Hepatic Fibrosis Risk Factors in Extracardiac-Fontan Patients: Observations From a Single Center.

We reviewed our experience with transvenous liver biopsy-derived hepatic fibrosis scores and possible associated risk factors in those postextracardiac Fontan patients. 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.

Isolated Vascular Rings Are Common Cardiovascular Malformations.

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.

Prenatal diagnosis of hypoplastic aortic arch without intracardiac malformations: The nevada experience.

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.

Isolated Balanced Complete Atrioventricular Septal Defects: Prenatal Detection and Outcome in Nevada.

We analyzed patients with isolated, balanced complete atrioventricular septal defects. We identified 71 patients born in Nevada, between January 2008 and December 2020. We also analyzed prenatal detection rates.

Complex neonatal congenital heart surgery in Nevada.

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.

Decreasing Mortality for STAT 4 and 5 Neonatal Heart Surgeries Concurrent With Improving Prenatal Detection: The Nevada Experience.

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. 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.

Stage-1 Hybrid Palliation for High-Risk 2-Ventricle Patients with Ductal-Dependent Systemic Circulation in the Era of High Prenatal Detection.

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.

Prenatal diagnosis in Nevada for patients undergoing cardiovascular surgery in the first six months.

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.

Common arterial trunk in the era of high prenatal detection rates: Results of neonatal palliation and primary repair.

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.

Prenatal diagnosis of isolated perimembranous ventricular septal defects undergoing primary surgical repair in infancy.

Objective: We retrospectively analyzed our center's experience with the prenatal diagnosis of isolated perimembranous ventricular septal defects that underwent primary surgical repair in infancy.

Methods: We identified patients born in Southern Nevada, between October 2012 and October 2020, with prenatal care that underwent surgical closure of an isolated large perimembranous ventricular septal defect between 1 and 12 months of age. The description at surgery defined ventricular septal defect morphology.

Fontan-associated liver disease and total cavopulmonary anatomical flow effectors.

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.

Fontan-Associated Anatomical Variants and Hepatic Fibrosis.

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.

Isolated vascular rings in the era of high prenatal detection rates: Demographics, diagnosis, risk factors, and outcome.

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.

Fontan venovenous collaterals and hepatic fibrosis.

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.

Detecting Critical Congenital Heart Disease in Nevada.

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.

Left Subclavian Artery Test Balloon Occlusion Before Covered Stent for Recoarctation and Aneurysm.

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.

Right aortic arch with situs solitus.

Patients: We reviewed all patients evaluated at our center with situs solitus, levocardia, and a right aortic arch that were born between January 2000 and January 2018.

Results: From our databases, we identified 204 patients. We excluded patients with a double aortic arch from analysis.

A composite noninvasive index correlates with liver fibrosis scores in post-Fontan patients: Preliminary findings.

Objective: We hypothesized that clinic-based, hepatic-ultrasound, elastography measurements, either alone or in combination with other noninvasive variables, might correlate with liver-biopsy fibrosis scores in patients post-Fontan.

Methods: Between March 2012 and February 2017, we identified patients post-Fontan that underwent elective cardiac catheterization and simultaneous transvenous hepatic biopsy. From this group, we selected patients that met inclusion criteria for liver-ultrasound, shear-wave elastography.

Right aortic arch with situs solitus frequently heralds a vascular ring.

Objective: We hypothesized that a right aortic arch in situs solitus, with or without an associated cardiovascular malformation, is often associated with a vascular ring.

Methods: From those born in Southern Nevada between March 2012 and March 2017, we identified 50 (3.6 per 10,000 live births) with a right aortic arch and situs solitus.

Vascular Ring Diagnosis and Management: Notable Trends Over 25 Years.

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.

MELD-XI Scores Correlate with Post-Fontan Hepatic Biopsy Fibrosis Scores.

We tested the hypothesis that MELD-XI values correlated with hepatic total fibrosis scores obtained in 70 predominately stable, post-Fontan patients that underwent elective cardiac catheterization. We found a statistically significant correlation between MELD-XI values and total fibrosis scores (p = 0.003).

An Observation from Liver Biopsies Two Decades Post-Fontan.

This brief report describes an observation from liver biopsy results in nonfailing Fontan patients, currently in their second postoperative decade. In three patients, with either atriopulmonary or atrioventricular connections and functional left ventricles, we found no portal fibrosis. In contrast, we found portal fibrosis in three clinically similar, nonfailing Fontan patients with lateral tunnel connections and functional left ventricles.

Hybrid Palliation for Ductal-Dependent Systemic Circulation.

We reviewed our hybrid palliation experience for 91 neonates, with ductal-dependent systemic circulation, born between August 2007 and October 2015. For analysis, we stratified the 91 patients by a risk factor (RF) score and divided them into three groups: (1) high-risk two-functional ventricles (2V) median RF score of 3 (N = 20); (2) low-risk one-functional ventricle (1V) RF score 0-1 (N = 32); and (3) high-risk 1V RF score ≥2 (N = 39). Midterm survival (median 4 years) by group was: (1) 95 %, (2) 91 %, and (3) 15 %, (p = 0.

Fontan Outcomes and Pulmonary Blood Flow at Birth.

We previously noted, in a small group of post-Fontan patients, a possible association between hepatic fibrosis scores and the status of pulmonary blood flow at birth. To further explore this observation, we examined data from all Fontan patients seen in our center from July 2010 to March 2015. We identified 200 patients for analysis.

Moving towards universal prenatal detection of critical congenital heart disease in southern Nevada: a community-wide program.

This study compares the current, prenatal detection rate for critical congenital heart disease in Southern Nevada with the previously reported rate, after developing and expanding a comprehensive, community-wide fetal cardiology program. For the current-period analysis, we inquired our database and electronic health records for patients born in Clark County, Nevada, with critical congenital heart disease between May 2012 and April 2014, and we compared the results with the previous period between May 2003 and April 2006. The major components of the community-wide program include fetal congenital heart disease screening via general obstetric ultrasound studies performed in obstetrician's offices, radiology imaging centers, or maternal-fetal medicine specialty practices; subsequent referral for comprehensive fetal echocardiography performed in maternal-fetal medicine offices under the on-site supervision by fetal cardiologists; and recurring community educational programs teaching the 5-axial plane, fetal echocardiographic screening protocol to general obstetric sonographers and instructing perinatal sonographers in advanced imaging topics.