Actively Recruiting
Acute Imposition of Fontan Physiology in The Single Ventricle Patient: Effects on Fibrosis, Function and Drug Intervention
Led by Children's Hospital of Philadelphia · Updated on 2025-06-08
145
Participants Needed
1
Research Sites
N/A
Total Duration
On this page
Sponsors
C
Children's Hospital of Philadelphia
Lead Sponsor
N
National Heart, Lung, and Blood Institute (NHLBI)
Collaborating Sponsor
AI-Summary
What this Trial Is About
This research aims to understand the early development of liver and cardiac fibrosis, as well as lymphatic congestion, in children with single ventricle (SV) heart conditions undergoing the Fontan operation. It investigates how acute changes in heart function from the Fontan procedure affect these complications and tests the antifibrotic drug spironolactone in a pilot trial. The study uses MRI and blood tests to non-invasively explore these complex interactions and aims to see if MRI can detect related changes. Children aged 1 to 6 years with single left or right ventricle heart conditions scheduled for Fontan surgery will be studied. Some children will receive spironolactone, a mild diuretic drug, starting after surgery either before hospital discharge or about two weeks later. The dose is adjusted by weight with a maximum daily dose of 200 mg. All children, including those not receiving the drug and control children with normal heart and liver function, will undergo MRI and blood tests before surgery and about one year after. Participants will have detailed assessments including MRI scans of the liver and heart to measure fibrosis and function, blood tests for fibrosis markers, and evaluations of lymphatic function. Data on demographics, medical history, adverse events, and heart function will be collected. Some MRIs will be coordinated with clinical care to minimize extra procedures. The main outcomes measured are changes in liver and heart tissue characteristics over one year, with ongoing monitoring and data collection throughout the study period.
CONDITIONS
Brief Title
Fibrosis and the Fontan
Who Can Participate
Eligibility Criteria
You may qualify if you...
- Single ventricle (SV) patients aged between 1 and 6 years
- Single left or single right ventricle
- Scheduled to undergo Fontan operation at Children's Hospital of Philadelphia
- Parents or guardians provide informed consent
- For controls: normal ventricular function and normal liver, undergoing clinically indicated MRI with anesthesia, and receiving contrast for clinical purposes
- Patients in other approved interventional studies may be included in observational arm
- Families willing to continue participation if stopping study medication early
You will not qualify if you...
- Any condition deemed detrimental to the patient by their physician
- Contraindication to sedated MRI such as having a pacemaker
- Current use of spironolactone, eplerenone, enalapril, or other angiotensin receptor blockers
- Hyperkalemia or Addison disease (for study drug group)
- Hypersensitivity to spironolactone or its components
- Severe renal insufficiency (eGFR less than 30 mL/min/1.73 m2)
- Participation in other studies that would affect trial results without approval
AI-Screening
AI-Powered Screening
Complete this quick 3-step screening to check your eligibility
Your Study Journey
Duration - 2 to 4 weeks
Participants are screened for eligibility to participate in the trial.
1 screening and enrollment visit (in-person)
Duration - Up to 1 year after Fontan surgery
Participants receive the study drug spironolactone starting after the Fontan surgery either in the hospital prior to discharge or at the first outpatient visit about 2 weeks after discharge. The drug dose is adjusted based on weight and clinically administered as per standard care.
Initial hospital stay and outpatient visits as per clinical care
Duration - Approximately 1 year post-surgery
Participants undergo characterization and measurement of liver and cardiac fibrosis with MRI and cardiac magnetic resonance (CMR), serum biomarkers, and assessment of lymphatic function approximately 1 year after the Fontan operation. Additional clinical and imaging assessments may occur depending on participant status.
1 follow-up visit (in-person) around 1 year after surgery
Trial Site Locations
Total: 1 location
1
Children's Hospital of Philadelphia
Philadelphia, Pennsylvania, United States, 19130
Actively Recruiting
Research Team
M
Mark Fogel, MD
C
Cassandra L Giner, MS
How is the study designed?
Study Type
INTERVENTIONAL
Masking
NONE
Allocation
NON_RANDOMIZED
Model
PARALLEL
Primary Purpose
TREATMENT
Number of Arms
5
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