Abdominal CT and MRI Findings of Portal Hypertension in Children and Adults with Fontan Circulation

作者：Faizeen Zafar, Adam M. Lubert, Andrew T. Trout, David A. Katz, Joseph J. Palermo, Alexander R. Opotowsky, Angela Lorts, Clifford C. Chin, Adam W. Powell, Chet R. Villa, Nadeem Anwar, David L.S. Morales, Jonathan R. Dillman, Tarek Alsaied · 发表于：Radiology · 年份：2022 · DOI：10.1148/radiol.211037 · 被引用次数：19 · 研究领域：Congenital Heart Disease Studies、Liver Disease and Transplantation、Pulmonary Hypertension Research and Treatments

Background Portal hypertension in the Fontan circulation is a function of elevated systemic venous pressure and liver fibrosis. Purpose To quantify the prevalence of radiologic evidence of portal hypertension and elevated VAST score (one point each for varices, ascites, splenomegaly, and thrombocytopenia) of 2 or greater in children and adults with Fontan circulation and to determine the association with hemodynamics and adverse outcomes. Materials and Methods This was a retrospective study of individuals with Fontan circulation who underwent abdominal MRI or CT for focal liver lesion surveillance between January 2012 and December 2019. Portal hypertension was defined as the presence of at least two of the following: varices, ascites, or splenomegaly. Fontan deterioration was defined as a composite of heart failure signs or symptoms requiring diuretic escalation, placement of a ventricular assist device, heart transplant, or death. Relationships between variables and the composite end point were assessed using univariable and multivariable logistic regression. Results A total of 123 patients (age range, 9–55 years; 32 children) were evaluated (median age, 23 years; IQR, 17–30 years; 63 male patients). Median time since diagnosis of Fontan circulation was 16 years (IQR, 12–23 years). Twenty-five of the 123 patients (20%) had radiologic evidence of portal hypertension, and 34 (28%) had a VAST score of 2 or greater. Fontan deterioration occurred in 25 of the 123 patients (20%); ...