Diffuse Myocardial Fibrosis in Fontan Patients

Oregon Health & Science University (OHSU)

Status: Completed

Conditions: Single Ventricle; Myocardial Fibrosis; Congenital Heart Disease

Study type: Observational

Funder types: Other

Identifiers

NCT01929174

OHSUIRB00009372

Details and patient eligibility

About

The purpose of this protocol is to measure the relaxation of the heart in subjects with single ventricles who have undergone the surgical Fontan procedure. We will do this by measuring relaxation with MRI, echocardiography, and cardiac catheterization and compare to blood levels that measure heart scarring. We will also measure relaxation before and after boluses of intravenous (IV) fluids to see if the relaxation changes when there is more fluid in the heart. Measurements of heart relaxation will be obtained from the MRI, echocardiogram, and cardiac catheterization for each patient and compared to blood markers of heart scarring. We aim to compare all of these measurements to see if we can accurately identify heart scarring and, if present, how much it correlates with impaired heart relaxation.

Enrollment: 18 patients

Sex: All

Ages: 18 to 90 years old

Volunteers: No Healthy Volunteers

Inclusion and exclusion criteria

Inclusion criteria: adult patients (age ≥18) with a previous Fontan procedure (including all variants) referred for catheterization. This includes individuals with tricuspid atresia, double inlet left ventricle, unbalanced atrioventricular septal defect, and pulmonary atresia with intact septum. Control subjects will be selected from individuals referred to the lab for evaluation/closure of a patent foramen ovale (PFO). All subjects must be willing to participate in all portions of the study and provide written informed consent.

Exclusion criteria: contraindications to MRI such as ferromagnetic objects in the chest, claustrophobia, or contraindication to gadolinium contrast (estimated creatinine clearance < 30 ml/min/1.73m2). Fontan patients with a morphologic right ventricle (namely hypoplastic left heart after a Norwood palliation) will be excluded, because of the disproportionate risk of systolic dysfunction in the morphologic right ventricle.33 Control patients with hypertension, diabetes, coronary disease, or reduced systolic function will be excluded.

Trial design

18 participants in 2 patient groups

Controls

Description:

Patients undergoing catheterization for other reasons who have a normal biventricular heart.

Single ventricle Fontan

Description:

Patients with a single functional ventricle (excluding hypoplastic left heart syndrome) palliated with a "Fontan" type operation involving passive blood flow to the lungs.