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Objective: We reviewed our center's experience with neonatal and infant hypoplastic aortic arch, unassociated with intracardiac malformations, and investigated changes in prenatal detection rates over time for those requiring therapeutic procedures., Methods: We identified all prenatal diagnoses of hypoplastic aortic arch with situs solitus, unassociated with intracardiac malformations, made in Nevada between May 2017 and April 2022. In addition, we identified all those 0-180 days old, with prenatal care, that underwent a surgical or interventional cardiac catheterization aortic arch procedure, whether prenatally or postnatally diagnosed. We excluded those with ventricular septal defects, functionally univentricular hearts, interrupted aortic arches, or any associated malformation requiring an additional surgical or interventional procedure ≤6 months old. Additionally, we calculated prenatal detection rates for those undergoing a surgical or interventional catheterization procedure for each of the 5 years., Results: We identified 107 patients prenatally and postnatally. Of the 107 patients, 56 (34 prenatally diagnosed and 22 postnatally diagnosed) underwent an aortic arch procedure, and 51 additionally prenatally diagnosed, live-born infants did not undergo a procedure. Of the 56 procedures, 2 were by interventional catheterization, and 54 underwent a surgical repair. Prenatal detection for those undergoing a procedure statistically significantly increased over the 5 years from 38% to 82%, rho = 0.95 (p = .04)., Conclusions: Currently in Nevada, our prenatal detection rate is >80% in the general population for those between 0 and 6 months old who require a therapeutic procedure for aortic arch obstruction without intracardiac malformations., (© 2022 Wiley Periodicals LLC.)

Objective: We investigated possible gender differences for hepatic fibrosis in extracardiac-Fontan patients., Methods: We identified extracardiac Fontan, performed between 2000 and 2016, who underwent cardiac catheterizations with transvenous hepatic biopsies between April 2012 and June 2022. We divided the patients by gender for analysis., Results: We identified 116 patients who underwent 145 transvenous biopsies, with 29 patients undergoing 2 biopsies at an average interval of 5 ± 1 years. We divided the 145 biopsies into two groups: 1) 98/145 (68%) males and 2) 47/145 (32%) females. For the 47 female liver biopsy specimens, the median total fibrosis score was 3 (0-8), and for the 98 male liver biopsy specimens, the median total fibrosis score was 2 (0-6), p = .007. The average age at surgery for females was 3 ± 1 years and for males 3 ± 1 years, p = .99. Average Fontan duration at biopsy for females was 11 ± 5 years and for males, 10 ± 4 years, p = .23. No other demographic, anatomic, echocardiographic, laboratory, or hemodynamic findings demonstrated statistically significant gender differences., Conclusions: Females had statistically significantly higher median total fibrosis scores than males for the similar average age at extracardiac Fontan and average Fontan duration., (© 2022 Wiley Periodicals LLC.)

Objective: We reviewed our center's surgical mortality rates for those who underwent a Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery (STAT) category 4 or 5 neonatal cardiovascular surgery., Methods: We identified all patients who underwent a STAT category 4 or 5 neonatal index cardiovascular surgical procedure between July 2015 and July 2021., Results: We identified 239 patients. We divided them into two groups: (1) 42 (17.6%) ≤2.5 kg, and (2) 197 (82.4%) were >2.5 kg at the time of neonatal surgery. Of those ≤2.5 kg, 18/42 (42.9%) had syndromes or associated noncardiac malformations versus 34/197 (17.3%) of those >2.5 kg, p = .0093. Thirty-day discharge mortality for those ≤2.5 kg was 3/42 (7.1%) versus l0/197 (5.1%) for those >2.5 kg, p = .83., Conclusions: Weight at the time of surgery, presence of syndromes, and associated noncardiac malformations did not affect mortality in those undergoing complex neonatal STAT 4 or 5 category cardiovascular surgery., (© 2022 Wiley Periodicals LLC.)

Objective: We reviewed our center's experience with prenatal detection in Nevada's general population for young infants undergoing cardiovascular surgery., Methods: We identified patients born in Nevada that underwent an initial cardiovascular surgery between 0 and 6 months old with birth dates between August 2012 and July 2021. Additionally, we calculated prenatal congenital cardiovascular malformation detection rates for each of the 9 years., Results: We identified 660 patients. For the 660 patients, 649 (98%) mothers underwent prenatal care, which included at least one anatomical-survey obstetric ultrasound. Of the 649 with prenatal care, 395 (61%) had a prenatal diagnosis overall. However, prenatal diagnosis improved over the 9 years from 44% in 2012 to 79% in 2021 (correlation coefficient of 0.93, p = .00024)., Conclusions: Our results demonstrated a progressive rise in prenatal detection rates for young infants undergoing cardiovascular surgery in Nevada., (© 2021 Wiley Periodicals LLC.)

Objective: We reviewed our center's experience with common arterial trunk., Methods: We included those with common arterial trunk in Nevada with estimated delivery dates or birth dates between June 2006 and May 2021. We excluded patients with functionally univentricular hearts., Results: We identified a total of 39: 32 prenatally and 7 postnatally. Of the 32 prenatally detected, 2 had elective termination, 2 had fetal demise, and 28 were live-born. Of the 7 postnatally diagnosed, 6 had prenatal care without a fetal echocardiogram, and 1 had no prenatal care. Overall, live-born prenatal detection was 28/34 (82%). Prenatal detection for 2006-2009 was 2/6 (33%) and for 2010-2021 was 26/28 (93%) p = .049. Of the 35 live-born infants, 1 died preoperatively, and 34 underwent neonatal surgery. Of the 34, 8 had palliation (birth weight 1.9±0.7 kg, range 0.8-2.6 kg), and 26 had a primary repair (birth weight 3.0±0.3 kg, range 2.6-4.0 kg) p = .0004. For all 34 neonatal surgical procedures, there were 2 (5.9%) deaths; however, there were no subsequent surgical or interventional catheterization mortalities., Conclusions: In Nevada, current state-wide, general population prenatal detection of the common arterial trunk was more than 90%. By employing a combination of neonatal palliation and primary repair, surgical mortality was less than 6% in a cohort that included those with birth weights less than 2.5 kg, truncal valve surgery, and interrupted aortic arches., (© 2021 Wiley Periodicals LLC.)

Objective: We investigated a relationship between a composite index comprised of Fontan-circuit anatomical features and hepatic fibrosis scores from biopsy., Methods: We identified living extracardiac Fontan patients, ≥7 years old and ≥5 but <20 years postoperative, that underwent cardiac catheterization and transvenous liver biopsy between March 2012 and September 2020. We divided patients into anatomical groups and applied a risk score to each patient. We compared average anatomical risk scores with average hepatic total fibrosis scores by group., Results: We identified 111 patients that met inclusion criteria. After excluding four patients, we assigned 107 to one of 12 anatomical variant groups (n ≥ 3). For the 107, the average age at liver biopsy was 14 ± 6 years old. Of the 107, 105 (98%) were New York Heart Association Class 1. We found average anatomical risk scores by group correlated with average total fibrosis scores by group (R = 0.8; p = .005). An average Fontan duration to biopsy of 10 ± 1 years was similar for all 12 anatomical groups. We found no other clinical variables, laboratory, or hemodynamic values that trended with anatomical risk scores or hepatic total fibrosis scores., Conclusions: In a cohort of relatively young, stable extracardiac Fontan patients, average composite anatomical risk scores strongly correlated with average hepatic total fibrosis scores by anatomical group. These findings suggest that some anatomical variants in extracardiac Fontan patients are associated with higher Fontan-associated liver disease (FALD)-related hepatic total fibrosis scores than others, despite similar Fontan durations., (© 2021 Wiley Periodicals LLC.)

Objective: We reviewed our center's isolated vascular ring data., Methods: Inclusion criteria were patients born in Nevada between June 2015 and July 2020 with situs solitus, levocardia, atrioventricular and ventriculoarterial concordance, and no significant intracardiac malformations., Results: We identified 95 patients. Of the 95, 56 (59%) were female (p = .033). For the study period, there were approximately 180,000 live births, for a prevalence of 5.3 isolated vascular rings per 10,000 live births. Of the 95, 78 (82%) were prenatally diagnosed. Of the 95, 63 (66%) were products of high-risk pregnancies (p = .0001). Additionally, we found advanced maternal age was an isolated vascular ring risk factor (relative risk ratio, 2.7; 95% confidence interval, 1.8, 4.1; p < .00001)., Conclusions: Isolated vascular rings are relatively common cardiovascular malformations and more common in females. High prenatal detection rates are achievable. Further, the majority with isolated vascular rings are products of high-risk pregnancies, and advanced maternal age is a statistically significant occurrence risk factor., (© 2021 Wiley Periodicals LLC.)

Objective: We hypothesized that a relationship might exist between angiographically demonstrable, post-Fontan venovenous collaterals, and hepatic fibrosis., Methods: We analyzed data from post-Fontan patients that underwent cardiac catheterization and transvenous-hepatic biopsy procedures between March 2012 and March 2020. From innominate vein angiography, we determined those that either had or lacked venovenous collaterals. Additionally, we examined data from post-Fontan patients that underwent hepatic ultrasound, shear-wave elastography between January 2017 and March 2020., Results: We identified 164 patients that met inclusion criteria. Of the 164, 101 (62%) had venovenous collaterals. Of the 101 with collaterals, average total fibrosis score (TFS) was 3.2 and the average rate of fibrosis progression was 0.28 vs an average TFS of 2.1 and an average fibrosis progression rate of 0.22 for those without collaterals (P = .00001 and P = .01, respectively). Of the 101 with collaterals, oxygen saturation was 91% ± 4% vs 93% ± 3% (P = .048) without collaterals. Of the 164, 86 (52%) underwent ultrasound shear-wave elastography. Of the 86 patients undergoing elastography, 50 (58%) were performed in those with collaterals, and 36 (42%) in those without collaterals. For the 50 with collaterals, average elastography values were 13.3 vs 11.2 kPa for the 36 without collaterals (P = .006). We found no statistically significant differences for age at biopsy, Fontan duration, Fontan-type, type of functional univentricle, laboratory, clinical, or hemodynamic values between those with or without collaterals., Conclusions: The presence of angiographically demonstrated venovenous collaterals was associated with statistically, significantly more advanced liver fibrosis than those without collaterals., (© 2020 Wiley Periodicals LLC.)