Pulmonary Capillary Recruitment in Fontan Patients

NCT ID: NCT04292483

Last Updated: 2022-04-07

Basic Information

• Recruitment Status: UNKNOWN
• Total Enrollment: 15 participants
• Study Classification: OBSERVATIONAL
• Study Start Date: 2020-06-11
• Study Completion Date: 2023-12-31

Brief Summary

This study evaluates the effects of pulmonary vasodilator therapy on pulmonary capillary blood flow by measuring the functional capillary surface area (FCSA) at baseline and post nitric oxide inhalation, through the injection of 3H-benzoyl-Phe-Ala-Pro (BPAP). FCSA will be related to flow and other hemodynamic parameters in order to determine if there is capillary recruitment or distention in Fontan patients. We will also compare baseline FCSA measurements with previously studied normal subjects, to assess the difference in hemodynamic pulmonary functional parameters between these single ventricle physiology patients and normal subjects.

Conditions

Congenital Heart Disease

Study Design

• Observational Model Type: COHORT
• Study Time Perspective: PROSPECTIVE

Interventions

Nitric Oxide

Inhaled vasodilator to increase pulmonary capillary perfusion

Intervention Type: DRUG

Eligibility Criteria

Inclusion Criteria

Fontan failures will be defined as patients with single ventricle physiology and TCPC with clinical signs of dysfunction defined as any of the following:

1. Decreased functional class WHO/NYHA II or more and clinical signs of congestion (ascites, peripheral edema, increased JVP) and/or NT-proBNP > 399 pg/ml based on [17].

2. Age > 18 years

3. Known Fontan's increased pressures a. Fontan mean pressure of ≥15 mmHg and/or transpulmonary gradient ≥ 5 mmHg. b. Pulmonary vascular resistance ≥ 2 woods units. And/or supporting evidence of end-organ damage

1. Clinical or paraclinical evidence of liver congestion and/or fibrosis

a. Evidence in ultrasound or other imaging techniques of liver congestion and/or changes related to cardiac cirrhosis.

b. Fibroscan with grade 2 or + 2. Paraclinical signs of portal hypertension defined as 2 or more:

1. Thrombocytopenia defined as platelet count < 150.0000

2. Leukocytopenia defined as white blood cell less than 4.500

3. Esophageal varices on esophagoduodenoscopy.

4. Splenomegaly on imaging -

Exclusion Criteria

1. Presence of fenestration - the shunts include veno-veno collaterals

2. Significant anemia defined as Hgb < 120 mg/dl

3. Pregnancy at the moment of the procedure.

4. Taking angiotensin-converting enzyme inhibitors or angiotensin receptor blockers.

5. Usage of nitrates, phosphodiesterase inhibitors or calcium blockers within 3 months of the study.

-

• Minimum Eligible Age: 18 Years
• Maximum Eligible Age: 60 Years
• Eligible Sex: ALL
• Accepts Healthy Volunteers: No

Sponsors

National and Kapodistrian University of Athens

OTHER

Sponsor Role: collaborator

Tel Aviv University

OTHER

Sponsor Role: collaborator

Old Dominion University

OTHER

Sponsor Role: collaborator

Jewish General Hospital

OTHER

Sponsor Role: lead

Responsible Party

Dr David Langleben

Professor of Medicine (Cardiology)

Responsibility Role: PRINCIPAL_INVESTIGATOR

Principal Investigators

David Langleben, MD

Role: PRINCIPAL_INVESTIGATOR

Jewish General Hospital

Locations

Jewish General Hospital

Montreal, Quebec, Canada

Site Status: RECRUITING

Countries

Canada

Central Contacts

David Langleben, MD

Role: CONTACT

david.langleben@mcgill.ca

5143407531

Judith Therrien, MD

Role: CONTACT

judith.therrien@mcgill.ca

514-340-8232

Other Identifiers

1900-5, 00-034

Identifier Type: -

Identifier Source: org_study_id