Variation in Prenatal Diagnosis of Congenital Heart Disease in Infants

We appreciate the comments from Dr. Yeager and Jones and thank them for sharing results from their internal data review. There are several potential explanations for the difference noted when comparing their data to the prenatal detection rates (PDRs) from the STS-CHS database (STS- CHSD). Vermont is a unique state in our report since it does not have a congenital surgical center. Our data reports PDRs for patients that underwent surgery at STS participating surgical institutions with information submitted to the database by the surgical center. It is possible some patients were not included in our analysis if the surgical institution did not enter data completely for these patients (i.e. cases meeting our exclusion criteria). Another possibility is that some patients in our study from Vermont who received surgery in the first 6 months of life were not evaluated by the University of Vermont program and included in their internal echocardiography database review. While we don't question the accuracy of the Vermont group's internal database, we utilized a database of patients that underwent surgery at STS centers, and this could lead to assessments of different study populations in the case of Vermont. Additionally, as stated in our study limitations our PDRs likely underestimate overall rates as we only included patients that received surgery. We were not able to include patients with fetal demise, terminations and those that underwent catheter based procedures. We do not know the clinical outcomes of all 115 patients in the Vermont review or whether they underwent surgery in the first 6 months of life and would be eligible for review in our analysis. When comparing relatively low numbers (115 patients over 6 years) this could also play an important role in the noted differences.

One of the methods used to ensure data quality during our STS data review process was to compare our PDRs to a variety of individual states including CA, UT, OH, NV and GA that had recently published PDRs and our findings were very similar (1-6). Additionally, upon review of particularly lesion specific PDRs from different states we found good correlation with our STS data. Therefore, we do not believe any differences with the state of Vermont should be generalized to the rest of the data from the other 46 states included in our analysis.

We thank the University of Vermont group for sharing their PDRs and highlighting impressive rates of diagnosis for lesion types such as HLHS (10 of 11). We acknowledge differences between our Vermont statewide data and the specific University of Vermont data likely related to their unique surgical referral system and our use of a surgical database. Our data was not intended to be center specific for reasons discussed above. We believe our overall conclusion in the manuscript stands in that the majority of patients with CHD that undergo surgery at STS centers in the first 6 months of life do not have a prenatal diagnosis and therefore opportunities exists to improve prenatal screening in the United States.

Sincerely,

Michael D. Quartermain, MD. Department of Pediatrics, Wake Forest University School of Medicine

Kevin D. Hill, MD. Department of Pediatrics, Duke University School of Medicine

Ross M. Ungerleider, MD. Department of Cardiovascular and Thoracic Surgery, Wake Forest University School of Medicine

References: 1. Friedberg MK, Silverman NH, Moon-Grady AJ, et al. Prenatal detection of congenital heart disease. J Pediatr 2009; 155:26-31. 2. Sklansky MS, Berman DP, Pruetz JD, Chang RK. Prenatal screening for major congenital heart disease: superiority of outflow tracts over the 4- chamber view. J Ultrasound Med 2009; 28:889-899 3. Pinto NM, Keenan HT, Minich LL, Puchalski MD, Heywood M, Botto LD. Barriers to prenatal detection of congenital heart. Ultrasound Obstet Gynecol 2012; 40:418-425. 4. Sekar P, Heydarian HC, Cnota JF, Hornberger LK, Michelfelder EC. Diagnosis of congenital heart disease in an era of universal prenatal ultrasound screening in southwest Ohio.Cardiol Young 2013;10:1-7 5. Acherman RJ, Evans WN, Luna CF, et al. Prenatal detection of congenital heart disease in southern Nevada: the need for universal fetal cardiac evaluation. J Ultrasound Med 2007; 26: 1715-1719. 6. Oster ME, Kim C, Kusano AS, et al. A population-based study of the association of prenatal diagnosis with survival rate for infants with congenital heart defects. Am J Cardiol 2014; 113:1036-1040.

Conflict of Interest:

None declared