Pulmonary Capillary Recruitment in Fontan Patients

Pulmonary Capillary Recruitment With Pulmonary Vasodilator Therapy in Patients With Univentricular Heart Physiology and Failing Total Cavo-pulmonary Connection.

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.

Study Overview

• Status: Recruiting
• Conditions: Congenital Heart Disease
• Intervention / Treatment: Drug: Nitric Oxide

• Study Type: Observational
• Enrollment (Anticipated): 15

Contacts and Locations

• Study Contact: Name: Judith Therrien, MD; Phone Number: 514-340-8232; Email: judith.therrien@mcgill.ca

Study Locations

• Canada
  • Quebec
    • Montreal, Quebec, Canada, H3T 1E2
      • Recruiting
      • Jewish General Hospital

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years to 60 years (Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Probability Sample

Study Population

Subjects with Fontan failure as described above

Description

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.

   -

Study Plan

How is the study designed?

Design Details

• Observational Models: Cohort
• Time Perspectives: Prospective

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Functional capillary surface area	Measurement of functional capillary surface area pre and post inhaled nitric oxide	30 minutes

Collaborators and Investigators

• Sponsor: Jewish General Hospital
• Collaborators: Tel Aviv University; National and Kapodistrian University of Athens; Old Dominion University
• Investigators: Principal Investigator: David Langleben, MD, Jewish General Hospital

Study record dates

Study Major Dates

• Study Start (Actual): June 11, 2020
• Primary Completion (Anticipated): December 31, 2023
• Study Completion (Anticipated): December 31, 2023

Study Registration Dates

• First Submitted: February 29, 2020
• First Submitted That Met QC Criteria: February 29, 2020
• First Posted (Actual): March 3, 2020

Study Record Updates

• Last Update Posted (Actual): April 7, 2022
• Last Update Submitted That Met QC Criteria: March 29, 2022
• Last Verified: March 1, 2022

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Cardiovascular Diseases; Congenital Abnormalities; Cardiovascular Abnormalities; Heart Diseases; Heart Defects, Congenital; Physiological Effects of Drugs; Neurotransmitter Agents; Molecular Mechanisms of Pharmacological Action; Vasodilator Agents; Autonomic Agents; Peripheral Nervous System Agents; Protective Agents; Bronchodilator Agents; Anti-Asthmatic Agents; Respiratory System Agents; Antioxidants; Free Radical Scavengers; Endothelium-Dependent Relaxing Factors; Gasotransmitters; Nitric Oxide
• Other Study ID Numbers: 1900-5, 00-034

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: No

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No