CAMEO-FONTAN -Dapagliflozin in the Failing Fontan Circulation

Prior treatment

• Any of the following medications: Insulin or Pramlintide, Sandostatin, Akeega (abiraterone acetate), Growth Hormone (Somatogrogon-GHLA), Lithium, Chloroquine, Hydroxychloroquine, Thioctic acid (alpha lipoic acid), Empagliflozin or Canagliflozin

Other illness

• Type I diabetes
• Significant valvular heart disease (≥moderate stenosis, \>moderate regurgitation)
• Dyspnea due to primary lung disease or myocardial ischemia in the opinion of the investigator
• History of serious hypersensitivity reaction to dapagliflozin
• Subjects with severe liver disease with history of decompensation including ascites or encephalopathy or variceal bleeding
• Single ventricle heart disease without Fontan palliation
• Those with a history of Fontan takedown

Lab values

• Severe anemia (hemoglobin \<9 gm/dl)
• Severe kidney disease (estimated GFR\<30) or liver disease

Other

• Recent hospitalization (\<30 days) or revascularization (\<90 days)
• Significant Fontan obstruction with resting SVC-IVC gradient \>4 mmHg
• Primary cardiomyopathy (such as amyloid)
• Women of childbearing potential not willing to use a medically accepted method of contraception OR who are currently pregnant (confirmed with positive pregnancy test) or breast feeding.
• Subjects on dialysis

• Signed informed consent prior to any study specific procedures
• History of Fontan procedure
• Male or female subject
• Age ≥ 18
• Symptoms of dyspnea (NYHA II-IV) with no non-cardiac or ischemia explanation
• Elevated peripheral venous pressure (PVP) at rest or during exercise (≥20 mmHg) ascertained at Visit 1. Patients that have consented to study procedures but do not meet this invasive criterion will be considered as screen failures and will not be randomized.