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. Author manuscript; available in PMC: 2016 Jun 16.
Published in final edited form as: Circulation. 2010 Nov 22;122(22):2254–2263. doi: 10.1161/CIRCULATIONAHA.110.947002

Mortality Resulting From Congenital Heart Disease Among Children and Adults in the United States, 1999 to 2006

Suzanne M Gilboa 1, Jason L Salemi 1, Wendy N Nembhard 1, David E Fixler 1, Adolfo Correa 1
PMCID: PMC4911018  NIHMSID: NIHMS793299  PMID: 21098447

Abstract

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 ≥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).

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.

Keywords: epidemiology, heart defects, congenital, mortality, race, vital statistics

Among 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 Mortality resulting from CHD during infancy and childhood reportedly 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 adulthood.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 CHD15 and for ongoing monitoring of CHD mortality among adults.

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 mortality 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, International 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 denominators for mortality rates for those ≥1 year of age were the US bridged-race postcensal population estimates for each year of interest.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 following 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, or other Pacific Islander]). To account for the different age compositions 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 (n=41 494); 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).

Table 1.

Overall CHD-Related Mortality and Overall and Age-Specific (Ages 17 and Younger) Mortality Resulting From CHD, United States, 1999–2006

Mortality Resulting From CHD
Age at Death, y
Mortality Related
to CHD, All Ages
 All Ages
 0–<1
 1–4
 5–17
CHD* 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
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*

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 >1 year of age calculated per 100 000 population of that age.

Mortality resulting from CHD declined 24.1% overall and 3.6% annually during the period of 1999 to 2006 (P<0.01; 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).

Table 3.

Annual Mortality Rates* Resulting From CHD by Age Group, Race-Ethnicity, and Sex, United States, 1999 –2006

Year of Death
 Change, %
1999 2000 2001 2002 2003 2004 2005 2006 Overall† Per Year‡ P §
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, 1–4 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
  Other NH 1.67 1.39 1.89 1.48 1.44 1.74 1.67 1.31 −22.0 −1.3 0.53
 Sex
  Male 1.64 1.52 1.49 1.65 1.62 1.53 1.29 1.23 −25.5 −3.4 0.03
  Female 1.25 1.32 1.31 1.11 1.59 1.17 1.21 1.07 −14.8 −1.9 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 −36.1 −4.8 0.02
 Race-ethnicity
  NH white 0.43 0.43 0.38 0.41 0.37 0.37 0.33 0.3 −30.3 −4.8 <0.01
  NH black 0.69 0.71 0.66 0.58 0.57 0.65 0.69 0.44 −37.3 −4.8 0.06
  Hispanic 0.42 0.32 0.29 0.40 0.34 0.37 0.40 0.21 −51.3 −5.2 0.19
  Other NH 0.42 0.49 0.33 0.29 0.40 0.32 0.46 0.25 −41.5 −5.1 0.17
 Sex
  Male 0.58 0.53 0.51 0.49 0.46 0.47 0.45 0.33 −43.3 −6.3 <0.01
  Female 0.35 0.39 0.30 0.36 0.33 0.36 0.36 0.27 −23.2 −2.3 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 −21.4 −3.0 0.01
 Race-ethnicity
  NH white 0.66 0.66 0.62 0.62 0.50 0.56 0.56 0.47 −28.4 −4.3 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 −15.2 −0.2 0.92
  Other NH 0.47 0.27 0.34 0.24 0.30 0.25 0.30 0.43 −8.1 −1.0 0.82
 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, 35–49 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, 50–64 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 white 1.50 1.35 1.27 1.24 1.17 1.00 0.95 0.87 −41.8 −7.3 <0.01
  NH black 1.39 0.88 0.76 0.89 0.67 0.63 0.39 0.87 −37.7 −10.7 0.06
  Hispanic 1.21 1.25 0.65 1.07 0.63 0.83 0.48 0.29 −75.8 −17.0 <0.01
  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 −33.6 −7.4 <0.01
  Female 1.59 1.26 1.25 1.34 1.25 1.03 0.93 0.83 −47.7 −7.8 <0.01
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*

Mortality rate calculated per 100 000 population in the age group.

†

Overall percent change computed as follows: [(mortality rate in 2006–mortality rate in 1999)/(mortality rate in 1999)]×100.

‡

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.

Figure 1.

Figure 1

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Annual age-standardized mortality resulting from CHD, by race-ethnicity, United States, 1999–2006

Table 2.

Age-Specific (Ages 18 and Older) Mortality Resulting from CHD, United States, 1999–2006

Age at Death, y
18–34
 35–49
 50–64
 ≥65
CHD 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
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*

Mortality rates among individuals >1 year of age calculated per 100 000 population of that age.

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 ratio=1.34; 95% confidence interval=1.27 to 1.43; calculation not shown). Mortality resulting from CHD was unchanged among adults 18 to 64 years of age, with a marked increase among individuals ≥65 years of age.

Figure 2.

Figure 2

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Age-specific mortality resulting from CHD, by race-ethnicity, United States, 1999–2006

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% (P<0.01) 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 (P=0.05; Table 3). However, no race-ethnicity studied experienced a statistically 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 (P=0.02). The magnitudes of the CHD mortality decrease among the different race-ethnicities were very similar, yet only NH whites experienced a statistically significant decrease; the decrease among NH blacks was of borderline significance (P=0.06). CHD mortality decreased significantly 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 ≥18 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 (P=0.01) among adults 18 to 34 years of age. This decrease was driven by a significant decline among NH whites. CHD mortality decreased significantly 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 (P=0.02) 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 (P<0.01) 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 ≥65 years of age, mortality caused by CHD declined significantly during the study period (42.7% overall, 7.7% annually; P<0.01). 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 (P=0.06). Mortality also declined for both men and women (Table 3). Consistently over time, NH blacks ≥65 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 <65 years of age, but among adults ≥65 years of age, CHD mortality was lower for men (mortality rate ratio=0.84; 95% confidence interval=0.81 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 <1 month (eg, patent ductus arteriosus, 11 days; HLHS, 14 days; and common truncus, 30 days) to >40 years (ventricularseptal 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).

Figure 3.

Figure 3

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Median age (in days) at death resulting from CHD, by UCOD, United States, 1999–2006

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 mortality 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, ≈76% of mortality resulting from CHD occurs in adulthood. Compared with all 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 <1 month to >70 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 ≥65 years of age, we observed lower mortality caused by CHD among NH blacks compared with NH whites. This 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 misclassification of UCOD by race-ethnicity. These analyses assumed homogeneity within population subgroups and did not atempt 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 subpopulation 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 Statistics to standardize the assignment of the UCOD.27 Despite the development of rules and decision tables to identify “acceptable” 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 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 ≥35 years of age (Table 1) might represent coding errors; the first successful Norwood palliation 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 underlying 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 infants31 and improved 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 ≈3000 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 Prenatal 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 termination 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 addition, there are known differences in pregnancy termination for chromosomal anomalies by race-ethnicity,43,44 which, if relevant to CHD, could contribute to some of the differences reported here.

CHD mortality continues to decline, and people with CHD are living longer, making managing care into adulthood increasingly important.45,46 In addition to the management 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 dysfunction such as renal impairment47 and abnormal glucose metabolism,47,48 as well as counseling on issues such as contraception and pregnancy, potential genetic transmission of CHD, dental care, diet, optimal weight, exercise, and physical activity.46

Supplementary Material

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.

Footnotes

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.

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. doi: 10.1002/(SICI)1096-9926(199707/08)56:1/2<8::AID-TERA3>3.0.CO;2-U. [DOI] [PubMed] [Google Scholar]
  • 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. doi: 10.1002/tera.90002. [DOI] [PubMed] [Google Scholar]
  • 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 Epidemiol. 1997;14:493–505. doi: 10.1002/(SICI)1098-2272(1997)14:5<493::AID-GEPI4>3.0.CO;2-2. [DOI] [PubMed] [Google Scholar]
  • 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. doi: 10.1002/bdra.20308. [DOI] [PubMed] [Google Scholar]
  • 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. doi: 10.1161/01.cir.103.19.2376. [DOI] [PubMed] [Google Scholar]
  • 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. doi: 10.1161/CIRCULATIONAHA.106.627224. [DOI] [PubMed] [Google Scholar]
  • 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. doi: 10.1016/s0735-1097(01)01272-4. [DOI] [PubMed] [Google Scholar]
  • 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. [PubMed] [Google Scholar]
  • 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. doi: 10.1016/j.ahj.2009.08.014. [DOI] [PubMed] [Google Scholar]
  • 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. doi: 10.1002/bdra.10119. [DOI] [PubMed] [Google Scholar]
  • 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. doi: 10.1002/tera.1073. [DOI] [PubMed] [Google Scholar]
  • 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. doi: 10.1002/bdra.20218. [DOI] [PubMed] [Google Scholar]
  • 13.Copeland GE, Kirby RS. Using birth defects registry data to evaluate infant and childhood mortality associated with birth defects: an alternative to traditional mortality assessment using underlying cause of death statistics. Birth Defects Res A Clin Mol Teratol. 2007;79:792–797. doi: 10.1002/bdra.20391. [DOI] [PubMed] [Google Scholar]
  • 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. doi: 10.1111/j.1365-3016.2008.00987.x. [DOI] [PubMed] [Google Scholar]
  • 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. doi: 10.1161/CIRCULATIONAHA.108.839464. [DOI] [PubMed] [Google Scholar]
  • 16.World Health Organization . International Statistical Classification of Diseases and Related Health Problems. 10th World Health Organization; Geneva, Switzerland: 1992. [Google Scholar]
  • 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. [PubMed] [Google Scholar]
  • 19.National Center for Health Statistics Vital statistics data available online: birth data files. Available at: 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. [PubMed] [Google Scholar]
  • 21.Gillum RF. Epidemiology of congenital heart disease in the United States. Am Heart J. 1994;127:919–927. doi: 10.1016/0002-8703(94)90562-2. [DOI] [PubMed] [Google Scholar]
  • 22.Eberstein IW, Nam CB, Heyman KM. Causes of death and mortality crossovers by race. Biodemography Soc Biol. 2008;54:214–228. doi: 10.1080/19485565.2008.9989143. [DOI] [PubMed] [Google Scholar]
  • 23.Nam CB. Another look at mortality crossovers. Social Biol. 1995;42:133–142. doi: 10.1080/19485565.1995.9988893. [DOI] [PubMed] [Google Scholar]
  • 24.Israel RA, Rosenberg HM, Curtin LR. Analytical potential for multiple cause-of-death data. Am J Epidemiol. 1986;124:161–179. doi: 10.1093/oxfordjournals.aje.a114375. [DOI] [PubMed] [Google Scholar]
  • 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. doi: 10.1016/j.amjmed.2004.04.018. [DOI] [PubMed] [Google Scholar]
  • 26.Ravakhah K. Death certificates are not reliable: revivification of the autopsy. South Med J. 2006;99:728–733. doi: 10.1097/01.smj.0000224337.77074.57. [DOI] [PubMed] [Google Scholar]
  • 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. doi: 10.1136/jech.57.6.470. [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 28.Norwood WI, Pigott JD. Recent advances in cardiac surgery. Pediatr Clin N Am. 1985;32:1117–1124. doi: 10.1016/s0031-3955(16)34896-9. [DOI] [PubMed] [Google Scholar]
  • 29.Botto LD, Correa A, Erickson JD. Racial and temporal variations in the prevalence of heart defects. Pediatrics. 2001;107:E32. doi: 10.1542/peds.107.3.e32. [DOI] [PubMed] [Google Scholar]
  • 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. doi: 10.1002/bdra.20350. [DOI] [PubMed] [Google Scholar]
  • 31.Hoffman JI, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol. 2002;39:1890–1900. doi: 10.1016/s0735-1097(02)01886-7. [DOI] [PubMed] [Google Scholar]
  • 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. [PubMed] [Google Scholar]
  • 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. doi: 10.1002/bdra.20403. [DOI] [PubMed] [Google Scholar]
  • 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. doi: 10.1002/bdra.20412. [DOI] [PubMed] [Google Scholar]
  • 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. doi: 10.1017/S1047951108002515. [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 36.Yates RS. The influence of prenatal diagnosis on postnatal outcome in patients with structural congenital heart disease. Prenat Diagn. 2004;24:1143–1149. doi: 10.1002/pd.1072. [DOI] [PubMed] [Google Scholar]
  • 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. doi: 10.1161/01.cir.99.7.916. [DOI] [PubMed] [Google Scholar]
  • 38.Tworetzky W, McElhinney DB, Reddy VM, Brook MM, Hanley FL, Silverman NH. Improved surgical outcome after fetal diagnosis of hypoplastic left heart syndrome. Circulation. 2001;103:1269–1273. doi: 10.1161/01.cir.103.9.1269. [DOI] [PubMed] [Google Scholar]
  • 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. doi: 10.1111/j.1471-0528.2008.01793.x. [DOI] [PubMed] [Google Scholar]
  • 40.Montana E, Khoury MJ, Cragan JD, Sharma S, Dhar P, Fyfe D. Trends and outcomes after prenatal diagnosis of congenital cardiac malformations by fetal echocardiography in a well defined birth population, Atlanta, Georgia, 1990–1994. J Am Coll Cardiol. 1996;28:1805–1809. doi: 10.1016/S0735-1097(96)00381-6. [DOI] [PubMed] [Google Scholar]
  • 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. doi: 10.1002/bdra.20508. [DOI] [PubMed] [Google Scholar]
  • 42.Khoo NS, Van Essen P, Richardson M, Robertson1 T. Effectiveness of prenatal diagnosis of congenital heart defects in South Australia: a population analysis 1999–2003. Aust N Z J Obstet Gynaecol. 2008;48:559–563. doi: 10.1111/j.1479-828X.2008.00915.x. [DOI] [PubMed] [Google Scholar]
  • 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. doi: 10.1002/ajmg.a.32200. [DOI] [PubMed] [Google Scholar]
  • 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. doi: 10.1002/bdra.20064. [DOI] [PubMed] [Google Scholar]
  • 45.Fernandes SM, Landzberg MJ. Transitioning the young adult with congenital heart disease for life-long medical care. Pediatr Clin N Am. 2004;51:1739–1748. doi: 10.1016/j.pcl.2004.07.006. [DOI] [PubMed] [Google Scholar]
  • 46.Hudsmith LE, Thorne SA. Transition of care from paediatric to adult services in cardiology. Arch Dis Child. 2007;92:927–930. doi: 10.1136/adc.2006.103812. [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 47.Billett J, Majeed A, Gatzoulis M, Cowie M. Trends in hospital admissions, in-hospital case fatality and population mortality from congenital heart disease in England, 1994 to 2004. Heart. 2008;94(3):342–348. doi: 10.1136/hrt.2006.113787. [DOI] [PubMed] [Google Scholar]
  • 48.Ohuchi H, Miyamoto Y, Yamamoto M, Ishihara H, Takata H, Miyazaki A, Yamada O, Yagihara T. High prevalence of abnormal glucose metabolism in young adult patients with complex congenital heart disease. Am Heart J. 2009;158:30–39. doi: 10.1016/j.ahj.2009.04.021. [DOI] [PubMed] [Google Scholar]

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