Agarwal A, Cunnington C, Sabanayagam A, Zier L, McCulloch CE,
Harris IS, Foster E, Atkinson D, Bryan A, Jenkins P, Dua J,
Parker MJ, Karunaratne D, Moore JA, Meadows J, Clarke B,
Hoschtitzky JA, Mahadevan VS.

Arch Cardiovasc Dis. 2018 Apr;111(4):276-284.

BACKGROUND: Liver disease (LD) is a long-term complication in patients with a
single ventricle who have had the Fontan operation. A decline in cardiopulmonary
exercise testing (CPET) variables is associated with increased risk of
hospitalization, but its association with LD is unknown.
AIM: To determine the association between CPET variables and LD in adults who
have had the Fontan operation.
METHODS: We retrospectively reviewed the medical records from two tertiary
institutions.
RESULTS: We identified 114 adults (≥18 years; mean 30.9±7.4 years) who had
undergone the Fontan operation: 56% were women; 63% had total cavopulmonary
connection; 66% had New York Heart Association (NYHA) class I status; 42% had
arrhythmias; 22% had systemic right ventricle; and 35% had ventricular
dysfunction. Of 81 patients with liver-imaging data, 41% had LD (i.e. imaging
evidence of cirrhosis, with or without portal hypertension, splenomegaly or
varices). There were no differences in clinical or echocardiographic variables
between those with and without LD. Among the 58 patients with CPET data, mean
peak oxygen consumption (VO2) was 18.6±5.7mL/kg/min, per-cent-predicted peak VO2
was 53.9±15.5%, peak oxygen pulse was 9.3±2.9mL/beat and per-cent-predicted peak
oxygen pulse was 82.6±21.5%. Of the 44 patients with liver and CPET data, each
standard deviation decrease in per-cent-predicted peak VO2 (16%) and
per-cent-predicted peak oxygen pulse (22%) was associated with a 2.3-fold
increase in the odds of LD, after adjusting for NYHA, institution and Fontan type
(P=0.04). Similarly, each standard deviation decrease in per-cent-predicted peak
VO2 and oxygen pulse was associated with an estimated 5.9-year and 4.9-year
earlier onset of LD, respectively (P>0.05).
CONCLUSIONS: Decline in per-cent-predicted peak VO2 and oxygen pulse was
associated with increased odds of LD in adults who had undergone the Fontan
operation. Our study supports more rapid hepatic evaluation among patients with
abnormal or worsening CPET variables.