The Fontan operation has been the final palliation for patients born with congenital heart
defects with a functional single ventricle for more than 4 decades. The “normal” Fontan …

sary to understand the underlying molecular and cellular mechanisms of determination of
the left-right body axis during early embryogenesis. 8–12 This process initiates in the …

2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A
Report of the American College of Cardiology/American Heart Association Task Force on …

Background: Patients with protein-losing enteropathy (PLE) following the Fontan operation
have a reported 50% mortality at 5 years after diagnosis. Objectives: The aim of this study …

A normal mammalian cardiovascular system consists of a double circuit, pulmonary and
systemic, connected in series and powered by a double pump. In the absence of congenital …

Fontan failure has been variably and inconsistently described in the literature, leading to
challenges in comparing studies and evaluating treatments. Development of a conceptual …

The Fontan operation, although part of a life-saving surgical strategy, manifests a variety of
end-organ complications and unique morbidities that are being recognised with increasing …

Fontan circulation is generally characterized by high central venous pressure, low cardiac
output, and slightly low arterial oxygen saturation, and it is quite different from normal …

OBJECTIVE: We characterized the histologic liver changes that occur after the Fontan
operation and identified the factors associated with worsening hepatic disease. METHODS …

Individuals with Fontan circulation are at risk of late mortality from both cardiac and
noncardiac causes. Despite the known risk of mortality, referral indications for advanced …