siffel C, Atkinson M, Correa A. Improving the quality of surveillance
data on congenital heart defects in the metropolitan Atlanta congenital
defects program. Birth Defects Res A Clin Mol Teratol. 2007;79:
743–753.
35. Strickland MJ, Riehle-Colarusso TJ, Jacobs JP, Reller MD, Mahle WT,
Botto LD, Tolbert PE, Jacobs ML, Lacour-Gayet FG, Tchervenkov CI,
Mavroudis C, Correa A. The importance of nomenclature for congenital
cardiac disease: implications for research and evaluation. Cardiol Young.
2008;18(suppl 2):92–100.
36. Yates RS. The influence of prenatal diagnosis on postnatal outcome in
patients with structural congenital heart disease. Prenat Diagn. 2004;24:
1143–1149.
37. Bonnet D, Coltri A, Butera G, Fermont L, Le Bidois J, Kachaner J, Sidi
D. Detection of transposition of the great arteries in fetuses reduces
neonatal morbidity and mortality. Circulation. 1999;99:916–918.
38. Tworetzky W, McElhinney DB, Reddy VM, Brook MM, Hanley FL,
Silverman NH. Improved surgical outcome after fetal diagnosis of hyp-
oplastic left heart syndrome. Circulation. 2001;103:1269–1273.
39. Blyth M, Howe D, Gnanapragasam J, Wellesley D. The hidden mortality
of transposition of the great arteries and survival advantage provided by
prenatal diagnosis. BJOG. 2008;115:1096–1100.
2262 Circulation November 30, 2010
by guest on April 16, 2014 http://circ.ahajournals.org/ Downloaded from
40. Montana E, Khoury MJ, Cragan JD, Sharma S, Dhar P, Fyfe D. Trends
and outcomes after prenatal diagnosis of congenital cardiac malfor-
mations by fetal echocardiography in a well defined birth population,
Atlanta, Georgia, 1990 –1994. J Am Coll Cardiol. 1996;28:
1805–1809.
41. Cragan JD, Gilboa SM. Including prenatal diagnoses in birth defects
monitoring: experience of the Metropolitan Atlanta Congenital Defects
Program. Birth Defects Res A Clin Mol Teratol. 2009;85:20–29.
42. Khoo NS, Van Essen P, Richardson M, Robertson T. Effectiveness of
prenatal diagnosis of congenital heart defects in South Australia: a pop-
ulation analysis 1999–2003. Aust N Z J Obstet Gynaecol. 2008;48:
559–563.
43. Crider KS, Olney RS, Cragan JD. Trisomies 13 and 18: population
prevalences, characteristics, and prenatal diagnosis, metropolitan Atlanta,
1994–2003. Am J Med Genet A. 2008;146:820–826.
44. Siffel C, Correa A, Cragan J, Alverson CJ. Prenatal diagnosis, pregnancy
terminations and prevalence of Down syndrome in Atlanta. Birth Defects
Res A Clin Mol Teratol. 2004;70:565–571.
45. Fernandes SM, Landzberg MJ. Transitioning the young adult with con-
genital heart disease for life-long medical care. Pediatr Clin N Am.
2004;51:1739–1748.
46. Hudsmith LE, Thorne SA. Transition of care from paediatric to adult
services in cardiology. Arch Dis Child. 2007;92:927–930.
47. Billett J, Majeed A, Gatzoulis M, Cowie M. Trends in hospital
admissions, in-hospital case fatality and population mortality from con-
genital heart disease in England, 1994 to 2004. Heart. 2008;94(3):
342–348.
48. Ohuchi H, Miyamoto Y, Yamamoto M, Ishihara H, Takata H, Miyazaki
A, Yamada O, Yagihara T. High prevalence of abnormal glucose metab-
olism in young adult patients with complex congenital heart disease. Am
Heart J. 2009;158:30–39.
CLINICAL PERSPECTIVE
Among infants and young children, congenital heart disease (CHD) is responsible for the largest proportion, 30% to 50%,
of mortality resulting from birth defects. Mortality caused by CHD during infancy and childhood is reportedly decreasing,
and the prevalence of CHD among adults is increasing. Until recently, limited population-based data have been available
on CHD mortality through adulthood. Using US multiple cause-of-death data from the National Center for Health Statistics
from 1999 to 2006, the present study examined recent temporal trends in mortality resulting from CHD, explored
differences in CHD mortality by race-ethnicity (non-Hispanic whites, non-Hispanic blacks or African Americans,
Hispanics, and other non-Hispanic race-ethnicities), and determined whether CHD mortality has declined to the same
extent among all race-ethnicities. Although CHD mortality continued to decline among both children and adults,
differences between race-ethnicities persist. A large proportion of CHD-related mortality continued to occur during
infancy, although significant CHD mortality occurred during adulthood. As CHD mortality continues to decline and people
with CHD live longer, managing care into adulthood is increasingly important, particularly during the transition from
pediatric to adult specialty care.
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SUPPLEMENTAL MATERIAL
CIRCULATIONAHA/2010/947002/R2

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Appendix 1. Congenital heart diseases and ICD-10 codes included in mortality analyses,
United States, 1999–2006
Congenital heart disease ICD-10 Code

Any congenital heart disease Q20-Q26

Anomalous pulmonary venous
connection Q26.2, Q26.3, Q26.4

Aortic valve anomalies
Q23.0

Atrial septal defect
Q21.1


Atrioventricular septal defect
Q21.2


Coarctation of the aorta
Q25.1


Common truncus
Q20.0


Common ventricle
Q20.4


Ebstein's anomaly
Q22.5


Hypoplastic left heart syndrome
Q23.4


Patent ductus arteriosus
Q25.0


Pulmonary artery atresia/stenosis
Q25.5, Q25.6


Pulmonary valve anomalies
Q22.0, Q22.1, Q22.3


Tetralogy of Fallot
Q21.3


Transposition of the great arteries
Q20.1, Q20.3, Q20.5


Tricuspid valve anomalies
Q22.4


Ventricular septal defect
Q21.0


CIRCULATIONAHA/2010/947002/R2

3


Other specified congenital heart disease
Q20.2, Q20.6, Q20.8, Q20.9,

Q21.4, Q21.8, Q21.9,

Q22.2, Q22.6, Q22.8, Q22.9,

Q23.1. Q23.2, Q23.3, Q23.8, Q23.9,
Q24.0, Q24.1, Q24.2, Q24.3, Q24.4, Q24.5, Q24.6,
Q24.8,

Q25.2, Q25.3, Q25.4, Q25.7, Q25.8, Q25.9,

Q26.0, Q26.1, Q26.5, Q26.6,

Q26.8, Q26.9


Unspecified congenital heart disease
Q24.9

ICD-10, International Classification of Diseases, Tenth Revision

CIRCULATIONAHA/2010/947002/R2

4
Appendix 2. Overall and annual age-specific congenital heart disease mortality rate ratios and 95% confidence intervals for non-
Hispanic Blacks and Hispanics compared with non-Hispanic Whites, United States, 1999-2006

Non-Hispanic Black versus Non-Hispanic White

All years 1999 2000 2001 2002 2003 2004 2005 2006

All ages* 1.25 (1.20-1.31) 1.23 (1.17-1.28) 1.24 (1.19-1.30) 1.29 (1.23-1.34) 1.24 (1.18-1.30) 1.25 (1.19-1.31) 1.25 (1.20-1.31) 1.18 (1.12-1.24) 1.39 (1.33-1.46)

0-<1 year 1.40 (1.34-1.46) 1.29 (1.13-1.45) 1.36 (1.19-1.52) 1.44 (1.26-1.62) 1.51 (1.32-1.70) 1.38 (1.20-1.56) 1.45 (1.26-1.63) 1.28 (1.11-1.45) 1.53 (1.34-1.74)

1-4 years 1.68 (1.49-1.89) 2.12 (1.44-2.80) 2.19 (1.47-2.91) 1.66 (1.10-2.22) 1.06 (0.66-1.46) 1.92 (1.33-2.50) 1.64 (1.06-2.21) 1.22 (0.77-1.67) 1.81 (1.25-2.60)

5-17 years 1.65 (1.47-1.85) 1.62 (1.13-2.12) 1.66 (1.16-2.16) 1.75 (1.20-2.31) 1.42 (0.95-1.88) 1.52 (1.01-2.03) 1.75 (1.19-2.31) 2.09 (1.42-2.77) 1.46 (1.00-2.14)

18-34 years 1.29 (1.18-1.42) 1.08 (0.79-1.38) 1.13 (0.83-1.43) 1.32 (0.98-1.65) 1.23 (0.91-1.56) 1.45 (1.06-1.85) 1.33 (0.97-1.68) 1.38 (1.02-1.74) 1.55 (1.18-2.04)

35-49 years 1.03 (0.92-1.15) 1.02 (0.73-1.31) 1.23 (0.88-1.58) 1.06 (0.75-1.38) 0.98 (0.68-1.27) 1.05 (0.74-1.35) 0.97 (0.64-1.30) 1.09 (0.76-1.42) 0.85 (0.60-1.20)

50-64 years 0.97 (0.84-1.12) 1.10 (0.68-1.52) 0.89 (0.52-1.27) 1.22 (0.77-1.68) 0.92 (0.53-1.32) 0.85 (0.48-1.23) 0.56 (0.28-0.85) 0.75 (0.40-1.10) 1.53 (1.08-2.16)

>65 years 0.69 (0.59-0.80) 0.93 (0.62-1.23) 0.65 (0.39-0.91) 0.60 (0.34-0.86) 0.72 (0.43-1.00) 0.58 (0.32-0.84) 0.63 (0.34-0.92) 0.41 (0.17-0.65) 0.99 (0.67-1.47)

Hispanic versus Non-Hispanic White

All years 1999 2000 2001 2002 2003 2004 2005 2006

All ages* 0.87 (0.83-0.92) 0.88 (0.84-0.92) 0.90 (0.86-0.94) 0.85 (0.81-0.90) 0.94 (0.90-0.99) 0.85 (0.81-0.90) 0.85 (0.81-0.89) 0.91 (0.86-0.95) 0.86 (0.81-0.90)

0-<1 year 1.04 (1.00-1.08) 1.02 (0.89-1.14) 1.06 (0.93-1.18) 1.13 (1.00-1.27) 1.16 (1.02-1.29) 1.04 (0.92-1.17) 0.89 (0.78-1.00) 1.04 (0.91-1.16) 1.05 (0.93-1.18)

1-4 years 1.22 (1.08-1.38) 1.41 (0.92-1.89) 1.67 (1.12-2.23) 1.04 (0.66-1.42) 1.09 (0.72-1.46) 1.27 (0.87-1.68) 1.41 (0.94-1.87) 0.81 (0.50-1.11) 1.27 (0.88-1.81)

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5-17 years 0.91 (0.79-1.04) 0.99 (0.62-1.35) 0.75 (0.45-1.06) 0.77(0.45-1.10) 0.99 (0.63-1.35) 0.91 (0.56-1.27) 1.00 (0.63-1.37) 1.22 (0.78-1.67) 0.69 (0.43-1.11)

18-34 years 0.65 (0.58-0.72) 0.69 (0.48-0.90) 0.52 (0.35-0.70) 0.53 (0.35-0.71) 0.67 (0.47-0.88) 0.64 (0.43-0.86) 0.60 (0.40-0.79) 0.78 (0.55-1.00) 0.82 (0.60-1.11)

35-49 years 0.58 (0.51-0.67) 0.57 (0.34-0.80) 0.68 (0.42-0.95) 0.63 (0.39-0.88) 0.54 (0.32-0.75) 0.35 (0.19-0.52) 0.60 (0.35-0.84) 0.70 (0.45-0.95) 0.65 (0.45-0.94)

50-64 years 0.72 (0.60-0.87) 0.56 (0.22-0.90) 0.45 (0.15-0.76) 0.56 (0.22-0.90) 0.75 (0.36-1.15) 0.89 (0.46-1.32) 0.84 (0.45-1.23) 1.07 (0.60-1.54) 0.66 (0.38-1.14)

>65 years 0.66 (0.55-0.79) 0.80 (0.44-1.16) 0.92 (0.53-1.32) 0.51 (0.22-0.80) 0.86 (0.48-1.25) 0.54 (0.24-0.84) 0.83 (0.43-1.22) 0.51 (0.20-0.81) 0.33 (0.16-0.71)

* Mortality rateratios for "all ages" arebased on age-standardized mortality rates. All other ratios arebased on age-specific mortality rates.
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Appendix 3. Annual infant mortality rate* due to congenital heart disease, United States, 1999–2006
Year of death Percent Change

1999 2000 2001 2002 2003 2004 2005 2006 Overall‡
Per
Year§
P-
value#

Any congenital heart disease† 45.56 45.07 42.76 42.36 40.54 39.50 38.61 37.69 -17.3 -2.8 <0.01

Race-ethnicity

Non-Hispanic White 44.31 42.53 39.54 38.38 38.32 38.92 37.54 35.21 -20.5 -2.6 <0.01

Non-Hispanic Black 57.04 57.75 56.96 58.10 52.94 56.31 47.95 53.77 -5.7 -1.8 0.07

Hispanic 45.08 44.98 44.84 44.37 39.99 34.65 38.91 36.82 -18.3 -3.6 0.01

Other non-Hispanic 29.20 38.25 31.32 35.33 34.63 24.82 25.81 25.52 -12.6 -3.9 0.10

Sex

Male 48.99 46.81 46.25 45.43 44.86 42.07 41.38 41.10 -16.1 -2.5 <0.01

Female 41.96 43.25 39.11 39.15 36.01 36.80 35.70 34.11 -18.7 -3.1 <0.01

Anomalous pulmonary venous
connection 1.12 0.52 1.05 0.83 0.86 0.69 0.73 0.82 -26.6 0.1 0.98

Aortic valveanomalies 1.10 0.75 0.96 0.43 0.76 0.64 0.68 0.80 -27.0 -2.5 0.66

Atrial septal defect 0.51 0.37 0.55 0.53 0.52 0.56 0.56 0.52 1.5 3.1 0.19

Atrioventricular septal defect 0.84 1.05 0.67 1.08 0.79 0.69 1.00 0.75 -10.5 -1.4 0.66

Coarctation of theaorta 0.89 0.97 0.50 0.90 0.61 1.00 0.68 0.92 2.8 0.8 0.87
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Common truncus 0.82 1.34 1.05 1.18 0.76 0.83 0.92 0.85 3.8 -2.4 0.44

Common ventricle 0.43 0.50 0.50 0.43 0.39 0.27 0.19 0.31 -29.4 -11.3 0.02

Ebstein's anomaly 0.66 0.72 0.57 0.70 0.84 0.73 0.66 0.68 2.9 0.8 0.65

Hypoplastic left heart syndrome 10.06 10.13 9.40 8.43 8.01 7.66 8.16 6.88 -31.6 -5.1 <0.01

Patent ductus arteriosus 0.87 0.85 0.67 1.33 1.52 1.86 1.80 1.51 73.7 14.0 0.02

Pulmonary artery atresia/stenosis 1.33 1.32 1.22 1.23 1.35 1.17 1.09 1.04 -21.9 -3.2 0.01

Pulmonary valveanomalies 0.13 0.12 0.25 0.15 0.20 0.17 0.05 0.21 66.1 -10.4 0.28

Tetralogy of Fallot 1.94 1.67 1.72 2.00 1.72 1.74 1.63 1.63 -16.2 -1.7 0.15

Transposition of thegreat arteries 2.55 2.02 1.70 1.80 1.94 1.57 2.14 1.67 -34.5 -3.1 0.21

Tricuspid valveanomalies 0.26 0.20 0.25 0.38 0.32 0.32 0.12 0.35 38.4 -4.1 0.56

Ventricular septal defect 1.51 1.10 1.17 1.35 0.93 0.98 0.68 0.75 -49.9 -9.4 <0.01


Other specified congenital heart
disease 3.80 3.76 3.60 2.98 3.66 3.23 3.23 2.99 -21.3 -3.0 0.04

Unspecified congenital heart disease 16.73 17.68 16.92 16.66 15.34 15.39 14.29 14.99 -10.4 -2.6 <0.01

* Infant mortality ratecalculated per 100,000 livebirths
† SeeAppendix 1 for ICD-10 codes for congenital heart diseasecategories
‡ Overall percent changecomputed as: ((mortality ratein 2006 - mortality ratein 1999)/(mortality ratein 1999))*100
§ Averageannual percent changecomputed fromweighted least squares (WLS) model regressing natural log of themortality rateon year of
death
#P-valuefor WLS model regressing natural log of themortality rateon theyear of death