It has been 50 years since Francis Fontan pioneered the operation that today bears his
name. Initially designed for patients with tricuspid atresia, this procedure is now offered for a …

Pulmonary arterial hypertension (PAH) is commonly associated with congenital heart
disease (CHD) and relates to type of the underlying cardiac defects and repair history. Large …

As a result of improvements in care for patients with congenital heart disease (CHD),> 90%
of children born with CHD are expected to survive to adulthood. For those adults, heart …

The Fontan circulation describes the circulatory state resulting from an operation in
congenital heart disease where systemic venous return is directed to the lungs without an …

Abstract In the current era, 5%-10% of Fontan patients die or need a transplant in childhood,
and approximately 50% will experience the same fate by age 40 years. Heart transplant …

Abstract Purpose of the Review This is a comprehensive update on failing Fontan
physiology and the role of heart and combined heart and liver transplantation in the current …

The Fontan operation is a palliative procedure that leads to increased survival of patients
with a functional single ventricle (SV). Starting from 1967 when the first operation was …

There is mounting recognition that some of the most urgent problems of adult congenital
heart disease (ACHD) are the prevention, diagnosis, and management of heart failure (HF) …

With improvement in survival after Fontan surgery resulting in an increasing number of older
survivors, there are more patients with a Fontan circulation experiencing circulatory failure …

Objectives Approximately 5%–10% of adults with congenital heart disease (CHD) develop
pulmonary arterial hypertension (PAH), which affects life expectancy and quality of life …