Table 1 In-/exclusion criteria

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Fontan/TCPC operated

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Expected clinical stability > 3 months, evaluated by investigator from clinical record

Exclusion criteria

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Age < 12 years

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Severe heart failure (NYHA-class IV)

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Oxygen saturation < 85% at rest

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Pre-existing liver condition (transaminases 2x > upper reference limit)

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Renal failure (creatinin > 150 mmol/l)

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Restrictive Fontan circuit

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History of work induced severe arrhythmia

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Systolic blood pressure below 80% of reference (< 88 mmHg)

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Use of any of following drugs: Fluconazole, Ketoconazole, CyclosporineA, Lopinavir, Ritonavir, Rifampicin, Carbamazepine and Phenytoin

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Severe extra-cardiac condition e.g. neurological impairment

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For women: Positive HCG or no use of contraception*

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History of low compliance