A neonatal case of vascular ring with Alagille syndrome.

A female infant, born at 37 week 5 days to a mother via induced vaginal delivery for preeclampsia, was prenatally diagnosed with a right aortic arch with vascular ring. On the third day of life, the infant exhibited a bronze-gray coloration, and a direct bilirubin of 1.7 mg/dL was detected.

Selective Use of Pulmonary Vasodilators in Patients with Fontan Physiology.

Background: Fontan-associated liver disease is a well-known sequela following the Fontan procedure for patients living with single-ventricle heart disease. Pulmonary vasodilators, such as phosphodiesterase type 5 inhibitors, have emerged as a potential therapeutic option for lowering central venous pressures by reducing pulmonary vascular resistance.

Method: We performed a single-center retrospective review of Fontan patients who were placed on pulmonary vasodilator therapy with prehemodynamic and posthemodynamic, MR elastography, and histologic assessments.

Alcohol use is prevalent among adults with the fontan circulation but does not correlate with liver disease.

Background: Alcohol consumption is associated with an increased risk of liver disease. There are limited studies on the epidemiology of alcohol use and its effects on Fontan-associated liver disease (FALD) in adulthood.

Methods: In this single-center prospective cohort study, patients were enrolled from the Fontan clinic between November 2019 and November 2020, excluding those with chronic hepatitis C or B.

Transjugular liver biopsy for Fontan associated liver disease surveillance: Technique, outcomes and hemodynamic correlation.

Background: Fontan associated liver disease (FALD) is attributed to the limitations of the Fontan circulation, resulting in congestive hepatopathy. The technique and outcomes of transjugular liver biopsies (TJLB) in Fontan patients warrant definition as part of a rigorous FALD surveillance program.

Method: Four year review of patients with Fontan physiology who underwent a TJLB during surveillance catheterizations.

Exercise MRI highlights heterogeneity in cardiovascular mechanics among patients with Fontan circulation: proposed protocol for routine evaluation.

Single ventricle physiology and palliation via the Fontan operation lead to a series of cardiovascular changes. In addition, organs such as the kidneys and liver have been shown to experience insults and subsequent injury. This has led to routine surveillance of patients.

Evaluation of Fontan liver disease: Correlation of transjugular liver biopsy with magnetic resonance and hemodynamics.

Introduction: Liver fibrosis and cirrhosis are late complications in Fontan palliation. Liver biopsy is the gold standard. The goal of this study is to correlate transjugular liver biopsy (TJLB) in the setting of Fontan palliation with noninvasive testing and hemodynamics.

Tissue plasminogen activator for neonatal coronary thrombosis presenting with mitral valve regurgitation and impaired ventricular function.

Objective: Neonatal coronary thrombosis is a rarely reported disorder, with variable outcomes described. This study assessed the feasibility and safety of an institutional protocol using tissue plasminogen activator (tPA) administration for the treatment of neonatal coronary artery thrombi.

Methods: They reviewed the outcome of three neonates with clinical evidence of myocardial infarction secondary to coronary thrombosis.