Hepatic and Renal Consequences of Single-Ventricle Physiology Palliated With the Fontan Operation.

Over time, long-term survival has dramatically increased for patients with complex congenital heart disease who undergo the Fontan operation. With this increased survival, it has become apparent that such a circulation has important consequences for other organ systems, particularly the liver and kidney. The adverse milieu created by chronic venous hypertension, low cardiac output, and an inflammatory state contribute to the pathologic changes observed in the liver and kidneys over the long term in Fontan patients.

Does community size or commute time affect severity of illness at diagnosis or quality of care in a centralized care model of pulmonary hypertension?

Background: Centralized care models are often used for rare diseases like pulmonary hypertension (PH). It is unknown how living in a rural or remote area influences outcomes.

Methods: We identified all patients from our PH database who carried a diagnosis of WHO Group 1 or WHO Group 4 PH.