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

Antegrade transseptal left ventricular decompression is feasible in patients on extracorporeal membrane oxygenation and persistent pulmonary edema.

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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.

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.