Background

Total Anomalous Pulmonary Venous Return (TAPVR) is a birth defect of the heart that occurs when oxygen-rich blood from the lungs does not return normally to the left atrium, but rather to the right atrium. Babies with TAPVR usually have a hole between the right and left atrium (an atrial septal defect) that allows oxygenated and deoxygenated blood to mix throughout the heart. [1] Mixed blood on the left side of the heart is then pumped to the rest of the body. [2] There are four types of TAPVR – 1) Supracardiac, where the pulmonary veins connect above the heart, Cardiac, where the pulmonary veins connect behind the heart, Infracardiac, where the pulmonary veins connect below the heart, and Mixed, which is a combination of any of the previously listed connections. [3] TAPVR is usually diagnosed after birth by pulse oximetry, chest x-ray, and echocardiography, but can be diagnosed during pregnancy. [1] Some of the signs and symptoms of TAPVR are cyanosis (blue skin tone) respiratory distress, and heart murmur. [4] Surgery is the most common treatment for this condition. Surgical repair includes connecting the pulmonary veins to the left atrium, closing the atrial septal defect and any other abnormal connections to the heart. [1] Because an infant with total anomalous pulmonary venous return may need these procedures soon after birth, this defect is considered a critical congenital heart defect.


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 derived 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

Total Anomalous Pulmonary Venous Return occurs in about 1.3 out of every 10,000 live births in the United States. This results in about 504 babies diagnosed with Total Anomalous Pulmonary Venous Return nationally each year.[1]

In Alaska, during 2007-2017, the prevalence of Total Anomalous Pulmonary Venous Return was 1.4 per 10,000 live births.
Reports Defects Births Prevalence (95% CI)
Total 20 16.8 123630 1.4 (0.8, 2.1)
Notes: 95% CI = 95% Confidence Interval

Trend

Prevalence per 10,000 births of Total Anomalous Pulmonary Venous Return 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 1.7 1.4 11404.7 1.2 (0.2, 4.3) 0.7
2008-2012 1.0 0.8 11354.0 0.7 (0.0, 3.2) 0.8
2009-2013 0.7 0.5 11349.0 0.5 (0.0, 3.2) 0.9
2010-2014 1.0 0.8 11334.7 0.8 (0.1, 3.8) 1.0
2011-2015 1.3 1.1 11377.3 1.0 (0.1, 4.3) 1.2
2012-2016 2.0 1.7 11295.0 1.5 (0.2, 4.9) 1.3
2013-2017 2.3 2.0 10979.7 1.8 (0.3, 5.6) 1.5
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
The p-value test for trend did not detected a significant change in the number of live births with Total Anomalous Pulmonary Venous Return during 2007-2017. See p-value estimate
Estimate Std. Error t value Pr(>|t|)
0.13276 0.07407 1.79236 0.13306

Regional Distribution

Distribution of Total Anomalous Pulmonary Venous Return 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 7 5.9 11881 4.9 (1.9, 9.8)
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 Total Anomalous Pulmonary Venous Return. 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 6 5 59998 0.8 (0.4, 1.9)
  Male 14 11.8 63632 1.9 (1.0, 3.1)
Birth weight (grams)
  <2500 - - 7231 -
  2500+ - - 116215 1.4 (0.8, 2.1)
Maternal age
  12-19 - - 9156 -
  20-24 - - 32872 -
  25-29 6 5 37743 1.3 (0.6, 3.1)
  30-34 - - 28152 -
  35-39 - - 12614 -
  40+ - - 3064 -
Maternal race
  Alaska Native/American Indian 12 10.1 31560 3.2 (1.7, 5.8)
  Asian/Pacific Islander - - 11810 -
  Black - - 5081 -
  White - - 73595 -
Maternal education (years)
  <12 - - 11929 4.2 (1.9, 9.8)
  12 - - 43162 1.8 (0.8, 3.3)
  12+ - - 65105 -
Marital status
  Married 11 9.2 78396 1.2 (0.6, 2.2)
  Unmarried 8 6.7 44625 1.5 (0.6, 2.9)
Maternal smoking use
  Reported smoking - - 16817 -
  Reported not smoking - - 104707 1.3 (0.7, 2.1)
Medicaid (mother or child)
  Medicaid - - 62666 2.0 (1.1, 3.3)
  non-Medicaid - - 60839 -
Father on birth certificate
  None - - 6328 -
  Present - - 117302 1.1 (0.7, 1.9)
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 TAVPR | CDC. (2019). Retrieved 17 March 2021, from https://www.cdc.gov/ncbddd/heartdefects/tapvr.html

[2] Total anomalous pulmonary venous return - Overview - Mayo Clinic. (2021). Retrieved 17 March 2021, from https://www.mayoclinic.org/diseases-conditions/total-anomalous-pulmonary-venous-return/cdc-20385613

[3] Total Anomalous Pulmonary Venous Return (TAPVR) | Boston Children’s Hospital. (2021). Retrieved 17 March 2021, from https://www.childrenshospital.org/conditions-and-treatments/conditions/t/total-anomalous-pulmonary-venous-return?utm_campaign=FY21HeartFetal&utm_medium=cpc&utm_source=google&utm_content=tapvr&gclid=EAIaIQobChMI26LUhZjM7gIVTT6tBh3n1wl_EAAYASAAEgJmxvD_BwE

[4] Total Anomalous Pulmonary Venous Return (TAPVR) | Children’s Hospital of Philadelphia. (2021). Retrieved 17 March 2021, from https://www.chop.edu/conditions-diseases/total-anomalous-pulmonary-venous-return-tapvr?gclid=EAIaIQobChMI28qEgZnM7gIVkgh9Ch2DHwn0EAAYAiAAEgIR9vD_BwE

Authorship

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: Total anomalous pulmonary venous return, Alaska, 2007-2017. Updated June 22, 2021. Available at: http://rpubs.com/AK_ABDR/TAPVR/07_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\TAPVR07_17.Rmd