Efficacy of Phosphodiesterase-type 5 Inhibitors in Patients With Univentricular Congenital Heart Disease

Exclusion Criteria

• •1. Patient who is unable to perform a cardio-pulmonary exercise test.
• •2. Cardiac surgery planned during the trial.
• •3. Patient treated by any pulmonary arterial vasodilator drug, as defined in the 2015 PH guidelines (52), within 6 months before inclusion, regardless the duration and the type(s) (oral, intravenous, subcutaneous, inhaled) of administration.
• •4. Patient treated by Sildenafil or any other type of phosphodiesterase-type 5 inhibitor (such as tadalafil) within 6 months before inclusion, regardless the duration of administration.
• •5. Interventional cardiac catheterization planned during the trial (collateral occlusion, fenestration occlusion, stenting, angioplasty, ablation of rhythm disorder), other than during the screening.
• •6. Participation in another clinical trial or administration of an off-label drug in the 4 weeks preceding the screening.
• •7. Pregnancy, desire for pregnancy, absence of contraception during the study period.
• •8. Severe hepatic insufficiency (Child-Pugh C class).
• •9. Hypersensitivity to the active substance or to any of the excipients of the tablet:
• •microcrystalline cellulose, calcium hydrogen phosphate anhydrous, croscarmellose sodium, stearate of magnesium, hypromellose, titanium dioxide (E171), monohydrate lactose, glycerol triacetate.
• •10. Combination with products called "nitric oxide donors" (such as amyl nitrite) or with nitrates in any form, due to the hypotensive effects of nitrates.
• •11. Concomitant administration of PDE5 inhibitors, such as Sildenafil, with guanylate cyclase stimulators, such as Riociguat.
• •12. Combination with the most potent inhibitors of CYP3A4 (eg ketoconazole, itraconazole, ritonavir).
• •13. Disposition to priapism, sclerosis of corpora cavernosa, disease of La Peyronie, sickle cell anemia, multiple myeloma, leukemia.
• •14. Uncontrolled hypotension or risk of hypotension: water depletion, obstruction to ejection of the left ventricle, dysfunction of the autonomic nervous system, patient under alpha-blocker.
• •15. Severe cardiovascular events, recent (\<3 months) or not stabilized: myocardial infarction, unstable angina, sudden cardiac death, ventricular arrhythmia, cerebrovascular hemorrhage.
• •16. Active hemorrhagic disorders.
• •17. Active gastro-duodenal ulcer.
• •18. Patients with loss of vision of an eye due to non-arteritic anterior ischemic optic neuropathy (NAION), whether or not this event has been associated with previous exposure to a PDE5 inhibitor.