Single ventricle anatomy is associated with increased frequency of nonalcoholic cirrhosis.
BACKGROUND
Single ventricle (SV) patients with Fontan physiology have multiple risk factors for liver disease but the prevalence of liver disease remains unknown in this population. We sought to determine whether hospitalized patients with a SV diagnosis have higher rates of nonalcoholic cirrhosis than patients without congenital heart disease.


METHODS
We used the 1998-2009 Healthcare Cost and Utilization Project's Nationwide Inpatient Sample, a nationally representative dataset, to identify patients 18-49 years old admitted to an acute care hospital. We compared the rate of nonalcoholic cirrhosis between those with a SV diagnosis and patients without congenital heart disease.


RESULTS
There were 7968 hospitalizations of patients with a SV diagnosis and 13,602,149 hospitalizations of patients without congenital heart disease. SV patients were more likely to have nonalcoholic cirrhosis than those without congenital heart disease (4.3 ± 0.7 vs. 0.3 ± 0.01%, univariate OR 15.2, 95%CI 10.9-21.3), even after adjusting for viral or chronic hepatitis and other cirrhosis risk factors (multivariable OR 21.6, 95%CI 4.3-32.5). The proportion of all hospitalizations among SV patients for nonalcoholic cirrhosis increased by 173% between 1998/9 and 2008/9, from 2.3% to 6.2% (p=0.009). Among those with nonalcoholic cirrhosis, SV patients were more likely to have congestive hepatopathy (6.6 ± 3.1 vs. 0.1 ± 0.0001%, OR 63.2, 95%CI 19.2-207.8), longer hospital stays and higher hospital charges.


CONCLUSIONS
A single ventricle diagnosis is associated with markedly higher risk for nonalcoholic cirrhosis in a population-based sample of hospitalized patients. The proportion of patients with single ventricle anatomy admitted for nonalcoholic cirrhosis or its complications is increasing rapidly.
Division of Cardiology, Department of Medicine, University of Washington Medical Center, Seattle, WA 98195, United States. ekrieger@cardiology.washington.edu
Eric V
EV
Krieger
Lilamarie E
LE
Moko
Fred
F
Wu
Michael J
MJ
Landzberg
Anne Marie
AM
Valente
Gabriele Egidy
GE
Assenza
Chinweike
C
Ukomadu
Alexander R
AR
Opotowsky
D000293:Adolescent; D000328:Adult; D015331:Cohort Studies; D016208:Databases, Factual; D005260:Female; D006330:Heart Defects, Congenital / Q000175:diagnosis* / Q000453:epidemiology*; D006352:Heart Ventricles / Q000002:abnormalities*; D006801:Humans; D008103:Liver Cirrhosis / Q000175:diagnosis* / Q000453:epidemiology*; D008297:Male; D008875:Middle Aged; D012307:Risk Factors; D055815:Young Adult