Coarctation of the Aorta
Alaska, 2007-2017
Background
The aorta is the main artery that carries oxygenated blood out of the heart to the body. With coarctation of the aorta, a portion of the aorta is too narrow, resulting in reduced blood flow, most commonly to the lower body. [1] Symptoms develop soon after birth and may include congestive heart failure or high blood pressure. [2] A coarctation obstruction can be treated by various surgeries and procedures that remove, bypass, or repair the narrow section of the aorta. [3] Causes of coarctation of the aorta, among newborns are unknown. However, coarctation of the aorta and other heart defects like it are thought to be caused by a combination of genetic and environmental factors. [4]
Epidemiology
Alaska Birth Defects Registry (ABDR) registers birth defects as reported from health care providers using International Classification of Disease (ICD) billing codes. The use of these ICD codes can lead to misclassification of diagnosed conditions. Prior to this report, all prevalence estimates were based on the number of unique children reported to ABDR with an ICD code representing a specified condition regardless of case confirmation status.
The estimates in this report were derived by conducting medical record review and case confirmation of a random sample of cases of the condition reported to ABDR. The confirmation probability from the sample was used to develop informed estimates of the actual diagnosed defect prevalence. See Defect prevalence calculation.
For explanations of table columns see Column descriptions.
Prevalence
Coarctation of the Aorta occurs in about 4 out of every 10,000 live births in the United States. This results in about 2,200 babies diagnosed with Coarctation of the Aorta nationally each year.[4]
In Alaska, during 2007-2017, the prevalence of Coarctation at the Aorta was 2.7 per 10,000 live births.| Reports | Defects | Births | Prevalence (95% CI) | |
|---|---|---|---|---|
| Total | 58 | 33.9 | 123630 | 2.7 (1.9, 3.7) |
| Notes: 95% CI = 95% Confidence Interval | ||||
Trend
Prevalence per 10,000 births of Coarctation at the Aorta during 2007-2017 by five-year moving averages, with 95% confidence interval band and Poisson estimated fitted line.
| Reports | Defects | Births | Prevalence (95% CI) | Predicted Prevalence† | |
|---|---|---|---|---|---|
| 2007-2011 | 7.0 | 4.1 | 11404.7 | 3.6 (1.4, 8.4) | 3.6 |
| 2008-2012 | 7.0 | 4.1 | 11354.0 | 3.6 (1.4, 8.4) | 3.4 |
| 2009-2013 | 6.7 | 3.9 | 11349.0 | 3.4 (1.4, 8.4) | 3.2 |
| 2010-2014 | 4.0 | 2.4 | 11334.7 | 2.1 (0.6, 6.3) | 3.0 |
| 2011-2015 | 6.0 | 3.5 | 11377.3 | 3.1 (1.0, 7.6) | 2.8 |
| 2012-2016 | 4.7 | 2.8 | 11295.0 | 2.4 (0.6, 6.3) | 2.7 |
| 2013-2017 | 5.3 | 3.1 | 10979.7 | 2.8 (0.7, 7.0) | 2.5 |
|
Notes: Each row is based on five-year moving averages; Prevalence reported per 10,000 live births; 95% CI=95% Confidence Interval † Estimated rate based on Poisson model |
|||||
| Estimate | Std. Error | t value | Pr(>|t|) |
|---|---|---|---|
| -0.05823 | 0.03116 | -1.86887 | 0.12059 |
Regional Distribution
Distribution of Coarctation at the Aorta in Alaska by Public Health Region of maternal residence at the time of birth. A description of regional breakdowns can be found here. Data suppressed for # of reports < 6.
| Reports | Defects | Births | Prevalence (95% CI) | |
|---|---|---|---|---|
| Anchorage | 22 | 12.9 | 50455 | 2.6 (1.4, 4.2) |
| Gulf Coast | 9 | 5.2 | 7725 | 6.8 (2.9, 15.1) |
| Interior | 8 | 4.7 | 22487 | 2.1 (0.7, 4.6) |
| Mat-Su | 9 | 5.3 | 14937 | 3.5 (1.5, 7.8) |
| Northern | - | - | 8520 | - |
| Southeast | - | - | 7625 | - |
| Southwest | 6 | 3.5 | 11881 | 3 (0.9, 7.4) |
| Notes:Prevalence reported per 10,000 live births; Data suppressed for # of reports < 6; 95% CI = 95% Confidence Interval | ||||
Demographics
Some subgroups may be more at risk for having a baby with Coarctation at the Aorta. This section provides the descriptive epidemiology of specified maternal, birth, and child characteristics identified from the birth certificate.
| Reports | Defects | Births | Prevalence (95% CI) | |
|---|---|---|---|---|
| Sex | ||||
| Female | 24 | 14.1 | 59998 | 2.4 (1.4, 3.9) |
| Male | 34 | 19.9 | 63632 | 3.1 (1.9, 4.7) |
| Birth weight (grams) | ||||
| <2500 | 13 | 7.5 | 7231 | 10.4 (4.8, 20.0) |
| 2500+ | 45 | 26.4 | 116215 | 2.3 (1.5, 3.3) |
| Maternal age | ||||
| 12-19 | - | - | 9156 | - |
| 20-24 | 18 | 10.5 | 32872 | 3.2 (1.7, 5.6) |
| 25-29 | 19 | 11.1 | 37743 | 2.9 (1.6, 5.2) |
| 30-34 | 12 | 7 | 28152 | 2.5 (1.2, 5.1) |
| 35-39 | - | - | 12614 | - |
| 40+ | - | - | 3064 | - |
| Maternal race | ||||
| Alaska Native/American Indian | 16 | 9.4 | 31560 | 3.0 (1.5, 5.4) |
| Asian/Pacific Islander | - | - | 11810 | - |
| Black | - | - | 5081 | - |
| White | 36 | 21.1 | 73595 | 2.9 (1.9, 4.4) |
| Maternal education (years) | ||||
| <12 | 15 | 8.7 | 11929 | 7.3 (3.5, 13.2) |
| 12 | 22 | 12.9 | 43162 | 3.0 (1.6, 4.9) |
| 12+ | 20 | 11.8 | 65105 | 1.8 (0.9, 3.0) |
| Marital status | ||||
| Married | 34 | 19.9 | 78396 | 2.5 (1.6, 3.8) |
| Unmarried | 22 | 12.9 | 44625 | 2.9 (1.6, 4.7) |
| Maternal smoking use | ||||
| Reported smoking | 7 | 4.1 | 16817 | 2.4 (1.0, 6.1) |
| Reported not smoking | 49 | 28.7 | 104707 | 2.7 (1.9, 3.9) |
| Medicaid (mother or child) | ||||
| Medicaid | 42 | 24.5 | 62666 | 3.9 (2.6, 5.7) |
| non-Medicaid | 15 | 8.9 | 60839 | 1.5 (0.7, 2.6) |
| Father on birth certificate | ||||
| None | 9 | 5.2 | 6328 | 8.2 (3.5, 18.4) |
| Present | 49 | 28.7 | 117302 | 2.5 (1.7, 3.5) |
| Notes: Prevalence reported per 10,000 live births; Data suppressed for # of reports < 6; 95% CI = 95% Confidence Interval | ||||
Technical notes
Column descriptions
# Reports: Unless otherwise noted, the number of unique reports of the defect received by ABDR during the specified birth year(s). Each report represents a unique child with the specified defect.
# Defects: The estimated true number of reports that are diagnosed defects based on medical record review and case confirmation.
# Births: The number of live births among Alaskan residents that occurred in Alaska during the specified birth year(s).
Prevalence (95% CI): The estimated diagnosed prevalence of the condition and corresponding 95% Confidence Interval. (For information on how the defect prevalence was estimated see below).
Defect prevalence calculation
The estimated defect prevalence was calculated using a Bayesian approach based on the reported prevalence, PPV and 1-NPV (see formula below).
Through medical records review and case confirmation of a random sample of reported cases, the defect prevalence is calculated as:
\[PPV (Positive Predictive Value) = p(defect|report)\] \[NPV (Negative Predictive Value) = p(\overline{defect}|\overline{report})\]
\[p(defect) \approx [p(report)\cdot PPV]+[p(\overline{report})\cdot (1-NPV)]\]
Defect prevalence estimates are a more accurate estimation of the actual diagnosed prevalance of birth defects compared to the reported prevalance estimates in Alaska. ABDR obtains reports from medical providers using International Classification of Disease (ICD) codes that are extracted from individual systems which when aggregated may not reflect true diagnostics. Caution should be used when interpreting and comparing the reported prevalence estimates with national estimates.
See Data analysis methods for more information.
P-value estimate
To evaluate the trend over time and account for under/over-dispersion we constructed a quasi-Poisson regression model. This model assumes the variance is a linear function of the mean and models the estimated number of annual defects by year with a natural log (ln) offset of the annual births. P-values < 0.05 are considered significant, which indicates that the predicted slope is significantly different from a slope of zero.
Data suppression
For region and demographic data tables, values are suppressed based on the number of reports received during the observation period. Counts less than 6 are suppressed (as indicated by ‘-’ in the table). For regions or demographics with only one cell count suppressed a second is suppressed to eliminate the ability to back-calculate the estimate.
References
[1] Coarctation of the Aorta | Children’s Hospital of Philadelphia. (2021). Retrieved 22 January 2021, from https://www.chop.edu/conditions-diseases/coarctation-aorta
[2] Coarctation of the Aorta (CoA). (2021). Retrieved 22 January 2021, from https://www.heart.org/en/health-topics/congenital-heart-defects/about-congenital-heart-defects/coarctation-of-the-aorta-coa
[3] Coarctation of the aorta - Symptoms and causes. (2021). Retrieved 22 January 2021, from https://www.mayoclinic.org/diseases-conditions/coarctation-of-the-aorta/symptoms-causes/syc-20352529
[4] Centers for Disease Control and Prevention. Congenital Heart Defects - Facts about Coarctation of the Aorta | CDC. (2019). Retrieved 6 March 2021, from https://www.cdc.gov/ncbddd/heartdefects/coarctationofaorta.html
Suggested Citation
State of Alaska Department of Health and Social Services, Division of Public Health, Section of Women’s, Children’s, and Family Health. Alaska Birth Defects Registry Condition Report: Coarctation of the Aorta, Alaska, 2007-2017. Updated June 22, 2021. Available at: http://rpubs.com/AK_ABDR/Coarctation of the Aorta/07_17.
Contact
Alaska Birth Defects Registry (ABDR)
3601 C Street, Suite 358
Anchorage, AK 99503
(907) 269-3400 phone
(907) 754-3529 fax
hssbirthdefreg@alaska.gov
Updated: June 22, 2021
Code source: R:\ABDR\Analysis_New\ABDR_CASECONF\cond_reports\Published_reports\Coarctation_Aorta07_17.Rmd
