circulation 2010 gilboa 2254 63
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Suzanne M. Gilboa, Jason L. Salemi, Wendy N. Nembhard, David E. Fixler and Adolfo CorreaUnited States, 1999 to 2006
Mortality Resulting From Congenital Heart Disease Among Children and Adults in the
Print ISSN: 0009-7322. Online ISSN: 1524-4539
Copyright 2010 American Heart Association, Inc. All rights reserved.is published by the American Heart Association, 7272 Greenville Avenue, Dallas, TX 75231Circulation
doi: 10.1161/CIRCULATIONAHA.110.9470022010;122:2254-2263; originally published online November 22, 2010;Circulation.
http://circ.ahajournals.org/content/122/22/2254
World Wide Web at:The online version of this article, along with updated information and services, is located on the
http://circ.ahajournals.org/content/suppl/2010/11/30/CIRCULATIONAHA.110.947002.DC1.htmlData Supplement (unedited) at:
http://circ.ahajournals.org/content/122/22/2254http://circ.ahajournals.org/content/suppl/2010/11/30/CIRCULATIONAHA.110.947002.DC1.htmlhttp://circ.ahajournals.org/content/suppl/2010/11/30/CIRCULATIONAHA.110.947002.DC1.htmlhttp://circ.ahajournals.org/content/suppl/2010/11/30/CIRCULATIONAHA.110.947002.DC1.htmlhttp://circ.ahajournals.org/content/suppl/2010/11/30/CIRCULATIONAHA.110.947002.DC1.htmlhttp://circ.ahajournals.org/content/122/22/2254 -
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Congenital Heart Disease
Mortality Resulting From Congenital Heart Disease AmongChildren 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
BackgroundPrevious 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 ResultsWe 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 1 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 (18 years of age).ConclusionsCHD 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
Among infants and young children, congenital heartdisease (CHD) is responsible for the largest proportion,30% to 50%, of mortality caused by birth defects.14 Mortal-
ity resulting from CHD during infancy and childhood report-
Editorial see p 2231Clinical Perspective on p 2263
The aims of the present study were to examine recent
l d i li l i f CHD f 1999
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l d i li l i f CHD f 1999
tional Classification of Disease(ICD) codes for the underlying causeof death (UCOD), and up to 20 conditions listed as contributingcauses of death.
For these analyses, we used the MCOD data files from 1999 to
2006, which include ICD 10th revision (ICD-10)16,17codes (Q20 toQ26) to identify the underlying and contributing causes of CHDmortality (Appendix I in the online-only Data Supplement). Deaths
resulting from CHD were defined as those with a CHD listed as theUCOD; CHD-related deaths had a CHD listed as an underlying orcontributing cause of death. Although the majority of analysesreported 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 1 year of age were the USbridged-race postcensal population estimates for each year of inter-
est.18
For infant mortality rates, live birth data from the NationalCenter for Health Statistics were used in the denominators. 19
Annual age-specific death rates for any CHD and specific CHDdiagnoses were calculated per 100 000 population among the fol-lowing age groups: 1, 1 to 4, 5 to 17, 18 to 34, 35 to 49, 50 to 64,and 65 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, orother Pacific Islander]). To account for the different age composi-tions among these subpopulations, we adjusted the overall, race-ethnicityspecific, and sex-specific mortality rates using direct
standardization by applying the age-specific mortality rates to the USstandard population for the year 2000.20 We calculated the overallpercentage of change in the mortality rates over the time period ofinterest by subtracting the rate in 1999 from the rate in 2006 anddividing by the rate in 1999. To calculate the average annual
percentage of change and to test the hypothesis that this change wasequal to zero, a weighted least squares regression model was fit tothe natural logarithm of the rate, with the calendar year of death usedas 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 withNH whites. We also calculated mortality rate ratios comparing agegroups and sexes. Finally, we calculated the median age at death byCHD cause of death, by demographic characteristics, and over time.
R lt
Age-Specific MortalityWe 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 amongchildren 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
ratio1.34; 95% confidence interval1.27 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 65 years of age.
Infant MortalityInfant 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% (P0.01)
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 theonline-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 ChildrenAmong children 1 to 4 years of age, mortality caused by CHD
decreased 21.0% overall and 2.8% annually (P0.05; Table
3). However, no race-ethnicity studied experienced a statis-
tically significant change in mortality. Except for 2002 and2005, 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
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Table 1. Overall CHD-Related Mortality and Overall and Age-Specific (Ages 17 and Younger) Mortality Resulting From CHD, United
States, 19992006
CHD*
Mortality Related
to CHD, All Ages
Mortality Resulting From CHD
Age at Death, y
All Ages 0 1 1 4 517
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
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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 65
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
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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, %
P1999 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 24.1 3.6 0.01
Race-ethnicity
NH white 1.36 1.28 1.22 1.21 1.17 1.13 1.11 1.01 25.7 4.0 0.01
NH black 1.67 1.60 1.57 1.50 1.46 1.41 1.31 1.41 15.6 3.2 0.01
Hispanic 1.20 1.16 1.04 1.14 0.99 0.96 1.00 0.86 28.3 3.1 0.01
Other NH 0.88 0.97 0.85 0.96 0.92 0.71 0.77 0.75 14.8 3.4 0.03
Sex
Male 1.46 1.41 1.35 1.33 1.26 1.22 1.19 1.16 20.5 3.2 0.01
Female 1.27 1.21 1.14 1.15 1.10 1.03 1.02 0.92 27.6 4.2 0.01
Age at death, 1 y
Overall 45.56 45.07 42.76 42.36 40.54 39.50 38.61 37.69 17.3 2.8 0.01
Race-ethnicity
NH white 44.31 42.53 39.54 38.38 38.32 38.92 37.54 35.21 20.5 2.6 0.01
NH 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 NH 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
Age at death, 14 y
Overall 1.45 1.42 1.40 1.38 1.60 1.35 1.25 1.15 21.0 2.8 0.05
Race-ethnicity
NH white 1.14 1.07 1.23 1.34 1.33 1.11 1.24 0.95 16.6 1.3 0.51
NH black 2.43 2.34 2.05 1.42 2.55 1.82 1.52 1.72 29.0 4.9 0.16
Hispanic 1.61 1.79 1.29 1.46 1.70 1.56 1.00 1.21 25.1 5.2 0.08
Oth NH 1 67 1 39 1 89 1 48 1 44 1 74 1 67 1 31 22 0 1 3 0 53
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Table 3. Continued
Year of Death Change, %
P1999 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 4.3 1.4 0.21
Female 0.56 0.46 0.46 0.42 0.38 0.41 0.43 0.32 43.5 5.7 0.01
Age at death, 3549 y
Overall 0.64 0.56 0.57 0.58 0.56 0.46 0.53 0.48 24.6 3.5 0.02
Race-ethnicity
NH white 0.69 0.59 0.60 0.63 0.63 0.50 0.57 0.53 22 2.9 0.05
NH black 0.70 0.72 0.64 0.61 0.66 0.48 0.62 0.45 35 5.7 0.01
Hispanic 0.39 0.40 0.38 0.34 0.22 0.30 0.40 0.35 11.2 3.2 0.42
Other NH 0.32 0.18 0.29 0.34 0.25 0.24 0.24 0.25 20.2 0.2 0.96
Sex
Male 0.68 0.63 0.61 0.59 0.60 0.53 0.56 0.54 20.3 3.0 0.01
Female 0.60 0.50 0.53 0.56 0.52 0.38 0.50 0.42 29.4 4.1 0.07
Age at death, 5064 y
Overall 0.65 0.59 0.57 0.55 0.52 0.52 0.47 0.47 27.7 4.4 0.01
Race-ethnicity
NH white 0.67 0.64 0.59 0.57 0.54 0.57 0.49 0.47 29.9 4.6 0.01
NH black 0.74 0.57 0.73 0.52 0.46 0.32 0.37 0.72 2.8 8.0 0.13
Hispanic 0.38 0.29 0.33 0.43 0.48 0.48 0.52 0.31 17.1 3.2 0.38
Other NH 0.47 0.33 0.15 0.44 0.28 0.22 0.25 0.20 58.6 5.2 0.42
Sex
Male 0.72 0.61 0.58 0.56 0.51 0.57 0.53 0.54 25.8 3.4 0.03
Female 0.59 0.57 0.55 0.54 0.53 0.47 0.41 0.41 29.8 5.5 0.01
Age at death, 65 y
Overall 1.45 1.30 1.18 1.20 1.09 0.94 0.86 0.83 42.7 7.7 0.01
Race-ethnicity
NH hit 1 50 1 35 1 27 1 24 1 17 1 00 0 95 0 87 41 8 7 3 0 01
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mortality crossover22,23 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
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
Figure 1. Annual age-standardized mor-tality resulting from CHD, by race-ethnicity, United States, 19992006.
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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
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
Figure 3. Median age (in days) at death resulting from CHD, by UCOD, United States, 19992006.
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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 impairment47 and abnormal glucose
metabolism,47,48 as well as counseling on issues such ascontraception and pregnancy, potential genetic transmis-
sion of CHD, dental care, diet, optimal weight, exercise,
and physical activity.46
DisclosuresNone.
References1. Petrini J, Damus K, Johnston RB Jr. An overview of infant mortality and
birth defects in the United States. Teratology. 1997;56:810.
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):S3S6.
3. Yang Q, Khoury MJ, Mannino D. Trends and patterns of mortality
associated with birth defects and genetic diseases in the United States,
19791992: an analysis of multiple-cause mortality data. Genet Epi-
demiol. 1997;14:493505.
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, 19892002. Birth Defects Res A Clin Mol Teratol. 2006;76:706713.
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, 19791997. Circulation. 2001;103:
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;120:302309.
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;135:155.
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;
47L116, 20.
21. Gillum RF. Epidemiology of congenital heart disease in the United States.
Am Heart J. 1994;127:919927.
22. Eberstein IW, Nam CB, Heyman KM. Causes of death and mortality
crossovers by race. Biodemography Soc Biol. 2008;54:214228.
23. Nam CB. Another look at mortality crossovers. Social Biol. 1995;42:
133142.
24. Israel RA, Rosenberg HM, Curtin LR. Analytical potential for multiple
cause-of-death data. Am J Epidemiol. 1986;124:161179.25. 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;99:728733.
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;57:470471.
28. Norwood WI, Pigott JD. Recent advances in cardiac surgery.Pediatr Clin
N Am. 1985;32:11171124.
29. Botto LD, Correa A, Erickson JD. Racial and temporal variations in theprevalence of heart defects. Pediatrics. 2001;107:E32.
30. Correa A, Cragan JD, Kucik JE, Alverson CJ, Gilboa SM, Balakrishnan
R, Strickland MJ, Duke CW, OLeary LA, Riehle-Colarusso T, Siffel C,
Gambrell D, Thompson D, Atkinson M, Chitra J. Reporting birth defects
ill d t 1968 2003 Bi h D f R A Cli M l T l
2262 Circulation November 30, 2010
http://www.cdc.gov/nchs/products/elec_prods/subject/mortmcd.htmhttp://www.cdc.gov/nchs/products/elec_prods/subject/mortmcd.htmhttp://www.cdc.gov/nchs/nvss/birth_methods.htmhttp://www.cdc.gov/nchs/nvss/birth_methods.htmhttp://www.cdc.gov/nchs/nvss/birth_methods.htmhttp://www.cdc.gov/nchs/nvss/birth_methods.htmhttp://www.cdc.gov/nchs/products/elec_prods/subject/mortmcd.htmhttp://www.cdc.gov/nchs/products/elec_prods/subject/mortmcd.htm -
5/25/2018 Circulation 2010 Gilboa 2254 63
11/18
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, 19901994. J A m C o l l C a rd i ol. 1996;28:
18051809.
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;85:2029.
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 19992003. Aust N Z J Obstet Gynaecol. 2008;48:
559563.
43. Crider KS, Olney RS, Cragan JD. Trisomies 13 and 18: population
prevalences, characteristics, and prenatal diagnosis, metropolitan Atlanta,
19942003.Am J Med Genet A. 2008;146:820826.
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;70:565571.
45. Fernandes SM, Landzberg MJ. Transitioning the young adult with con-
genital heart disease for life-long medical care. Pediatr Clin N Am.2004;51:17391748.
46. Hudsmith LE, Thorne SA. Transition of care from paediatric to adult
services in cardiology. Arch Dis Child. 2007;92:927930.
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):
342348.
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;158:3039.
CLINICAL PERSPECTIVEAmong 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
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Appendix 1. Congenital heart diseases andICD-10codes included in mortality analyses,
United States, 19992006
Congenital heart disease ICD-10Code
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
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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
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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)
0-65 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)
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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 rate ratios for "all ages" are based on age-standardized mortality rates. All other ratios are based on age-specific mortality rates.
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Appendix 3. Annual infant mortality rate* due to congenital heart disease, United States, 19992006
Year of death Percent Change
1999 2000 2001 2002 2003 2004 2005 2006 OverallPer
YearP-
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
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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