Background

Tricuspid atresia is a birth defect of the heart. The tricuspid valve controls blood flow between the two chambers on the right side of the heart (right atrium and right ventricle). Tricuspid atresia occurs when this valve forms incorrectly, or not at all. [1] A malformed, or absent tricuspid valve affects blood flow to the right ventricle and the main pulmonary artery, causing them to become underdeveloped. [2] It is common for newborns with tricuspid atresia also have, a hole between the right and left sides of the heart (either an atrial septal defect or a ventricular septal defect). Through these defects, oxygen-rich and oxygen-poor blood mix and this mixture of blood is then pumped to the rest of the body by the functioning left ventricle. [1]

Tricuspid atresia can be detected during pregnancy through ultrasound or after birth, with, pulse oximeter, echocardiogram, Chest X-ray, or blood test. [3] Symptoms of tricuspid atresia include rapid breathing or heart rate, blue skin tone (cyanosis), sweating, disinterest in feeding, poor weight gain, and heart murmur. [4] Currently, the cause of tricuspid atresia is unknown. Treatment of tricuspid atresia include medication to strengthen the heart and lower blood pressure, high-calorie formula to encourage adequate weight gain, and surgery to improve the function of the right side of the heart. [1] In some cases, total heart transplantation may be needed. Individuals recovering from treatment of tricuspid atresia will require regular visits with a pediatric cardiologist to monitor their progress and check for other health conditions that may develop. Additional surgeries and medical care may also be necessary well into adulthood. [1]

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 all reported cases between 2007 and 2018. The confirmation probability calculated from this time period is used to develop informed estimates of the defect prevalence beyond 2018. See Defect prevalence calculation.

For explanations of table columns see Column descriptions.

Prevalence

Single ventricle occurs in about 1 in every 10,000 live births in the United States. [5]

In Alaska, during 2007-2017, the prevalence of Tricuspid atresia was 0.3 per 10,000 live births.
Reports Defects Births Prevalence (95% CI)
Total 8 3.3 123630 0.3 (0.1, 0.7)
Notes: 95% CI = 95% Confidence Interval

Trend

Prevalence per 10,000 births of Tricuspid atresia 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 0.3 0.1 11404.7 0.1 (0, 3.2) 0.2
2008-2012 0.3 0.1 11354.0 0.1 (0, 3.2) 0.2
2009-2013 0.7 0.3 11349.0 0.3 (0, 3.2) 0.2
2010-2014 0.7 0.3 11334.7 0.3 (0, 3.2) 0.1
2011-2015 0.7 0.3 11377.3 0.3 (0, 3.2) 0.1
2012-2016 0.3 0.1 11295.0 0.1 (0, 3.3) 0.1
2013-20117 0.0 0.0 10979.7 0.0 (0, 3.4) 0.1
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
The p-value test for trend did not detect a significant change in number of live births with Tricuspid atresia during 2007-2017. See p-value estimate
Estimate Std. Error t value Pr(>|t|)
-0.07989 0.12265 -0.65133 0.54358

Regional Distribution

Distribution of Tricuspid atresia 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 - - 50455 -
Gulf Coast - - 7725 -
Interior - - 22487 -
Mat-Su - - 14937 -
Northern - - 8520 -
Southeast - - 7625 -
Southwest - - 11881 -
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 Tricuspid atresia. 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 - - 59998 -
  Male - - 63632 0.4 (0.1, 1.1)
Birth weight (grams)
  <2500 - - 7231 -
  2500+ - - 116215 0.2 (0.0, 0.6)
Maternal age
  12-19 - - 9156 -
  20-24 - - 32872 -
  25-29 - - 37743 -
  30-34 - - 28152 -
  35-39 - - 12614 -
  40+ - - 3064 -
Maternal race
  Alaska Native/American Indian - - 31560 -
  Asian/Pacific Islander - - 11810 -
  Black - - 5081 -
  White - - 73595 -
Maternal education (years)
  <12 - - 11929 -
  12 - - 43162 -
  12+ - - 65105 -
Marital status
  Married - - 78396 -
  Unmarried - - 44625 -
Maternal smoking use
  Reported smoking - - 16817 -
  Reported not smoking - - 104707 0.3 (0.1, 0.7)
Medicaid (mother or child)
  Medicaid - - 62666 0.4 (0.1, 1.1)
  non-Medicaid - - 60839 -
Father on birth certificate
  None - - 6328 -
  Present - - 117302 0.3 (0.1, 0.8)
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] Congenital Heart Defects - Facts about Tricuspid Atresia | CDC. (2019). Retrieved 12 April 2021, from https://www.cdc.gov/ncbddd/heartdefects/tricuspid-atresia.html

[2] Tricuspid atresia - Symptoms and causes. (2021). Retrieved 13 April 2021, from https://www.mayoclinic.org/diseases-conditions/tricuspid-atresia/symptoms-causes/syc-20368392

[3] Tricuspid Atresia | Children’s Hospital of Philadelphia. (2021). Retrieved 13 April 2021, from https://www.chop.edu/conditions-diseases/tricuspid-atresia

[4] Tricuspid Atresia | Symptoms & Causes | Boston Children’s Hospital. (2021). Retrieved 13 April 2021, from https://www.childrenshospital.org/conditions-and-treatments/conditions/t/tricuspid-atresia/symptoms-and-causes

[5] Mai CT, Isenburg JL, Canfield MA, Meyer RE, Correa A, Alverson CJ, Lupo PJ, Riehle-Colarusso T, Cho SJ, Aggarwal D, Kirby RS; National Birth Defects Prevention Network. National population-based estimates for major birth defects, 2010-2014. Birth Defects Res. 2019 Nov 1;111(18):1420-1435. doi: 10.1002/bdr2.1589. Epub 2019 Oct 3. PMID: 31580536; PMCID: PMC7203968.

Authorship

Maternal and Child Health (MCH) senior epidemiologist Dr. Jared Parrish, PhD conceived of the presented analysis. Alaska Birth Defects Registry program manager and epidemiologist Chris Barnett, MS MPH and Dr. Jared Parrish, PhD developed the theory and performed the computations. Research analysts Monica Mills and Jordyn Lord managed the project and data storage. Pediatric cardiologist Dr. James Christiansen, MD performed defect confirmations and provided medical consultation. All authors discussed the results and contributed to the final report.

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: Tricuspid Atresia, Alaska, 2007-2017. Updated June 22, 2021. Available at: http://rpubs.com/AK_ABDR/tricuspid_atresia07_17.

Contact

Alaska Birth Defects Registry (ABDR)
3601 C Street, Suite 358
Anchorage, AK 99503
(907) 269-3400 phone
(907) 754-3529 fax

Updated: June 22, 2021

Code source: R:\ABDR\Analysis_New\ABDR_CASECONF\cond_reports\Published_reports\tricuspid_atresia07_17.Rmd