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Cardiopulmonary exercise testing in the evaluation of liver disease in adults who have had the Fontan operation
Anushree Agarwal1, Colin Cunnington2, Aarthi Sabanayagam1
1University of California San Francisco, San Francisco, CA, USA.
Insights
Declines in cardiopulmonary exercise testing (CPET) variables like peak oxygen consumption are linked to higher odds of liver disease (LD) in adults after the Fontan operation. This suggests closer monitoring of liver health in these patients.
Area of Science:
- Cardiology
- Hepatology
- Pediatric Cardiology
Background:
- Liver disease (LD) is a known complication in adults following the Fontan operation for single-ventricle congenital heart disease.
- While reduced cardiopulmonary exercise testing (CPET) variables correlate with hospitalization risk, their association with LD remains unclear.
Purpose of the Study:
- To investigate the relationship between CPET variables and the presence of LD in adults who have undergone the Fontan operation.
Main Methods:
- Retrospective review of medical records from two tertiary institutions.
- Analysis of clinical, echocardiographic, liver imaging, and CPET data from 114 adult Fontan survivors.
- Statistical analysis to determine associations between CPET metrics and LD, adjusting for relevant covariates.
Main Results:
- Among 81 patients with liver imaging, 41% exhibited LD.
- A decrease in per-cent-predicted peak oxygen consumption (VO2) and per-cent-predicted peak oxygen pulse on CPET was significantly associated with increased odds of LD (odds ratio 2.3 per SD decrease).
- Reduced CPET variables were also linked to an earlier estimated onset of LD.
Conclusions:
- Deterioration in per-cent-predicted peak oxygen consumption and oxygen pulse is associated with a higher likelihood of developing LD in Fontan survivors.
- These findings underscore the importance of prompt hepatic evaluation in Fontan patients presenting with abnormal or declining CPET results.
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.
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