Michael V. Di Maria1, Lindsey Silverman2, Adel K. Younoszai1, Mariana L. Meyers3, Kelley E. Capocelli4, Michael R. Narkewicz5
Congenital Heart Disease, Vol.13, No.3, pp. 444-449, 2018, DOI:10.1111/chd.12594
Abstract Background: The current management paradigm for children with single ventricle heart disease
involves a series of palliative surgeries, culminating in the Fontan operation. This physiology results
in a high central venous pressure, and in the setting of single ventricle heart disease, results in
hepatic injury and fibrosis over time. Fontan-associated liver disease (FALD) is universally present
in this cohort, and the current gold standard for diagnosis remains biopsy. Noninvasive assessments of liver fibrosis, such as ultrasound with elastography or acoustic radiation force impulse
(ARFI), has been utilized in this cohort. The effect of poor cardiovascular efficiency, as measured
by cardiopulmonary… More >
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