Years of Potential Life Lost for Children and Adults With Congenital Heart Defects: United States, 2007 to 2017

Abstract

Background

Although most children with congenital heart defects (CHDs) live into adulthood, many have increased mortality risk across the lifespan. Little is known about years lost due to premature CHD‐related deaths. We estimated the years of potential life lost (YPLL) among individuals with CHDs in the United States.

Methods

We used 2007 to 2017 death records from the US National Center for Health Statistics to identify decedents with a CHD listed as the underlying or contributing cause of death. We calculated the average percent change in YPLL and the total, mean, crude, and age‐standardized YPLL overall, by sex, race and ethnicity, and age group.

Results

Of 28.35 million deaths, 42 158 were CHD‐related. The premature deaths attributed to CHD for individuals younger than 65 years was almost 2.1 million years; of those 169 756 and 124 067 years were lost prematurely for children and adolescents, respectively. Men and women with CHDs had 1.13 million and 941 115 years lost prematurely, respectively. Non‐Hispanic Black individuals and men had the highest age‐standardized YPLL (per 100 000) (95.5 [95% CI, 93.2–97.7] and 74.1 [95% CI, 73.0–75.1]). The overall mean YPLL was 70 years (per 100 000) and non‐Hispanic Black men and women had the highest mean YPLL. During 2007 to 2017, the YPLL average percent change declined by 17.8% overall, but the YPLL for non‐Hispanic Black individuals aged 1 to 4 years (−31.6%) and 35 to 49 years (−24.3%) had the greatest decline.

Conclusions

Children with CHDs experience significant premature deaths. Non‐Hispanic Black and male individuals experienced the highest burden of premature deaths associated with CHDs. Further research is needed to elucidate these disparities.

Nonstandard Abbreviations and Acronyms

APC

average percent change

CDC WONDER

Centers for Disease Control and Prevention—Wide‐ranging ONline Data for Epidemiologic Research

CHD

congenital heart defect

NCHS

National Center for Health Statistics

NH

non‐Hispanic

Clinical Perspective.

What Is New?

• Between 2007 and 2017, 42 158 congenital heart defect–related deaths occurred among US residents, resulting in nearly 2.1 million years of potential life lost (YPLL) for individuals younger than 65 years.

• The age‐standardized YPLL was highest for non‐Hispanic Black individuals among all racial groups.

• Male individuals experienced a higher YPLL compared with female individuals; there was an overall decline of 17.8% in the average percent change in YPLL during the study period, with the greatest decrease seen in children aged 1 to 4 years and adults aged 35 to 49 years.

What Are The Clinical Implications?

• Patients with congenital heart defects have a higher risk of premature death across the lifespan, but the burden of premature death disproportionately affects minority populations.

• The burden of premature deaths in the population with congenital heart defects has significant implications for resource allocation, economic sustainability, adult intervention, and quality of life throughout the lifespan.

• Given the higher YPLL burden among male individuals, clinicians should prioritize congenital heart defect prevention and management among male patients.

Congenital heart defects (CHDs) are the primary cause of deaths related to birth defects among infants in the United States, with nearly 40% of birth defect–related mortalities occurring in the United States and globally. ¹ , ² , ³ Risk factors such as preterm birth, ⁴ , ⁵ low birth weight, ⁵ CHD phenotype, severity, ⁶ number of co‐occurring birth defects, ⁷ age at surgical procedure, ⁸ types of surgical procedure, ⁹ infant sex, ¹⁰ and race and ethnicity ¹¹ , ¹² , ¹³ , ¹⁴ , ¹⁵ , ¹⁶ , ¹⁷ influence the mortality rate throughout the lifespan of an infant with CHD, from infancy into adulthood. With effective postoperative management and improved long‐term care, many children (85%) will live into their adolescence and adulthood; however, a substantial proportion of them will experience complications and mortality, especially during the early years of life. ¹⁵ , ¹⁸ , ¹⁹ , ²⁰ , ²¹ , ²²

Mortality rates are frequently used to estimate the clinical and public health impact and the relative importance of different causes of death. ²³ , ²⁴ , ²⁵ Although mortality rates play an important role in estimating health status, they do not provide information about premature deaths, which is an important indicator of the health status of a population. ²⁶ Years of potential life lost (YPLL) is a commonly used measure in public health to quantify the impact of mortality on a population ²⁷ and set health priorities. ²⁸ The YPLL estimates the average years a person would have lived if they had not died prematurely. Thus, the YPLL counts age at death rather than only the occurrence of death itself. Compared with standard mortality rates or case‐fatality rates, the YPLL provides more information about the impact of mortality since it includes the temporal component of estimating the loss of life before advanced age. It also reflects the potential deficit to the economy and society due to the loss of productive years within a specific age group, which might be further stratified by sex and race and ethnicity to present a more informative picture.

Although survival statistics are important for CHD surveillance, mortality rates alone cannot completely describe the disease burden caused by premature deaths due to CHDs. Few studies have examined the impact of childhood death caused by chronic conditions, in particular cancer and brain tumors. ²⁹ Studies estimating the impact of premature death due to CHDs are lacking and the source most commonly consulted for YPLL estimates for CHDs is not specific to the US population. ³⁰ Therefore, we aimed to measure the impact of death attributable to CHDs by estimating the YPLL due to CHDs in the United States overall and by age, sex, and race and ethnicity, as well as assessing trends in YPLL from 2007 to 2017.

METHODS

Study Design and Population

All data used in these analyses are publicly available and at no‐cost from the National Center for Health Statistics (NCHS). The analytic methods that support the findings of this study are available from the corresponding author upon reasonable request.

In this cross‐sectional study, we used the multiple cause of death public‐use data files from the NCHS ³¹ from 2007 to 2017. The files are composed of data collected from all death certificates issued in the 50 states in the United States and the District of Columbia. Deaths that occurred to nonresidents, individuals residing outside the continental United States, and during the fetal period are excluded from these files.

Ascertainment of CHD and Cause of Mortality

The multiple cause of death files are compiled from death certificates that contain: (1) a single underlying cause of death, (2) up to 20 conditions listed as a contributing cause of death, (3) demographic, and (4) geographic data on decedents. Deaths are coded using International Classification of Diseases, Tenth Revision, Clinical Modification (ICD‐10‐CM), codes. For the study period, the cause of death was coded following the ICD‐10‐CM guidelines. ³² The ICD‐10‐CM codes in the Q20 to Q26 range (congenital malformations of the circulatory system, specifically the heart and the great veins) were used as our definition of CHDs and to identify individuals with CHD‐related deaths who had a CHD code listed as an underlying cause of death or contributing causes of death. The final study population consisted of individuals with the ICD‐10‐CM codes associated with CHDs listed under any one of the 2 categories: underlying cause of death or contributing cause of death.

Variables

For decedents younger than 1 years old, estimates of the US population were based on live birth certificate data obtained from the NCHS ³³ and were used for the denominator to calculate mortality rates. For decedents 1 year or older, we used the decennial and annual estimates from the US Census Bureau accessed via the Centers for Disease Control and Prevention—Wide‐ranging ONline Data for Epidemiologic Research (CDC‐WONDER) data ³⁴ Application Program Interface system as the denominator population. The years 2007 to 2009 used the intercensal estimates between 2000 and 2010 and the bridged‐race postcensal estimates from 2010 were used for the years 2011 to 2017. ³⁵

We obtained demographic and geographic information on all decedents including age (<1, 1–4, 5–17, 18–34, 35–49, and 50–64 years), sex (male and female), and race and ethnicity. Race and ethnicity are reported as separate variables and were defined as categories used in the bridged‐race population estimates in which the category is first created based on ethnicity, then classified by race (non‐Hispanic [NH] White, NH Black, and Hispanic). Individuals with other or unknown race and ethnicity were excluded from the stratified race and ethnicity results. CHD codes were classified into severe and nonsevere by a pediatric cardiologist (E.B.). The codes and lesion description corresponding to each category are provided in Table S1.

Statistical Analysis

We calculated annual age‐specific YPLL and 95% CIs for any CHD for the population overall and by age group, sex, and race and ethnicity. Total YPLL in each age group was defined as: (the benchmark age – the midpoint of age of premature death in the age group)× total deaths in the age group. ³⁶ The midpoint of an age group was estimated as follows: (lowest age + highest age + 1)/2 to obtain the specific midpoint value. YPLL per 100 000 in this age group was defined as: (total YPLL in the age group/population of the age group) × 100 000. In our analyses, the benchmark age was predetermined at 65 years (the age limit of working life and healthy life expectancy based on quality‐of‐life experience). ³⁶ , ³⁷ For CI estimates, we treated CHD counts as a numerical outcome and calculated its 95% CIs using a normal distribution, and for rare counts (≤100) as a Poisson distribution function. ³⁸ Crude YPLL estimates cumulative of all of the age groups up to 65 years was calculated, accounting for higher burden of CHD‐related mortality during early years. For the age‐standardized YPLL, estimates for each age group were adjusted by the weight age factor specific for that age group from the standard population based on 2010 ³⁹ (Table S2).

We calculated the average percent change (APC) in YPLL, with the series spanning from 2007 as the base year to 2017 as the end point year. The APC was determined using the formula: ((YPLL_{end year} – YPLL_{base year}) × 100/YPLL_{base year}). We also computed the annual APC by dividing the APC by the total number of years encompassed in the series (2007–2017). To estimate the trend in rate of change, we first calculated the year‐over‐year rate of change in YPLL estimates using a variation of the above formula: ((YPLL_{current year} – YPLL_{previous year}) × 100/YPLL_{previous year}). Next, we implemented the Mann‐Kendall Trend test assuming nonparametric distribution using year‐over‐year change with continuity correction to derive the final P value. Since the impact on YPLL is expected to vary by lesion severity, ⁴⁰ we stratified YPLL based on CHD severity. All statistical analyses were performed using R version 3.6.3 (R Core Team). ⁴¹ Since the study used publicly available data, the study was deemed exempt by the institutional review board of the University of Arkansas for Medical Sciences.

RESULTS

In the United States, 28.35 million deaths occurred between 2007 and 2017, of which ≈0.14% were CHD‐related deaths (n=42 158) (Table 1). We found 27 428 deaths with CHD listed as the underlying cause of death and 14 730 with CHD listed as a contributing cause of death. For all CHD‐related deaths combined, most decedents were male (55.9%), NH White (55.8%), and infants at the time of death (53.7%). There were a smaller subset of patients (n=4260) who were diagnosed with multiple CHD conditions and had the highest mortality among infants (72.5%).

Table 1.

Characteristics of Decedents with Congenital Heart Defects as the Underlying or Contributing Cause of Death on the Death Certificate: United States, 2007 to 2017 (n=42 158)

Cause of death
	UCOD*	CCOD†	MCOD‡
Characteristics	(n=27 428), n (%)	(n=14 730), n (%)	(n=4260), n (%)
Age
<1 y	14 524 (53.0)	8129 (55.2)	3087 (72.5)
1–4 y	2009 (7.3)	729 (5.0)	354 (8.3)
5–17 y	3133 (11.4)	1256 (8.5)	247 (5.8)
18–34 y	3127 (11.4)	1756 (11.9)	178 (4.2)
35–49 y	1778 (6.5)	541 (3.7)	224 (5.3)
50–64 y	2857 (10.4)	2319 (15.7)	170 (4.0)
Sex
Female	11 919 (43.5)	6695 (45.5)	2021 (47.4)
Male	15 509 (56.5)	8035 (54.5)	2239 (52.6)
Race and ethnicity§
NH White	15 257 (55.6)	8283 (56.2)	2103 (49.4)
NH Black	5206 (19)	2659 (18.1)	816 (19.2)
Hispanic	5486 (20)	3020 (20.5)	1086 (25.5)

^*UCOD defined as the main condition (ICD code) attributed to the direct cause of death.

^† CCOD defined as a condition (ICD code) listed anywhere in MCOD column‐axis.

^‡ Multiple CHD conditions listed within the same patient.

^§ NH—others and NH—unknowns are not included in the table. Total NH—other and NH—unknown in each group as follows: 2247 in MCOD, 1974 in UCOD, 768 in CCOD, and 255 for multiple CHDs.

CCOD indicates contributing cause of death; CHD, congenital heart defect; ICD, International Classification of Diseases; MCOD, multiple cause of death; NH, non‐Hispanic; and UCOD, underlying cause of death.

YPLL by Age, Sex, and Race and Ethnicity

Table 2 displays the crude, age‐standardized, and total YPLL for each age group overall, as well as by sex and race and ethnicity. Among the population younger than 65 years, the total YPLL was 2.07 million years; of those, a total of 169 756 and 124 067 years were lost for children and adolescents with CHDs, respectively (Table 2). More men died prematurely from CHD than women (1.13 million versus 941 115 years, respectively) (Table 2). Total YPLL was highest for NH White individuals (1.05 million), followed by Hispanic individuals (483 352) and NH Black individuals (420 136 years) (Table 2). The YPLL for people with CHD listed as multiple cause of death by age groups are shown in Tables S3–S8.

Table 2.

Total, Crude, Age‐Standardized, and Mean YPLL (per 100 000 People) for Patients Aged 0 to 64 Years With a CHD‐Related Death^* Overall and by Age, Sex, and Race and Ethnicity: United States, 2007 to 2017

	Total YPLL	Crude YPLL (95% CI)	Age‐standardized YPLL (95% CI)	Mean YPLL (95% CI)
				<1 y	1–4 y	5–17 y	18–34 y	35–49 y	50–64 y
Overall	2 072 605	69.8 (69.1–70.5)	69.2 (68.4–69.9)	3298.2 (3255.3–3341.2)	96.2 (92.6–99.8)	21.0 (20.1–21.8)	21.0 (20.4–21.6)	15.9 (15.5–16.4)	5.9 (5.7–6)
Sex
Female	941 115	63.5 (2.5–64.5)	64.1 (63.1–65.1)	3187.4 (3127–3247.9)	94.7 (89.6–99.8)	17.7 (16.6–18.8)	14.5 (13.8–15.2)	13.0 (12.4–13.6)	4.7 (4.5–4.9)
Male	1 131 490	76 (74.9–77.1)	74.1 (73.0–75.1)	3403.9 (3342.9‐3464.9)	97.6 (92.5–102.7)	24.1 (22.9–25.4)	27.3 (26.3–28.3)	18.9 (18.2–19.6)	7.1 (6.8–7.3)
Race and ethnicity
NH White	1 049 299	57.5 (56.7–58.3)	63.5 (62.6–64.4)	2939.9 (2884.5‐2995.3)	81.4 (76.9–86.0)	18.8 (17.7–19.9)	21.8 (21.0–22.6)	18.4 (17.8–19.1)	6.5 (6.3–6.7)
NH Black	420 135.5	104.4 (102–106.8)	95.5 (93.2–97.7)	4577.2 (4445.6‐4708.8)	142.1 (130.8–153.3)	34.1 (31.4–36.9)	29.1 (27.1–31.0)	16.2 (14.9–17.4)	5.2 (4.8–5.7)
Hispanic	483 351.5	88.8 (86.8–90.8)	69.7 (68.1–71.2)	3611.2 (3518.6‐3703.8)	98.0 (90.8–105.2)	17.3 (15.7–18.9)	15.1 (13.9–16.3)	9.8 (8.9–10.6)	3.8 (3.4–4.2)

^*Decedent had a CHD listed as an underlying or contributing cause of death listed on death certificate.

CHD indicates congenital heart defect; NH, non‐Hispanic; and YPLL, years of potential lives lost.

The overall mean YPLL for all CHD decedents was 70 years per 100 000 (data not shown). The mean YPLL per 100 000 was highest for infants (3298.2 [95% CI, 3255.3–3341.2]) followed by children ages 1 to 4 years (96.2 [95% CI, 92.6–99.8]). Men had a higher crude (76 [95% CI, 74.9–77.1]) and age‐standardized YPLL (74.1 [95% CI, 73.0–75.1]) per 100 000 than women. Although NH Black individuals had the lowest total YPLL, both crude and age‐standardized YPLL estimates (per 100 000) were higher among NH Black individuals (104.4 [95% CI, 102.0–106.8] and 95.5 [95% CI, 93.2–97.7], respectively) than NH White and Hispanic individuals (Table 2, Figure S1).

Total, crude, and age‐standardized YPLL by CHD severity are shown in Table S9. Individuals with nonsevere CHD had a total of 1 247 927.5 YPLL, whereas those with severe CHD had a total of 824 677.5 YPLL, likely because of the larger number of nonsevere cases. Decedents with nonsevere CHD had a higher crude (42 [95% CI, 41.4–42.6]) and age‐standardized (41.7 [95% CI, 41.1–42.2]) YPLL per 100 000 than decedents with severe CHD.

Mean YPLL by Sex and Race and Ethnicity

Table 3 displays the mean YPLL (per 100 000) for individuals with CHDs by race and ethnicity and sex combined. NH Black infants (both female  and male) had the highest mean YPLL (boys: 4526.5 [95% CI, 4345.1–4707.9] and girls: 4406.4 [95% CI, 4244.5–4588.3) followed by Hispanic boys and girls. This pattern persisted among children aged 1 to 4 years. NH Black children (both male and female) also had the highest mean YPLL among children aged 5 to 17 years (male: 39.8 [95% CI, 35.6–44.0] and female: 28.3 [95% CI, 24.7–31.9]); however, NH White men had the second highest mean YPLL, followed by Hispanic men (Table 3). NH Black men also had the highest mean YPLL among adults 18 to 34 years. However, NH White men had the highest mean YPLL for ages 35 to 49 years and 50 to 64 years, whereas Hispanic men and women had the lowest mean YPLL for both age groups (Table 3, Figure S1).

Table 3.

Total and Mean YPLL (per 100 000 Person) for People Aged 0 to 64 Years With a CHD‐Related Death^* by Race and Ethnicity, Sex, and Age Group: United States, 2007 to 2017

	NH White		NH Black		Hispanic
	Total YPLL	Mean YPLL (95% CI)	Total YPLL	Mean YPLL (95% CI)	Total YPLL	Mean YPLL (95% CI)
<1 y
Female	321 468	2864.5 (2784.9‐2944)	145 383.00	4406.4 (4224.5‐4588.3)	181 180.5	3258.6 (3138.1–3379.1)
Male	377 260.5	3195.2 (3113.3‐3277.1)	154 348.50	4526.5 (4345.1–4707.9)	195 499.5	3384 (3263.5‐3504.4)
1–4 y
Female	36 828	80.8 (74.3–87.3)	18 228	138.3 (122.5–154.2)	20 708	94.2 (84.1–104.3)
Male	39 308	82.0 (75.7–88.4)	19 840	145.7 (129.7–161.6)	23 250	101.6 (91.4–111.9)
5–17 y
Female	24 610	15.4 (14.0–16.8)	12 519	28.3 (24.7–31.9)	10 593	16 (13.7–18.1)
Male	37 182.5	22.1 (20.4–23.7)	18 190	39.8 (35.6–44)	12 947	18.7 (16.3–21)
18–34 y
Female	34 496	15 (14.0–16.0)	12 012	20.6 (18.3–22.9)	8162	10.4 (9–11.8)
Male	67 221	28.4 (27.1–29.7)	21 367.5	37.8 (34.7–41)	16 709	19.3 (17.5–21.2)
35–49 y
Female	33 165	15.3 (14.5–16.1)	6277.5	13.4 (11.9–15)	3892.5	6.7 (5.7–7.7)
Male	46 800	21.6 (20.6–22.5)	8010	19.3 (17.3–21.3)	7762.5	12.8 (11.4–14.1)
50–64 y
Female	12 585	5.2 (4.9–5.4)	1920	4.7 (4.1–5.3)	1095	3.1 (2.6–3.6)
Male	1552.5	7.8 (7.5–8.1)	2040	5.9 (5.2–6.5)	1552.5	4.6 (4–5.2)
Crude YPLL
Female	463 152	51.0 (49.9–52.1)	196 339.5	94.9 (91.6–98.2)	225 631	84.7 (82.0–87.4)
Male	586 147	63.9 (62.7–65.1)	223 796	114.4 (110.8–118)	257 720.5	92.3 (89.5–95.1)
Age‐standardized YPLL
Female	463 152	58.8 (57.5–60.2)	196 339.5	88.5 (85.5–91.5)	225 631	61.8 (59.8–63.8)
Male	586 147	70.8 (69.4–72.1)	223 796	99.3 (96.2–102.4)	257 720.5	68.8 (66.8–70.9)
Total	1 049 299		420 135.5		483 351.5

^*Deceased had a CHD listed as an underlying or contributing cause of death listed on death certificate.

The crude and age‐standardized YPLL presented in this table are calculated as explained in the Methods section.

CHD indicates congenital heart defect; NH, non‐Hispanic; YPLL, years of potential lives lost.

APC in YPLL

During the study period, the YPLL declined overall and by race and ethnicity and sex (Table 4; Figures S2–S4). During the study period, the APC in YPLL for CHD declined by 17.8%, while the annual APC declined by 1.6%. During the overall study period, the average percent decrease for women was less than for men. NH Black individuals had the highest decrease in APC (23%) followed by NH White (19%) and Hispanic individuals (8.2%). NH Black individuals had the highest decrease in APC (29.4%) followed by NH White individuals (20%) and Hispanic individuals (17.3%). However, among women, NH White individuals experienced the highest decline in APC (23%), followed by NH Black (19.7%) and Hispanic individuals (14.8%).

Table 4.

APC and AAPC for the Study Period for YPLL for People Aged 0 to 64 Years With a CHD‐Related Death^* by Sex, Race and Ethnicity, and Sex: United States, 2007 to 2017

Age groups	0–64 y		<1 y		1–4 y		5–17 y		18–34 y		35–49 y		50–64 y
	APC†	AAPC‡	APC	AAPC	APC	AAPC	APC	AAPC	APC	AAPC	APC	AAPC	APC	AAPC
Overall	−17.8	−1.6	−17.4	−1.6	−31.6	−2.9	−11.2	−1.0	−9.0	−0.8	−24.3	−2.2	−3.2	−0.3
Sex
Female	−17	−1.5	−12.3	−1.1	−38.2	−3.5	−18.4	−1.7	−23.7	−2.2	−37.1	−3.4	−4.0	−0.4
Male	−18.4	−1.7	−21.9	−2.0	−25.2	−2.3	−5.5	−0.5	−1.1	−0.1	−14.3	−1.3	0.0	0.0
Race and ethnicity
NH White	−19	−1.7	−19.3	−1.8	−41.0	−3.7	−17.7	−1.6	−3.2	−0.3	−18.0	−1.6	0.0	0.0
NH Black	−23.8	−2.2	−25.4	−2.3	−19.8	−1.8	−8.5	−0.8	−24.4	−2.2	−38.2	−3.5	6.8	0.6
Hispanic	−8.2	−0.7	−5.9	−0.5	−29.8	−2.7	3.6	0.3	−5.5	−0.5	−16.1	−1.5	−15.9	−1.4
Race and ethnicity and sex
Male sex
NH White	−20	−1.8	−24.8	−2.3	−36.8	−3.3	−18.3	−1.7	13.8	1.3	−1.9	−0.2	0.0	0.0
NH Black	−29.4	−2.7	−33.8	−3.1	−19.3	−1.8	17.3	1.6	−31.3	−2.8	−51.2	−4.7	17.7	1.6
Hispanic	−17.3	−1.6	−22.3	−2.0	−15.3	−1.4	23.7	2.2	1.6	0.1	−15.6	−1.4	0.0	0.0
Female sex
NH White	−23	−2.1	−19.9	−1.8	−45.2	−4.1	−16.1	−1.5	−25.9	−2.4	−36.4	−3.3	1.9	0.2
NH Black	−19.7	−1.8	−18.9	−1.7	−20.3	−1.8	−37.2	−3.4	−12.3	−1.1	−16.4	−1.5	−3.5	−0.3
Hispanic	−14.8	−1.3	−10.0	−0.9	−44.7	−4.1	−13.9	−1.3	−21.6	−2.0	−16.4	−1.5	−33.3	−3.0

^*Decedent had a CHD listed as an underlying or contributing cause of death listed on death certificate.

^† For the APC, the calculation was performed by using the 2007 as base year and 2017 as the end year.

^‡ For the AAPC, the calculation was performed by dividing the APC over the entire length of time series.

APC indicates average percent change; AAPC, annual average percent change; CHD, congenital heart defect; NH, non‐Hispanic; and YPLL, years of potential life lost.

The APC in YPLL between 2007 and 2017 was highest for children aged 1 to 4 years (−31.6%) followed by adults aged 35 to 49 years (−24.3%) and infants (−17.4%) (Table 4). Among women, the APC in YPLL followed a similar pattern when stratified by age groups; however, men had a slightly different pattern. The APC in YPLL was greatest among male individuals aged 1 to 4 years, followed by infants and men aged 35 to 49 years (Table 4). The year‐on‐year change in rate did not show any statistically significant trend for the same study period (2007–2017) (Table S10).

DISCUSSION

We sought to estimate the YPLL to better characterize the impact of mortality associated with CHDs across the lifespan. Overall, during the 10‐year study period, the total YPLL for individuals younger than 65 years was 2.1 million years, and male individuals experienced more than half of the estimated YPLL. Racial and ethnic disparities in CHD‐attributable age‐standardized YPLL was also observed, with a prevailing pattern of NH Black and Hispanic individuals experiencing disproportionately high CHD‐attributable age‐standardized YPLL. In general, the YPLL declined in all age groups; however, it was more pronounced among children aged 1 to 4 years and adults aged 35 to 49 years. Compared with other racial and ethnic groups, NH Black individuals experienced the greatest decline, particularly for adults aged 35 to 49 years and infants. As might be expected, YPLL varied by CHD severity; nonsevere CHD was associated with a higher number of YPLL when adjusted for age. Since CHDs are associated with a higher mortality during infancy and childhood, the YPLL estimates reported have important public health implications for individuals with CHDs in the United States.

In comparison to other common conditions among children and adolescents in the United States (eg, including pediatric cancer, ²⁹ leukemia, central nervous system tumors, and both Hodgkin lymphoma and non‐Hodgkin lymphoma), ⁴² , ⁴³ premature deaths from CHDs are much higher. For example, in 2009 (the most recent data available), a total of 153 390 years were lost prematurely due to cancer among children and adolescents combined. ²⁹ Among those, 47 631 years lost were due to brain and central nervous system neoplasms while 43 854 years were lost due to leukemia. ²⁹ Additionally, 5055 and 1383 YPLL lost were due to non‐Hodgkin and Hodgkin lymphoma, respectively. ²⁹ We found that 169 756 and 124 067 years were lost for children and adolescents with CHDs, respectively, which is higher than combined estimates of YPLL from NCHS data (children and adolescents) for pediatric cancers, specifically myelomonocytic leukemia (108 510 years), lymphoid leukemia (135 366 years), and Hodgkins lymphoma (4762 years) (data not shown). Thus, despite improvements in treatment and management of infants and children with CHDs during the past few decades, the impact on mortality and number of lives lost prematurely remains significant.

Comparison of our results to the published literature is hampered by the paucity of relevant publications, but a few publications ³⁰ , ⁴⁴ were identified. Vos et al reported from the Global Burden of Diseases, Injuries, and Risk Factors Study in 2019 ³⁰ that overall, CHDs caused 18.1 million (95% uncertainty interval (UI), 14.7–22.1) years of life lost globally in 2019. ³⁰ The years of life lost rate for CHDs was 272.4 per 100 000 (95% UI, 220.3–333.7) and male individuals had a higher rate of years of life lost than female individuals. ³⁰ They also found that between 2010 and 2019, the percent change in age‐standardized years of life lost rates overall decreased by 21.9%. ³⁰ We observed similar results in our study. However, in contrast to our findings, Vos et al found that the percent change was greater for female than male individuals. ³⁰

We found that NH Black individuals had a higher YPLL than NH White and Hispanic individuals with CHDs until age 34; thus, NH Black children, adolescents, and young adults with CHDs are dying more prematurely than their counterparts. This is most evident in the APC during the study period. NH Black children (ages 1–17 years) experienced less decline in YPLL during the study period than NH White or Hispanic children. However, NH Black infants experienced the greatest decline in YPLL, which suggests that improvements in surgical interventions and management of infants with CHDs may have benefitted NH Black children most during the study period. Similar racial and ethnic disparities were previously reported by Lopez and colleagues ⁴⁵ for mortality due to CHDs in the United States between 1999 and 2017, by Gilboa and colleagues for mortality due to CHDs in the United States between 1999 and 2006, ¹⁹ and by Boneva and colleagues for 1979 to 1997. ¹⁸ Nembhard and colleagues ¹⁵ also reported that NH Black male individuals had a greater risk of dying from CHDs than NH White male individuals. However, none of those studies reported YPLL estimates, only mortality rates.

Explanations for this ongoing racial and ethnic disparity remain unknown but are likely complex and multifactorial, involving social determinants of health, including disparities in health insurance, access to quality health care, and structural racism. A missed prenatal diagnosis is more likely to occur in families living in rural communities. ⁴⁶ Moreover, pediatric cardiac centers specializing in CHDs provide advanced knowledge and resources, delivering care to complex patients. A national population‐based study ⁴⁷ found that infant mortality rates are 28% higher for those who do not live in proximity to a top‐50 center than those who do. Distance to care has also been linked to socioeconomic status. Mothers of infants with hypoplastic left heart syndrome who traveled from a further distance typically lived in areas with >20% poverty and were less likely to have completed >12 years of schooling. ⁴⁸ Therefore, there is a need for efforts and interventions to reduce mortality disparities among NH Black newborns, children, and young adults. Findings from this study contribute to the growing evidence of racial and ethnic disparities among individuals with CHDs and the significant impact of mortality in minority populations.

Our study has several strengths. We used death records for the entire noninstitutionalized US population during an 11‐year period, which allowed us to determine population‐based YPLL estimates specific to the United States. Unlike prior studies, we were able to calculate sex‐ and race‐ and ethnic‐specific YPLL estimates. When analyzing premature mortality in populations, it is typical to use either the gross or age‐adjusted mortality rate. However, these measurements may be heavily influenced by deaths among the older population. ⁴⁹ Due to variations in populations, YPLL is a more accurate measure of disease burden in populations because it accounts for premature death at younger ages, can be adjusted to compare populations with different age structures, and can be tailored to use age limits specific to the populations under study. ⁵⁰ As with any study, the use of death certificates for conditions such as CHDs has notable limitations, despite the strengths inherent in these data sources. The International Classification of Diseases, Ninth Revision (ICD‐9) and International Classification of Diseases, Tenth Revision (ICD‐10) codes have inherent constraints in accurately classifying CHDs, largely due to the quality and completeness of information recorded on death certificates. ⁵¹ Studies show that ICD‐9 codes can misclassify CHD cases, with accuracy rates as low as 48.7% for diagnosing CHD. ⁵¹ Although ICD‐10 codes have improved in some respects, their effectiveness in reflecting the true burden of CHD remains variable and dependent on factors such as code specificity and data collection practices. ⁵² Therefore, while ICD‐10 codes have been used on death certificates since 1999 and remain the primary source of mortality data in the United States, ¹⁵ they also present challenges in accurately capturing the full scope of CHD, which can impact research and policy decisions. ⁵³ Another limitation of our study is the cross‐sectional design. We did not perform follow‐up on a large defined cohort of individuals with CHDs to determine their mortality experience over time. We also know that it is highly likely that CHDs are underreported on death certificates, especially at older ages. Unless the health care professional, coroner, or person completing the death certificate knew the person had a CHD, the CHD may not have been listed as a contributing cause of death on the death certificate. For these reasons, our YPLL estimates most likely underestimate the true impact of CHDs on premature death at all age groups. Last, patients with CHD are more likely to have significant genetic syndromes ⁵⁴ and noncardiac structural anomalies ⁵⁵ ; however, we were unable to consider additional patient‐related factors beyond age‐standardized YPLL.

The determination of YPLL in our study provides estimates of premature loss of life due to CHD‐associated mortality, which is highly informative for public health professionals, genetic counselors, and clinicians, especially pediatric cardiology. These findings underscore the tremendous impact that CHDs have on affected populations regarding the short‐ and long‐term consequences of CHDs among survivors of CHDs in the US population. This also informs policymakers and health care leaders when deciding how to allocate resources to improve outcomes and quality of life for individuals with CHDs across the lifespan. Our results also quantify the significance of the differential impact that CHDs have across the lifespan for different racial and ethnic groups.

Sources of Funding

None.

Supporting information

Tables S1–S10

Figures S1–S4