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Objective: We reviewed our experience with transvenous liver biopsy-derived hepatic fibrosis scores and possible associated risk factors in those postextracardiac Fontan patients. Methods: We identified extracardiac-Fontan patients with postoperative durations <20 years who underwent cardiac catheterizations with transvenous hepatic biopsies between April 2012 and July 2022. If a patient underwent two liver biopsies, we averaged the two total fibrosis scores and concurrent time, pressure, and oxygen saturation data. We grouped patients by the following factors: (1) sex , (2) venovenous collaterals, and (3) type of functionally univentricular heart. We identified potential hepatic fibrosis risk factors as the following: female, presence of venovenous collaterals, and a functional univentricle of right-ventricular type. For statistical analysis, we used Kruskal-Wallis nonparametric testing. Results: We identified 127 patients who underwent 165 transvenous biopsies, with 38 patients undergoing 2 biopsies. We found that females with two additional risk factors had the highest median total fibrosis scores, 4 (1-8); males with <2 risk factors had the lowest median total fibrosis scores, 2 (0-5); and females with <2 additional risk factors and males with two risk factors were in the middle, median total fibrosis score 3 (0-6), P =.002; and there were no statistical differences for the other demographic or hemodynamic variables. Conclusions: For extracardiac-Fontan patients with similar demographic and hemodynamic variables, identifiable risk factors are associated with the degree of hepatic fibrosis.

Objective: We investigated the prevalence of isolated vascular rings in the general population of Southern Nevada., Methods: We identified those prenatally and postnatally diagnosed with an isolated vascular ring between January 2014 and December 2021. We included only those with vascular or ligamentous structures completely encircling the trachea and esophagus. To investigate the prevalence of isolated vascular rings, we included only those with situs solitus, levocardia, and no significant intracardiac malformations., Results: We identified 112 patients. Of the 112, 66 (59%) were female. There were approximately 211,000 total live births in Southern Nevada for the study period, for an overall prevalence of 5.3 isolated vascular rings per 10 000 live births. However, for the years 2014 to 2017, the average prevalence figure was 3.5 per 10 000 live births, and for the years 2018 to 2021, the average prevalence figure was 7.1 (range 6.5-8.0) per 10 000 live births. Simultaneously, the prenatal detection rate rose from 66% to 86%., Conclusions: Isolated vascular rings are common cardiovascular malformations. As prenatal detection rates in the Southern Nevada general population approach 90%, the prevalence figures for isolated vascular rings appear to asymptote at about 7 per 10 000 live births.

A 12-year-old with a history of critical pulmonary stenosis and moderate right ventricular hypoplasia underwent neonatal pulmonary valve dilation, an aortopulmonary shunt, and an infant cavopulmonary anastomosis with aortopulmonary shunt takedown. During a diagnostic cardiac catheterization at 12 years of age, angiography showed interruption in the midportion of the coronary sinus, which required no intervention.

Objective: Our objective was to investigate whether a relationship existed between our center's STAT 4 and 5 category surgical mortality and general-population prenatal detection rates in Nevada. Methods: We identified patients who underwent STAT 4 and 5 neonatal index cardiovascular surgeries at our center between October 2012 and September 2021. Additionally, we calculated prenatal detection rates for each of the 9 retrospective study years. We used descriptive statistics and nonparametric testing, including the Spearman Rho correlation ( R ) and the Mann-Whitney U -tests, with a significant P -value set at < .05. Results: We identified 356 patients. We noted a statistically significant increasing trend in prenatal detection percentages (rho = 0.79, P  = .01), concurrent with a statistically significant decreasing trend in surgical mortality (rho = -0.82, P  = .007). Conclusions: Despite encouraging results, we could not establish a cause-and-effect relationship between concurrent decreased surgical mortality and increased prenatal detection rates for patients undergoing STAT 4 and 5 surgical procedures at our center.

Objective: We reviewed our center's prenatal detection and surgical experience with high-risk, 2-ventricle patients, with complex congenital heart disease that underwent stage-1 hybrid palliation., Methods: We retrospectively identified those born between March 2008 and March 2021 with 2-ventricle hearts, complex congenital cardiovascular malformations, and ductal-dependent systemic circulation that underwent stage-1 hybrid palliation consisting of surgical bilateral pulmonary artery banding and interventional catheterization placed ductus arteriosus stents., Results: We identified 30 patients. Of the 30, 19 (63%) were male. For the 30, median gestational age was 35 weeks (29-39 weeks), and median birth weight was 2.2 kg (0.6-4.5 kg). Of the 30, 1 was transferred from an adjacent state, and 29 were born in Nevada. Of the 29 born in Nevada, overall statewide prenatal detection was 18 of 29 (62%); however, for 2008 to 2011 the prenatal detection rate was 3 of 10 (30%) and 15 of 19 (79%) for 2012 to 2021, P  = .03. For the last 5 years, prenatal detection for Nevada-born patients was 8 of 8 (100%). Two full-term newborns, without a prenatal diagnosis, presented postnatally in extremis. For the 30 patients, there were 0 stage-1 hybrid palliation mortalities, 1 subsequent repair mortality, and 3 late nonsurgical deaths., Conclusions: Stage-1 hybrid palliation may result in excellent surgical outcomes for high-risk, 2-ventricle patients. Additionally, high rates of population-wide prenatal detection are possible for high-risk congenital heart disease, allowing prenatal planning and possibly reducing postnatal extremis presentations.

Objective: We hypothesized that a relationship between post-Fontan hepatic fibrosis and anatomical variants might exist., Methods: Attempting to limit confounding variables, we analyzed data from living, stable, post-extracardiac Fontan patients who underwent cardiac catheterization and transvenous hepatic biopsy procedures between March 2012 and June 2020., Results: We identified 120 patients who met the inclusion criteria. Of the 120, 35 (29%) had pulmonary artery stents. For the 35 with pulmonary artery stents, the average total fibrosis score was 3.2 ± 1.9 and the fibrosis progression rate was 0.36 ± 0.33, and for those with no pulmonary artery stents, the total fibrosis score was 2.6 ± 1.8 and the fibrosis progression rate was 0.27 ± 0.33 ( P = .13 and P = .11, respectively). Of the 120, 65 had functional univentricles of right ventricular type. Of these 65, 27 had pulmonary artery stents. For the 27 with pulmonary artery stents, the average total fibrosis score was 3.4 ± 1.8 and the average fibrosis progression rate was 0.39 ± 0.30, and for the 38 without pulmonary artery stents, the average fibrosis score was 2.3 ± 1.5 and the average fibrosis progression rate was 0.23 ± 0.21 ( P = .01 for comparison of both values)., Conclusions: This study's findings suggest that a post-extracardiac Fontan with a functional univentricle of right ventricular type plus a pulmonary artery stent may have more advanced liver pathology than those without a pulmonary artery stent at similar Fontan duration years and ages at liver biopsy.

A 57-year-old man, with a history of coarctation patch repair at three years of age, presented with left ventricular dilatation and moderate to severe dysfunction. A computed tomographic angiogram of the chest revealed moderate residual stenosis of the distal arch and proximal descending aorta and a large aneurysm adjacent to the origin of the left subclavian artery. Due to high surgical risk, a hybrid approach was undertaken with temporary balloon occlusion of the left subclavian artery, followed by surgical left common carotid to left subclavian artery graft and percutaneous covered stent implantation to relieve the obstruction and exclude the aneurysm.

Background: We reviewed data on patients born with critical congenital heart disease in the state of Nevada and analyzed detection via prenatal diagnosis versus newborn pulse oximetry screening, location of birth, and gestational age at birth., Methods: We inquired our databases and electronic health records for all patients with critical congenital heart disease born in Nevada between January 2016 and May 2019., Results: We identified 218 live born patients. Of the 218, average gestational age was 38 weeks (±2.2 weeks). Of the 218, 171 (78%) were prenatally diagnosed, 37 (17%) were diagnosed by immediate postnatal signs and symptoms, 8 (4%) had false-negative pulse oximetry screens that resulted in post-hospital discharge presentations, and 2 (1%) had positive pulse oximetry screens. The eight post-hospital discharge presentations included four in extremis, two with extreme cyanosis, and two dying at home. Of the 171 prenatally diagnosed patients, 157 (92%) were born at the Nevada hospital with the congenital cardiac unit., Conclusion: To the best of our knowledge, our results represent the highest statewide, general population prenatal detection of critical congenital heart disease in the United States. Our high prenatal detection rate led to the majority of patients being born at the Nevada facility with the congenital heart unit, limiting intrastate neonatal transports. On average, patients were born at term. Further, in Nevada, state-mandated, universal pulse oximetry screening resulted in more false-negative results than positive results.

Background: Vascular rings (VRs) are recognized as uncommon but not rare cardiovascular malformations., Methods: We analyzed data from all patients born in Southern Nevada, who underwent diagnosis and management of VR from 1990 to 2015, RESULTS: From 1990 to 2015, a total of 92 patients were diagnosed prenatally and postnatally. Of the 92 patients, 73 (79%) had right aortic arch and aberrant left subclavian artery (RAA-ALS) with a left ductus arteriosus or ligamentum, 17 (19%) had a double aortic arch (DAA), and 2 (2%) had a pulmonary artery sling. Of the 92 patients, 75 had an isolated VR and 17 VR had significant additional congenital heart disease (CHD). Of the 75 patients with an isolated VR, 52 underwent surgical repair, and the most common surgical diagnosis was DAA in 6 (66%) of 9 for the period 1990 to 2005 versus less common in 9 (21%) of 43 during the period 2006 to 2015, P < .05. The isolated VR repair age significantly negatively correlated with increasing time from 1990 to 2015, R = -0.7 (P < .0001). Of the 75 isolated VR, 23 remain asymptomatic. The 17 VR with CHD were treated during infant palliation or intracardiac repair. Of the total 92 VR, 60 were born after a 2004 community introduction of the three-vessel fetal echocardiography view, from then the prenatal-detection rate has significantly increased-2004 to 2006, 0 (0%) of 9; 2007 to 2009, 1 (9%) of 11; 2010 to 2012, 11 (55%) of 20; and 2013 to 1015, 14 (70%) of 20 (P < .0001)., Conclusion: Over 25 years in Southern Nevada, VR prenatal diagnosis has increased, isolated VR age at surgery has decreased, and the percentage of those with RAA-ALS has increased., (© The Author(s) 2016.)

Perinatal management of severe mitral regurgitation (MR) and aortic stenosis (AS) is difficult; mortality is high, and there are few reports of successful postnatal biventricular repairs. We report a patient with severe MR and AS, diagnosed prenatally, that underwent a fetal aortic valvuloplasty and a successful modified Ross-Konno procedure with concomitant mitral valve repair shortly after birth.