PMID- 29198937 OWN - NLM STAT- MEDLINE DCOM- 20181003 LR - 20181004 IS - 1875-2128 (Electronic) IS - 1875-2128 (Linking) VI - 111 IP - 4 DP - 2018 Apr TI - Cardiopulmonary exercise testing in the evaluation of liver disease in adults who have had the Fontan operation. PG - 276-284 LID - S1875-2136(17)30224-3 [pii] LID - 10.1016/j.acvd.2017.09.001 [doi] 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 (VO(2)) was 18.6+/-5.7mL/kg/min, per-cent-predicted peak VO(2) 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 VO(2) (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 VO(2) 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 VO(2) 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. CI - Copyright (c) 2017 Elsevier Masson SAS. All rights reserved. FAU - Agarwal, Anushree AU - Agarwal A AD - University of California San Francisco, San Francisco, CA, USA. FAU - Cunnington, Colin AU - Cunnington C AD - Manchester Royal Infirmary, Central Manchester University Hospitals NHS Trust, Manchester, United Kingdom. FAU - Sabanayagam, Aarthi AU - Sabanayagam A AD - University of California San Francisco, San Francisco, CA, USA. FAU - Zier, Lucas AU - Zier L AD - University of California San Francisco, San Francisco, CA, USA. FAU - McCulloch, Charles E AU - McCulloch CE AD - University of California San Francisco, San Francisco, CA, USA. FAU - Harris, Ian S AU - Harris IS AD - University of California San Francisco, San Francisco, CA, USA. FAU - Foster, Elyse AU - Foster E AD - University of California San Francisco, San Francisco, CA, USA. FAU - Atkinson, Dougal AU - Atkinson D AD - Manchester Royal Infirmary, Central Manchester University Hospitals NHS Trust, Manchester, United Kingdom. FAU - Bryan, Angela AU - Bryan A AD - Manchester Royal Infirmary, Central Manchester University Hospitals NHS Trust, Manchester, United Kingdom. FAU - Jenkins, Petra AU - Jenkins P AD - Manchester Royal Infirmary, Central Manchester University Hospitals NHS Trust, Manchester, United Kingdom. FAU - Dua, Jaspal AU - Dua J AD - Manchester Royal Infirmary, Central Manchester University Hospitals NHS Trust, Manchester, United Kingdom. FAU - Parker, Michael J AU - Parker MJ AD - Manchester Royal Infirmary, Central Manchester University Hospitals NHS Trust, Manchester, United Kingdom. FAU - Karunaratne, Devinda AU - Karunaratne D AD - Manchester Royal Infirmary, Central Manchester University Hospitals NHS Trust, Manchester, United Kingdom. FAU - Moore, John A AU - Moore JA AD - Manchester Royal Infirmary, Central Manchester University Hospitals NHS Trust, Manchester, United Kingdom. FAU - Meadows, Jeffrey AU - Meadows J AD - University of California San Francisco, San Francisco, CA, USA. FAU - Clarke, Bernard AU - Clarke B AD - Manchester Royal Infirmary, Central Manchester University Hospitals NHS Trust, Manchester, United Kingdom. FAU - Hoschtitzky, J Andreas AU - Hoschtitzky JA AD - Manchester Royal Infirmary, Central Manchester University Hospitals NHS Trust, Manchester, United Kingdom. FAU - Mahadevan, Vaikom S AU - Mahadevan VS AD - University of California San Francisco, San Francisco, CA, USA; Manchester Royal Infirmary, Central Manchester University Hospitals NHS Trust, Manchester, United Kingdom. Electronic address: vaikom.mahadevan@ucsf.edu. LA - eng PT - Journal Article PT - Multicenter Study DEP - 20171201 PL - Netherlands TA - Arch Cardiovasc Dis JT - Archives of cardiovascular diseases JID - 101465655 SB - IM MH - Adult MH - Cardiorespiratory Fitness MH - Child MH - Child, Preschool MH - England MH - *Exercise Test MH - Female MH - Fontan Procedure/*adverse effects MH - Heart Defects, Congenital/physiopathology/*surgery MH - Hemodynamics MH - Humans MH - Liver Diseases/*diagnosis/etiology/physiopathology MH - Liver Function Tests MH - Male MH - Medical Records MH - Oxygen Consumption MH - Predictive Value of Tests MH - Retrospective Studies MH - Risk Factors MH - San Francisco MH - Tertiary Care Centers MH - Time Factors MH - Treatment Outcome MH - Ventricular Function MH - Young Adult OTO - NOTNLM OT - Cardiopulmonary exercise test OT - Fontan OT - Hepatopathie OT - Liver disease OT - Operation de Fontan OT - Test d'effort cardiopulmonaire EDAT- 2017/12/05 06:00 MHDA- 2018/10/04 06:00 CRDT- 2017/12/05 06:00 PHST- 2017/01/16 00:00 [received] PHST- 2017/09/08 00:00 [revised] PHST- 2017/09/13 00:00 [accepted] PHST- 2017/12/05 06:00 [pubmed] PHST- 2018/10/04 06:00 [medline] PHST- 2017/12/05 06:00 [entrez] AID - S1875-2136(17)30224-3 [pii] AID - 10.1016/j.acvd.2017.09.001 [doi] PST - ppublish SO - Arch Cardiovasc Dis. 2018 Apr;111(4):276-284. doi: 10.1016/j.acvd.2017.09.001. Epub 2017 Dec 1.