Cardiopulmonary exercise testing in the evaluation of liver disease in adults who have had the Fontan operation

Anushree Agarwal; Colin Cunnington; Aarthi Sabanayagam; Lucas Zier; Charles E McCulloch; Ian S Harris; Elyse Foster; Dougal Atkinson; Angela Bryan; Petra Jenkins; Jaspal Dua; Michael J Parker; Devinda Karunaratne; John A Moore; Jeffrey Meadows; Bernard Clarke; J Andreas Hoschtitzky; Vaikom S Mahadevan

doi:10.1016/j.acvd.2017.09.001

Cardiopulmonary exercise testing in the evaluation of liver disease in adults who have had the Fontan operation

Anushree Agarwal, Colin Cunnington, Aarthi Sabanayagam, Lucas Zier, Charles E McCulloch, Ian S Harris, Elyse Foster, Dougal Atkinson, Angela Bryan, Petra Jenkins, Jaspal Dua, Michael J Parker, Devinda Karunaratne, John A Moore, Jeffrey Meadows, Bernard Clarke, J Andreas Hoschtitzky, Vaikom S Mahadevan

Division of Cardiovascular Sciences (L5)

Research output: Contribution to journal › Article › peer-review

Abstract

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.

Original language	English
Pages (from-to)	276-284
Number of pages	9
Journal	Archives of cardiovascular diseases
Volume	111
Issue number	4
Early online date	1 Dec 2017
DOIs	https://doi.org/10.1016/j.acvd.2017.09.001
Publication status	Published - 1 Apr 2018

Keywords

Cardiopulmonary exercise test
Fontan
Liver disease

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

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10.1016/j.acvd.2017.09.001

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Agarwal, A., Cunnington, C., Sabanayagam, A., Zier, L., McCulloch, C. E., Harris, I. S., Foster, E., Atkinson, D., Bryan, A., Jenkins, P., Dua, J., Parker, M. J., Karunaratne, D., Moore, J. A., Meadows, J., Clarke, B., Hoschtitzky, J. A., & Mahadevan, V. S. (2018). Cardiopulmonary exercise testing in the evaluation of liver disease in adults who have had the Fontan operation. Archives of cardiovascular diseases, 111(4), 276-284. https://doi.org/10.1016/j.acvd.2017.09.001

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title = "Cardiopulmonary exercise testing in the evaluation of liver disease in adults who have had the Fontan operation",

abstract = "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.",

keywords = "Cardiopulmonary exercise test, Fontan, Liver disease",

author = "Anushree Agarwal and Colin Cunnington and Aarthi Sabanayagam and Lucas Zier and McCulloch, {Charles E} and Harris, {Ian S} and Elyse Foster and Dougal Atkinson and Angela Bryan and Petra Jenkins and Jaspal Dua and Parker, {Michael J} and Devinda Karunaratne and Moore, {John A} and Jeffrey Meadows and Bernard Clarke and Hoschtitzky, {J Andreas} and Mahadevan, {Vaikom S}",

year = "2018",

month = apr,

day = "1",

doi = "10.1016/j.acvd.2017.09.001",

language = "English",

volume = "111",

pages = "276--284",

journal = "Archives of cardiovascular diseases",

issn = "1875-2128",

publisher = "Elsevier BV",

number = "4",

}

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 2018, 'Cardiopulmonary exercise testing in the evaluation of liver disease in adults who have had the Fontan operation', Archives of cardiovascular diseases, vol. 111, no. 4, pp. 276-284. https://doi.org/10.1016/j.acvd.2017.09.001

TY - JOUR

T1 - Cardiopulmonary exercise testing in the evaluation of liver disease in adults who have had the Fontan operation

AU - Agarwal, Anushree

AU - Cunnington, Colin

AU - Sabanayagam, Aarthi

AU - Zier, Lucas

AU - McCulloch, Charles E

AU - Harris, Ian S

AU - Foster, Elyse

AU - Atkinson, Dougal

AU - Bryan, Angela

AU - Jenkins, Petra

AU - Dua, Jaspal

AU - Parker, Michael J

AU - Karunaratne, Devinda

AU - Moore, John A

AU - Meadows, Jeffrey

AU - Clarke, Bernard

AU - Hoschtitzky, J Andreas

AU - Mahadevan, Vaikom S

PY - 2018/4/1

Y1 - 2018/4/1

N2 - 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.

AB - 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.

KW - Cardiopulmonary exercise test

KW - Fontan

KW - Liver disease

U2 - 10.1016/j.acvd.2017.09.001

DO - 10.1016/j.acvd.2017.09.001

M3 - Article

C2 - 29198937

SN - 1875-2128

VL - 111

SP - 276

EP - 284

JO - Archives of cardiovascular diseases

JF - Archives of cardiovascular diseases

IS - 4

ER -

Cardiopulmonary exercise testing in the evaluation of liver disease in adults who have had the Fontan operation

Abstract

Keywords

UN SDGs

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