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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 Copyright © 2010 American Heart Association, Inc. All rights reserved. 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. 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.html Data Supplement (unedited) at:  http://circ.ahajournals.org//subscriptions/ is online at: Circulation Information about subscr ibing to Subscriptions:  http://www.lww.com/reprints  Information about reprints can be found online at: Reprints:  document. Permissions and Rights Question and Answer this process is available in the click Request Permissions in the middle column of the Web page under Services. Further information about Office. Once the online version of the published article for which permission is being requested is located, can be obtained via RightsLink, a service of the Copyright Clearance Center, not the Editorial Circulation in  Requests for permissions to reproduce figures, ta bles, or portions of articles originall y published Permissions:  by guest on April 16, 2014 http://circ.ahajournals.org/ Downloaded from by guest on April 16, 2014 http://circ.ahajournals.org/ Downloaded from by guest on April 16, 2014 http://circ.ahajournals.org/ Downloaded from by guest on April 16, 2014 http://circ.ahajournals.org/ Downloaded from by guest on April 16, 2014 http://circ.ahajournals.org/ Downloaded from by guest on April 16, 2014 http://circ.ahajournals.org/ Downloaded from by guest on April 16, 2014 http://circ.ahajournals.org/ Downloaded from by guest on April 16, 2014 http://circ.ahajournals.org/ Downloaded from by guest on April 16, 2014 http://circ.ahajournals.org/ Downloaded from 

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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.

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    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
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    40. Montana E, Khoury MJ, Cragan JD, Sharma S, Dhar P, Fyfe D. Trends

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    18051809.

    41. Cragan JD, Gilboa SM. Including prenatal diagnoses in birth defects

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    42. Khoo NS, Van Essen P, Richardson M, Robertson T. Effectiveness of

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    ulation analysis 19992003. Aust N Z J Obstet Gynaecol. 2008;48:

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    43. Crider KS, Olney RS, Cragan JD. Trisomies 13 and 18: population

    prevalences, characteristics, and prenatal diagnosis, metropolitan Atlanta,

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    44. Siffel C, Correa A, Cragan J, Alverson CJ. Prenatal diagnosis, pregnancy

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    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

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    48. Ohuchi H, Miyamoto Y, Yamamoto M, Ishihara H, Takata H, Miyazaki

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    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.

    Gilboa et al Mortality From Congenital Heart Disease 2263

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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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    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)

    0-65 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-

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    CIRCULATIONAHA/2010/947002/R2 5

    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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    CIRCULATIONAHA/2010/947002/R2 6

    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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    CIRCULATIONAHA/2010/947002/R2 7

    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