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Critical congenital heart disease is the target of postnatal, pulse-oximetry screening; nevertheless, prenatal detection is preferable. Prenatal diagnosis allows for advanced perinatal planning, parental counseling, potential fetal cardiac intervention if indicated, and avoidance of complicated, costly, and at times, risky neonatal transport. The purpose of this study was to investigate a possible association between the occurrence of critical congenital heart disease and pregnancies complicated by maternal comorbidities. We identified all those prenatally and postnatally diagnosed with critical congenital heart disease in Nevada between July 2014 and June 2020. For each identified case of critical congenital heart disease, we assessed each mother's medical history. We divided pregnant women into two groups: 1) those with maternal comorbidities and 2) those without comorbidities. We identified 367 cases of critical congenital heart disease. Of the 367, 229 (62%) occurred in those with maternal comorbidities. Of the 367, 321 (87%) were live-born. Of the 321 live-born, 201 (63%) occurred in those with maternal comorbidities, however, the prevalence of maternal comorbidities in the general pregnancy population is not known. The majority of patients born with critical heart disease were born to mothers with comorbidities versus those without comorbidities for both the total and live-born cohorts (p = 0.00001). Of the 321 live-born, 10 had no prenatal care. Of the 311 with prenatal care, 248 (80%) were prenatally diagnosed. During the study period, there were approximately 210,000 live births in Nevada, for a prevalence of 15.2 (321/210,000) critical congenital cardiac malformations per 10,000 live births. In Nevada, most of those with critical congenital heart disease are products of pregnancies complicated by maternal comorbidities. Further, the state -wide, live-born, critical congenital heart disease prenatal-detection rate was 80%. • Prenatal detection of critical congenital heart disease is preferred to postnatal diagnosis. • The majority of patients born with critical heart disease were born to mothers with comorbidities. • In Nevada, critical congenital heart disease prenatal detection rate is 80%. [ABSTRACT FROM AUTHOR]

Controversy exists regarding an association between advanced maternal age and the occurrence of fetal congenital heart disease and whether advanced maternal age is an indication for fetal echocardiography. Our purpose was to undertake a retrospective, multi-year review of critical congenital heart disease in Nevada to investigate a possible association with advanced maternal age. We identified all those prenatally and postnatally diagnosed between September 2012 and August 2020. For each identified case, we tabulated each pregnant woman's age at the estimated date or actual date of delivery. Using Nevada population demographic data, we calculated the percentages of pregnant women ≥ 35 years old (defined as advanced maternal age) and of pregnant women < 35 years old. Subsequently, we calculated occurrence rates for critical congenital cardiac malformations in advanced and non-advanced maternal age populations. A chi-square risk ratio analysis, excluding aneuploidy cases, was used to determine statistical significance for each malformation. We identified 467 total cases. Of the 467, 39 (8.4%) had aneuploidy. Of the 467, 413 (88.4%) were live born, 32 (6.9%) had fetal demise, and 22 (4.7%) had elective termination. There were approximately 280,000 live births during the study period for a prevalence of 14.8 cases (inclusive of both those with and without aneuploidies) per 10,000 live births. From the 428 cases exclusive of aneuploidy, the following malformations were statistically significantly associated with advanced maternal age (risk ratios with 95% CI): 1) coarctation ± ventricular septal defect 1.75 (1.12, 2.73) p = 0.01, 2) D-transposition of the great arteries ± ventricular septal defect 2.13 (1.18, 2.83) p = 0.01, 3) double outlet right ventricle 2.10 (1.01, 4.35) p = 0.04, and 4) tetralogy of Fallot ± pulmonary atresia 2.40 (1.39, 4.31) p = 0.001. Excluding aneuploidy cases, advanced maternal age was statistically significantly associated with some critical congenital cardiac malformations and lacked association with others. • Advanced maternal age is associated with selected critical congenital cardiac malformations • Advanced maternal age as an indication for fetal echocardiography may improve the detection of critical heart diseases • Robust program database management may obviate a reliance on third-party registries [ABSTRACT FROM AUTHOR]