Approximately 70% of congenital heart disease (CHD) is diagnosed through prenatal screening, allowing for early assessment and intervention. Expert consensus recommends genetic testing for apparently isolated CHD (iCHD), which can provide valuable insights into potential underlying genetic diagnoses. In the prenatal setting, parents may have access to non-invasive screening options as well as diagnostic testing via amniocentesis. While some families may choose to decline amniocentesis, they may be more receptive to postnatal genetic testing as an alternative. Timing of genetic counseling (prenatal vs. postnatal) can affect parental decision-making and genetic testing uptake. The primary aim of this study was to examine postnatal genetic testing uptake among families of infants with iCHD who received prenatal versus postnatal cardiovascular genetic counseling (CVGCing). We hypothesized that families of infants with iCHD who received prenatal CVGCing would have a higher uptake of postnatal genetic testing than those who received postnatal CVGCing. A retrospective chart review was completed for infants with iCHD admitted to the Cincinnati Children's Hospital Medical Center (CCHMC) Cardiac Intensive Care Unit (CICU) between January 1, 2019, and April 30, 2023. A total of 228 infants received CVGCing from a Cardiovascular Genetic Counselor (CVGC). Seventy-six (33%) families received prenatal CVGCing and 152 (67%) were counseled postnatally. Patients receiving prenatal CVGCing were approximately four times more likely to consent for postnatal genetic testing (OR = 3.88, p = 0.03) than those who received postnatal CVGCing. Genetic testing confirmed a genetic diagnosis in 14% of infants. Positive genetic testing results included both copy number variants (70%) and single-nucleotide variants (30%). In conclusion, families of infants with iCHD who received prenatal CVGCing were significantly more likely to consent for postnatal genetic testing. Patients receiving prenatal CVGCing were able to receive results faster, meet parental goals of earlier genetic counseling access, and increase genetic testing uptake. Although expert consensus statements often recommend chromosomal microarray as a first line of genetic testing for iCHD, the yield reported in our study supports consideration of CHD gene sequencing for all infants with iCHD. Preliminary results suggest prenatal CVGCing for iCHD, with consideration of comprehensive genetic testing including copy number variant analysis and gene sequencing, should be considered standard of care.

BACKGROUND:Thrombotic events, including acute ischemic stroke, are more common in individuals with congenital heart disease (CHD). Whether common thrombophilia variants contribute to thrombotic risk in this population remains unclear. We evaluated whether prothrombin G20210A (F2 c.97G>A) and factor V Leiden (F5 c.1601G>A; p.Arg534Gln) are associated with thrombotic events in CHD. METHODS:Participants in the Pediatric Cardiac Genomics Consortium with exome sequencing and electronic medical record data were identified. Individuals were stratified by prothrombin G20210A and factor V Leiden genotypes, ventricular physiology, and antithrombotic therapy. The primary outcome was the presence of International Classification of Diseases (ICD) or Phecodes (phenotype codes) for thrombotic events. RESULTS:Among 4008 participants (median age, 11.4 [interquartile range, 5.1-17.9] years; 44.4% boys), thrombotic events occurred in 737 (18%), including 93 (13%) with acute ischemic stroke. Compared with the Genome Aggregation Database, the CHD cohort had a lower prevalence of heterozygous prothrombin G20210A and factor V Leiden variants. Variant prevalence did not differ between participants with and without thrombotic events. Single-ventricle CHD was associated with higher thrombosis frequency than biventricular CHD (35% versus 16%, P≤0.0001), without differences in variant prevalence. CONCLUSIONS:In this multicenter CHD cohort, prothrombin G20210A and factor V Leiden were not significantly associated with thrombotic outcomes, supporting recommendations against routine screening. Given low variant prevalence, the study was powered to exclude only large associations. Reduced variant frequency suggests survivorship bias beginning in fetal life. Larger integrated clinical-genomic studies are needed to refine thrombotic risk stratification in CHD. REGISTRATION:URL: https://www.clinicaltrials.gov; Unique Identifier: NCT03347214.

Prenatal diagnosis of critical congenital heart disease (cCHD) and subsequent perinatal recommendations are vital components of prenatal care that affect infant morbidity and mortality. However, there is limited understanding of the relationship between fetal cardiology prenatal care quality, infant outcomes, and maternal social determinants of health (SDoH). The aim of this study was to better understand this relationship, and to further characterize the continuum of prenatal to postnatal care in a population of maternal-infant dyads diagnosed with cCHD. This was a single-site, retrospective cohort study of 217 maternal-infant dyads with cCHD cared for between 2021 and 2022. A community deprivation index was used as a marker of maternal SDoH. 198 dyads had a prenatal cCHD diagnosis. Maternal deprivation was similar in those with prenatal versus postnatal diagnosis. The majority were identified on obstetric anatomy ultrasound, with median 15 days (IQR: 7, 28) from concern to fetal echocardiogram. Nearly all had perinatal delivery recommendations, while 73

Improved understanding of late gestation fetal growth patterns and postnatal outcomes in fetuses with single ventricle heart disease may inform decisions about elective delivery timing. This was a retrospective cohort analysis of patients in the National Pediatric Cardiology Quality Improvement Collaborative database from 2016 to 2019 anticipated to need stage 1 palliation (S1P) and had a gestational age (GA) of ≥ 36 weeks. Birthweight (BW) and BW z-scores were compared across GA. Survival to stage 2 palliation (S2P), post-S1P length of stay, and duration of mechanical ventilation were compared by GA. In 1290 infants, the mean BW increased with increasing GA (p < 0.001). Mean BW z-scores were below zero in late gestation (p < 0.001), with declining growth velocity 38 to > 40 weeks GA. Of 1216 patients with mechanical ventilation data, those at later GA had shorter durations of ventilation (p < 0.001). Among 1083 survivors to discharge or S2P if not discharged, infants born at later GA had shorter S1P hospitalizations (p = 0.015) and were more likely to undergo S2P (p = 0.003). Infants with single ventricle heart disease requiring S1P have decreased late gestation growth velocity, but growth continues. Infants born after longer gestation have shorter duration of ventilation and greater likelihood of undergoing S2P.

BACKGROUND:Multicenter collaborative studies examining disparities in in-hospital outcomes are lacking in pediatric cardiology. We hypothesized that Black and Hispanic patients in pediatric acute care cardiology units (ACCUs) have increased length of stay (LOS) and complications compared with their counterparts. METHODS:Utilizing the Pediatric Acute Care Cardiology Collaborative registry, we examined ACCU hospitalizations from January 2, 2019, to July 30, 2021. Hospitalizations were categorized by race and ethnicity and subcategorized by region. Differences in LOS and mean total number of complications were assessed using Wilcoxon rank sum and Kruskal-Wallis testing. Generalized linear models were constructed to evaluate differences between regions, race, and ethnicity. Multiple testing correction was performed which established a significance threshold of .001. RESULTS:Analysis included 29 079 hospitalizations from 28 centers. Patients identifying as non-Hispanic (NH) Black (median, 5.0 [IQR, 3.0-13.0]) and NH other (6.0 [3.0-15.0]) had longer LOS than NH white patients (5.0 [IQR, 2.0-11.0]; P < .0001). Hispanic patients had a shorter LOS than NH patients (median, 5.0 [IQR, 2.0-11.0] vs 6.0 [3.0-14.0]; P < .0001). The number of complications was significantly higher for patients who identify as NH Black or NH other compared with NH white patients. The gaps between LOS and complications persisted in US regions with some areas demonstrating greater difference: LOS difference for NH Black patients was more pronounced in the West than in the Northeast (median, 5.5 [IQR, 3.0-15.0] vs 5.0 [2.0-12.0]). For Hispanic patients in the South, LOS and complications were lower compared with those in the West and the Northeast. CONCLUSION:Significant racial and ethnic disparities exist in hospital LOS and complications in pediatric cardiology, with some regions of the United States demonstrating greater disparity than others.

Special delivery units (SDU) within freestanding pediatric hospitals eliminate transport-associated risks and enable timely subspecialized care for infants with critical congenital heart defects (CCHD). However, outcome data is limited. We conducted a single-center retrospective cohort study of mother-infant dyads with prenatally diagnosed CCHD delivered between 2019 and 2025. We compared outcomes between delivery sites (SDU vs. level III hospitals) and hypothesized that SDU delivery would be associated with improved maternal and infant outcomes. We also assessed how social drivers of health (deprivation index, insurance, race/ethnicity) influenced SDU eligibility. Among 384 dyads evaluated, 91

Handmade trileaflet valves using expanded polytetrafluorethylene (ePTFE) have been considered a viable option for pulmonary valve replacement. Studies have performed ex vivo testing of adult sized valves, data on pediatric-sized ePTFE valves is limited. Our aim was to evaluate pediatric-sized ePTFE valves and ovine tissue valves as comparison to assess performance. Trileaflet valves were constructed using 0.1 mm ePTFE Gore-Tex membrane ranging from 14 to 20 mm in size and sutured into Hemashield conduits. Ovine pulmonary valves were harvested and ranged from 14 to 20 mm. Valves were tested under pediatric pulmonary conditions using a hydrodynamic tester. Regurgitation fraction (RF%), closing volume (CV%), closing volume duration (CVd, second), effective orifice area (EOA, cm(2)), and peak pressure (PMax, mm Hg), and mean pressure (PMean, mm Hg) were measured. All valves reached goal cardiac output with mild regurgitation. Ovine pulmonary valves had significantly lower RF% than ePTFE valves (14 mm: 4.1 vs. 9.4%; 16 mm: 5.6 vs. 13.8%; 18 mm: 3.5 vs. 9.8%; 20 mm: 5.4% vs. 14.4%; p < 0.05, Table 3). Peak pressure was not significantly different between ovine versus ePTFE groups (14 mm: 37 vs. 24 mm Hg; 16 mm: 27 vs. 27 mm Hg; 18 mm: 26 vs. 27 mm Hg; 20 mm: 13 vs. 12 mm Hg). Expanded polytetrafluorethylene valves offer a viable option for pulmonary valve replacement given mild RF%, and comparable PMax. Further work is needed to understand the interplay between leaflet design and function.

Living allogenic valve transplantation (LAVT) refers to transplantation of viable human heart valves in an orthotopic or heterotopic fashion and has recently garnered significant interest for children in need of a living, growing, regenerating valve replacement option. However, at present, there is no standardized approach for establishing and implementing such a program. We provide a practical, step-by-step blueprint of the operational, administrative and regulatory requirements needed to establish an LAVT program based on our center's experience.

Variants with large effect contribute to congenital heart disease (CHD). To date, recessive genotypes (RGs) have commonly been implicated through anecdotal ascertainment of consanguineous families and candidate gene-based analysis; the recessive contribution to the broad range of CHD phenotypes has been limited. We analyzed whole exome sequences of 5,424 CHD probands. Rare damaging RGs were estimated to contribute to at least 2.2% of CHD, with greater enrichment among laterality phenotypes (5.4%) versus other subsets (1.4%). Among 108 curated human recessive CHD genes, there were 66 RGs, with 54 in 11 genes with >1 RG, 12 genes with 1 RG, and 85 genes with zero. RGs were more prevalent among offspring of consanguineous union (4.7%, 32/675) than among nonconsanguineous probands (0.7%, 34/4749). Founder variants in GDF1 and PLD1 accounted for 74% of the contribution of RGs among 410 Ashkenazi Jewish probands. We identified genome-wide significant enrichment of RGs in C1orf127, encoding a likely secreted protein expressed in embryonic mouse notochord and associated with laterality defects. Single-cell transcriptomes from gastrulation-stage mouse embryos revealed enrichment of RGs in genes highly expressed in the cardiomyocyte lineage, including contractility-related genes MYH6, UNC45B, MYO18B, and MYBPC3 in probands with left-sided CHD, consistent with abnormal contractile function contributing to these malformations. Genes with significant RG burden account for 1.3% of probands, more than half the inferred total. These results reveal the recessive contribution to CHD, and indicate that many genes remain to be discovered, with each likely accounting for a very small fraction of the total.

We examined the current practice, factors associated with delay, and impact of timing of arterial switch operation (ASO) on outcomes of d-transposition of great arteries (d-TGA) with intact ventricular septum (IVS). Neonates with d-TGA + IVS undergoing ASO were identified from the Pediatric Health Informational System database. Factors associated with delayed repair, practice trends, and outcomes related to surgical timing were analyzed. A cut-point analysis was performed to determine if an age threshold predicted in-hospital death or post-op ECMO. A total of 3523 patients were included. The distribution by age was: 0–3 d: 24

Individuals with congenital heart disease (CHD) have an increased risk of neurodevelopmental impairments. Given the hypothesized complexity linking genomics, atypical brain structure, cardiac diagnoses and their management, and neurodevelopmental outcomes, unsupervised methods may provide unique insight into neurodevelopmental variability in CHD. Using data from the Pediatric Cardiac Genomics Consortium Brain and Genes study, we identified data-driven subgroups of individuals with CHD from measures of brain structure. Using structural magnetic resonance imaging (MRI; N = 93; cortical thickness, cortical volume, and subcortical volume), we identified subgroups that differed primarily on cardiac anatomic lesion and language ability. In contrast, using diffusion MRI (N = 88; white matter connectivity strength), we identified subgroups that were characterized by differences in associations with rare genetic variants and visual-motor function. This work provides insight into the differential impacts of cardiac lesions and genomic variation on brain growth and architecture in patients with CHD, with potentially distinct effects on neurodevelopmental outcomes.

BackgroundUtilization of Fontan fenestration varies considerably by center.ObjectivesUsing a multicenter Pediatric Heart Network dataset linking surgical and preoperative hemodynamic variables, the authors evaluated factors associated with use of Fontan fenestration and the impact of fenestration on post-Fontan length of stay (LOS).MethodsPatients 2 to 6 years old at Fontan surgery from 2010 to 2020 with catheterization <1 year prior were included. Factors associated with fenestration were evaluated using multivariable logistic regression adjusting for key covariates. Restrictive cubic spline analysis was used to evaluate potential cut-points for hemodynamic variables associated with longer postoperative LOS stratified by fenestration with multivariable linear regression to evaluate the magnitude of effect.ResultsFenestration was used in 465 of 702 patients (66.2%). Placement of a fenestration was associated with center (range 27%-93% use, P < 0.0001) and Fontan type (OR: 14.1 for lateral tunnel vs extracardiac conduit, P < 0.0001). No hemodynamic variable was independently associated with fenestration. In a multivariable linear model adjusting for center, a center-fenestration interaction, prematurity, preoperative mean pulmonary artery pressure (mPAP), and cardiac index, fenestration was associated with shorter hospital LOS after Fontan (P = 0.0024). The benefit was most pronounced at mPAP ≥13 mm Hg (median LOS: 9 vs 12 days, P = 0.001).ConclusionsThere is wide center variability in use of Fontan fenestration that is not explained by preoperative hemodynamics. Fenestration is independently associated with shorter LOS, and those with mPAP ≥13 mm Hg at pre-Fontan catheterization benefit the most. We propose this threshold as minimal criteria for fenestration.

Background: Fetal cardiomyopathy (FCM) affects 8 per 100,000 live births, often with unknown etiology. Progress in fetal cardiac screening, genetic testing and management may have impacted historically poor outcomes. Aims: We sought to investigate genetic associations and clinical outcomes of FCM in the current era. Methods: This was a retrospective Fetal Heart Society Research Collaborative cohort study of FCM cases diagnosed from January 2017-December 2021 in 39 centers. Cases of fetal myocardial disease attributable to maternal diabetes, structural heart disease, arrhythmia or extra-cardiac conditions were excluded. Data including outcomes to 1 year after birth were collected. Significance of genetic testing results and FCM subtype were classified by each center. Results: There were 138 FCM cases diagnosed at a median gestational age of 27 +4 weeks (range 13-39 weeks). Suspected fetal heart disease (41%, 56/138) was the most common referral indication. There was a known family history of CM in 19% (26/138). FCM phenotypes included 38% (52/138) dilated, 24% (33/138) hypertrophic, 17% (24/138) non-compacted, 10% (14/138) mixed, 5% (7/138) restrictive, 3% (4/138) ventricular aneurysm, 3% (4/138) unspecified. Hydrops was present at diagnosis in 15% (20/138) and by delivery or at fetal demise in 26% (36/138). Of 128 continued pregnancies, 7% (9/128) had a fetal demise, 92% (118/128) a live birth and one was lost to follow-up. Among live births, 10% (12/118) received only palliative care. Of 106 actively treated, 75% (80/106) survived to 1 year; 7% (7/106) required ECMO, and 22% (23/106) were listed for transplant with 18 of these successfully transplanted. Of the cohort, a total of 80% (110/138) had genetic testing (including 30 with invasive prenatal testing), with 38% (52/138) having a confirmed genetic etiology and 28% (38/138) with a variant of uncertain significance (VUS) (Figure 1). Transplant-free survival to 1 year was similar for those with and without a genetic diagnosis (40%,21/52 vs 47%,40/86, p=0.48). Conclusion: Outcomes remain poor in FCM, with <50% 1-year survival overall. Confirmed genetic etiologies are now identified in nearly 40%, highlighting the importance of genetic testing.

Background We sought to measure frequency of achieving an optimal outcome after stage 1 palliation (S1P) for hypoplastic left heart syndrome and variants, determine factors associated with optimal outcomes, and compare outcomes after stage 2 palliation (S2P) using the National Pediatric Cardiology Quality Improvement Collaborative database (2008–2016). Methods and Results This is a retrospective cohort study with optimal outcome defined a priori as meeting all of the following: (1) discharge after S1P in <19 days (top quartile), (2) no red flag or major event readmissions before S2P, and (3) performing S2P between 90 and 240 days of age. Optimal outcome was achieved in 256 of 2182 patients (11.7%). Frequency varied among centers from 0% to 25%. Factors independently associated with an optimal outcome after S1P were higher gestational age (odds ratio [OR], 1.1 per week [95% CI, 1.0–1.2]; P =0.02); absence of a genetic syndrome (OR, 2.5 [95% CI, 1.2–5]; P =0.02); not requiring a post‐S1P catheterization (OR, 2.7 [95% CI, 1.5–4.8]; P =0.01), intervention (OR, 1.5 [95% CI, 1.1–2]; P =0.006), or a procedure (OR, 4.5 [95% CI, 2.8–7.1]; P <0.001) before discharge; and not having a post‐S1P complication (OR, 2.7 [95% CI, 1.9–3.7]; P <0.001). Those with an optimal outcome after S1P had improved S2P outcomes including shorter length of stay, less ventilator days, shorter bypass time, and fewer postoperative complications. Conclusions Identifying patients at lowest risk for poor outcomes during the home interstage period could shift necessary resources to those at higher risk, alter S2P postoperative expectations, and improve quality of life for families at lower risk.

Clustering of cardiovascular disease (CVD) risk factors is associated with greater CVD incidence. In pregnant women, such risk factors are associated with congenital heart disease in the infant. This study aimed to determine the association of maternal clustered CVD risk with infant critical congenital heart disease (CCHD) and associated severe maternal morbidity (SMM). This retrospective cohort study used Centers for Disease Control birth certificate data for all live births from 2014-2018. Maternal CVD risks factors (yes/no) included pregestational diabetes and hypertension, pre-pregnancy smoking status, and history of preterm delivery. SMM was defined as maternal intensive care unit admission, ruptured uterus, unplanned hysterectomy, or maternal transfusion. Cases were grouped as having 0, 1, 2, or 3 maternal CVD risk factors. Incidence and relative risk (RR) of infant CCHD and SMM were calculated for each cluster group and compared to a group with no risk factors. The cohort included 11,638 infants with CCHD (0.06%) and 19,329,552 without. CCHD incidence increased with a greater number of maternal CVD risk factors (Figure). When compared to the group with no risk factors, the RR for infant CCHD in groups with 1, 2, or 3 risk factors were RR 1.63 (1.56-1.71), RR 3.37 (3.04-3.75), and RR 9.22 (7.15-11.91) respectively. SMM was more common in mothers with infant CCHD (1.30% vs 0.51%, p< 0.001) and incidence increased with additional maternal risk factors (Figure). In mothers with infant CCHD, the RR for SMM in groups with 1, 2, or 3 risk factors were RR 1.52 (1.05-2.20), RR 2.54 (1.33-4.82), and RR 7.74 (3.27-18.31) respectively when compared to the group with no risk factors. CVD risk clustering in mothers of CCHD infants suggests that biological mechanisms underlying CVD may also disturb fetal cardiac development and contribute to poor maternal outcomes. These findings could inform CHD screening and delivery planning. Prevention strategies targeting these risks may benefit both mother and child.

Background: With significant advancements in fetal cardiac imaging, patients with complex congenital heart disease (CHD) carrying a high risk for postnatal demise are now being diagnosed earlier. We sought to assess an interdisciplinary strategy for delivering these children in an operating room (OR) adjacent to a cardiac OR for immediate surgery or stabilization. Methods: All children prenatally diagnosed with CHD at risk for immediate postnatal hemodynamic instability and cardiogenic shock who were delivered in the operating room (OR) between 2012 and 2023 in which the senior author was consulted were included. Results: Eight patients were identified. Six (75%) patients were operated on day-of-life zero, all requiring obstructed total anomalous pulmonary venous return (TAPVR) repair. Of these six patients, 2 (33%) required a simultaneous Norwood procedure, 2 (33%) required pulmonary artery unifocalization and modified Blalock-Taussig-Thomas shunt, and 2 (33%) patients had repair of obstructed mixed TAPVR. The remaining 2 patients potentially planned for immediate surgery had nonimmune hydrops fetalis and went into cardiogenic shock at 12 and 72 hours postnatally, requiring a novel Norwood procedure with left-ventricular exclusion for severe aortic/mitral valve insufficiency. The median ventilation and inpatient durations were 19 [IQR: 11-26] days and 41 [IQR: 32-128] days, respectively. Three(38%) patients required one or more in-hospital reoperations. Subsequent staged procedures included Glenn (n = 5), Fontan (n = 3), biventricular repair (n = 2), ventricular assist device placement (n = 1), and heart transplant (n = 1). Median follow-up was 5.7 [IQR:1.3-7.8] years. The five-year postoperative survival was 88% (n = 7/8). Conclusion: While children with these diagnoses have historically had poor survival, the strategy of birth in the OR adjacent to a cardiac OR where emergent surgery is planned is a potentially promising strategy with excellent clinical outcomes. However, this is a high-resource strategy whose feasibility in any program requires thoughtful assessment.

BACKGROUND Morbidity and mortality after the Norwood procedure remains high. Shunt size selection is not standardized and the impact of shunt size on outcomes is poorly understood. The Single Ventricle Reconstruction trial randomized infants to modified Blalock-Taussig-Thomas shunt (MBTTS) or right ventricle-to-pulmonary artery shunt at the Norwood procedure. We assessed shunt size distribution and its association with postoperative outcomes. METHODS We included 544 patients, excluding 5 with ambiguous shunt crossover data. Normalized shunt diameter 1 and 2 were calculated as shunt diameter divided by patient's weight and body surface area, respectively. The primary outcome was 30-day mortality after Norwood. Secondary outcomes were intensive care and total length of stay, and survival to Glenn procedure. Logistic and ordinal regression models evaluated the association of normalized shunt diameter with outcomes. RESULTS Thirty-day mortality after Norwood was 11.4% (n = 62), survival to Glenn procedure was 72.6% (n = 395), median length of stay was 14.0 (interquartile range, 9.0-27.7) days and 24.0 (interquartile range, 16.0-41.0) days in the intensive care and total, respectively. Normalized shunt diameters exhibited variation in both shunt types but were not associated with 30-day mortality. Right ventricle-to-pulmonary artery shunt size was not associated with secondary outcomes. However, a MBTTS diameter >= 1.5 mm/kg predicted longer Norwood (odds ratio, 4.89; 95% CI, 1.41-16.90) and intensive care (odds ratio, 4.11; 95% CI, 1.25-13.49]) duration. CONCLUSIONS Shunt size selection was variable. Right ventricle-to-pulmonary artery shunt had a wider size range seen with favorable outcomes compared with MBTTS. A MBTTS either too large or too small is associated with worse postoperative outcomes. Refining shunt sizing practices can improve surgical outcomes after the Norwood procedure.

BackgroundPregnancies with prenatally diagnosed congenital heart disease (CHD) have increased cesarean delivery (CD) rates, with no outcome improvement.ObjectiveWe aim to examine indications for delivery, indications for CD and risk factors associated with CD.Study designRetrospective cohort of 322 singleton pregnancies prenatally diagnosed with moderate to severe CHD. We compared maternal and fetal factors correlated with delivery route.ResultsCD rate was 46% (95% CI 40, 51%). Of all CD, 31.3% (95% CI 23.8, 38.7) were secondary to urgent fetal indications. However, 79.7% of inductions resulted in vaginal delivery (VD). Factors associated with CD include morbid obesity (RR 3.0, 95% CI 1.5, 6.1), diabetes (RR 3.9, 95% CI 2.0, 7.3) and severe pre-eclampsia (6.0, 95% CI 1.7, 21.4). Of the 10 most frequent CHD diagnoses, only hypoplastic-left-heart was associated with CD (OR 1.9, 95% CI 1.02, 3.4).ConclusionsAlthough the CD rate is higher in fetal CHD, most indications for CD are maternal.

Right ventricular outflow dysfunction, manifesting as stenosis, regurgitation, or both, is nearly universal in patients with repaired tetralogy of Fallot, precipitating a complex pathophysiological cascade that leads to increasing rates of morbidity and mortality with advancing age. As the number of adolescent and adult patients with repaired tetralogy of Fallot continues to grow as a result of excellent survival during infancy, the need to improve late outcomes has become an urgent priority. This American Heart Association scientific statement provides an update on the current state of knowledge of the pathophysiology, methods of surveillance, risk stratification, and latest available therapies, including transcatheter and surgical pulmonary valve replacement strategies, as well as management of life-threatening arrhythmias. It reviews emerging evidence on the roles of comorbidities and patient-reported outcomes and their impact on quality of life. In addition, this scientific statement explores contemporary evidence for clinical choices such as transcatheter or surgical pulmonary valve replacement, discusses criteria and options for intervention for failing implanted bioprosthetic pulmonary valves, and considers a new approach to determining optimal timing and indications for pulmonary valve replacement.