Clinical Research Directory

The Effect of Dapagliflozin on Exercise and Cardiac Functional Status of Patients After Fontan Procedure

While the Fontan operation improves survival for patients with a functional single ventricle, complications like heart failure remain prevalent during follow-up, causing frequent hospitalizations and reduced quality of life. Currently, there are no evidence-based heart failure therapies or guidelines for patients with Fontan palliation. There is growing interest in using sodium-glucose cotransporter 2 inhibitors (SGLT2i) to optimize cardiac conditions in this group. SGLT2i's observed benefits relate to protective effects on cardiac energy metabolism, renal function, sympathetic activation, inflammation, oxidative stress, erythropoiesis, and vascular function. Therefore, SGLT2i may represent a new therapeutic agent to improve exercise capacity and ventricular function in patients with Fontan circulation. This prospective, open-label, single-arm clinical trial investigates the FDA-approved drug dapagliflozin. An open-label design is utilized due to the relatively small cohort of patients with complex underlying hearts. The trial will be conducted at Hong Kong Children's Hospital (the sole pediatric cardiology center in Hong Kong) and Queen Mary Hospital (the largest tertiary referral center for adult congenital heart patients). The total study duration is 2 years. Subjects will participate for 3 months, receiving oral dapagliflozin 10mg once daily. The primary endpoint is VO2 max (maximum rate of oxygen consumption) during cardiopulmonary exercise testing at 3 months. Secondary assessments include questionnaires, history taking, physical examinations, 12-lead electrocardiogram, blood tests, echocardiography, bioelectrical impedance analysis, and urine pregnancy tests. If the hypothesis holds true, clinical translation of this project will: i) Improve long-term outcomes and quality-of-life for Fontan patients, reducing the healthcare burden from repeated heart failure hospitalizations; ii) Revolutionize the management of Fontan-associated heart failure by introducing SGLT2i as a novel, evidence-based therapy locally and globally; iii) Pave the way for multicenter international trials evaluating SGLT2i in the Fontan population and broader congenital heart diseases.

Adult Congenital Heart Disease International EValuation of the Effectiveness of SGLT2i Registry

This real-world, international registry aims to evaluate the current experience with sodium-glucose cotransporter 2 inhibitors (SGLT2i) in adult congenital heart disease (ACHD) patients by investigating the prescription patterns, safety, tolerability, and potential beneficial effects on heart failure-related outcomes.

Cholate Clearance in Fontan and Heart Failure

The goal of this study is to use the HepQuant dual cholate clearance assay, which has been shown to measure liver function in people with known chronic liver conditions, to assess severity of Fontan-associated liver disease (FALD). This study aims to understand the role impaired blood flow to the liver plays in liver function in Fontan patients compared to patients with right heart failure and healthy controls. The study will also determine whether cholate clearance is a good measure to use in this population, and whether it will be able to predict clinical outcomes. Participants will undergo a HepQuant dual cholate clearance assay and a cardiac magnetic resonance imaging (MRI) at the beginning of the study, and then data on health status will be collected for 5 years.

Canadian Adult Congenital Heart Disease Intervention Registry

The ACHDi Registry study will create a foundational database for adult congenital heart disease interventions. This Pan-Canadian Registry will collect clinical and patient-reported information that will enable the evaluation of care processes and outcomes in five most common ACHDi interventions by enabling prospective and retrospective registry-based studies to answer important clinical practice and policy-relevant questions.