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A Study Evaluating the Efficacy and Safety of IV L-Citrulline for the Prevention of Clinical Sequelae of Acute Lung Injury Induced by Cardiopulmonary Bypass in Pediatric Patients Undergoing Surgery for Congenital Heart Defects

Phase 3
Recruiting
Conditions
Ventricular Septal Defect
Atrioventricular Septal Defect
Primum Atrial Septal Defect
Interventions
Registration Number
NCT05253209
Lead Sponsor
Asklepion Pharmaceuticals, LLC
Brief Summary

This is a randomized, double-blind, placebo controlled, multicenter study to compare the efficacy and safety of L-citrulline versus placebo in patients undergoing surgery for congenital heart defects. Eligible patients undergoing repair of a large unrestrictive ventricular septal defect (VSD), a partial or complete atrioventricular septal defect (AVSD), or an ostium primum atrial septal defect (primum ASD) will be eligible for enrollment.

Detailed Description

This is a randomized, double-blind, placebo controlled, multicenter study that will compare the efficacy and safety of L- citrulline versus placebo in patients undergoing surgery for congenital heart defects. Eligible patients undergoing repair of a large unrestrictive ventricular septal defect (VSD), a partial or complete atrioventricular septal defect (AVSD), or an ostium primum atrial septal defect (primum ASD) will be eligible for enrollment.

Each enrolled patient will be randomized to receive either L citrulline or placebo throughout all administrations in the study. Patients will receive:

1. an L-citrulline bolus of 150 mg/kg or placebo at the initiation of cardiopulmonary bypass

2. the addition L-citrulline or placebo to maintain a steady state target concentration of approximately 100 μmol/L of L-Citrulline or placebo during cardiopulmonary bypass

3. an L-citrulline bolus of 10 mg/kg or placebo 30 minutes after decannulation from cardiopulmonary bypass, followed immediately by a 9 mg/kg/hour continuous L-citrulline infusion or placebo for up to 48 hours post-first dose. The infusion rate will be adjusted (up or down titration of drug infusion) to achieve a target steady state concentration of 100 µmol/L.

The study drug or placebo infusion will be discontinued once invasive arterial blood pressure monitoring is discontinued or at 48 hours, whichever occurs first. Patients will be followed until Day 28 or discharge from the hospital, whichever occurs first. For patients discharged prior to Day 28, a final assessment via telephone will be conducted at Day 28.

Recruitment & Eligibility

Status
RECRUITING
Sex
All
Target Recruitment
97
Inclusion Criteria
  • Patients, parents, or legal guardian willing and able to sign informed consent
  • Male and female subjects aged ≤18 years of age (females of child-bearing potential willing to practice an acceptable form of birth control)
  • Patients undergoing cardiopulmonary bypass for repair of a large unrestrictive ventricular septal defect, an ostium primum/secundum atrial septal defect, or a partial or complete atrioventricular septal defect
  • Pre-operative echocardiogram confirming cardiovascular anatomy and defect to be repaired
Exclusion Criteria
  • Evidence of pulmonary artery or vein abnormalities that will not be addressed surgically. Specific abnormalities excluded include:

    • significant pulmonary artery narrowing not amenable to surgical correction
    • previous pulmonary artery stent placement
    • significant left sided AV valve regurgitation not amenable to surgical correction
    • pulmonary venous return abnormalities not amenable to surgical correction
    • pulmonary vein stenosis not amenable to surgical correction
  • Preoperative requirement for mechanical ventilation or IV inotrope support

  • Presence of fixed or idiopathic pulmonary hypertension (i.e. Eisenmenger's Syndrome) prior to surgical repair

  • Pre-operative use of medications to treat pulmonary hypertension

  • Pregnancy; Sexually active females of child-bearing potential must be willing to practice an acceptable method of birth control for the duration of study participation (e.g. oral contraceptive, hormonal implant, intra-uterine device)

  • Participation in another clinical trial within 30 days of Screening or while participating in the current study, including the 28 days of follow-up post study drug administration.

  • Any condition which, in the opinion of the investigator, might interfere with the study objectives

Study & Design

Study Type
INTERVENTIONAL
Study Design
PARALLEL
Arm && Interventions
GroupInterventionDescription
PlaceboPlasmalyte APlasmalyte A administered to the same schedule as the active treatment arm.
ActiveL-citrullinePatients will receive: 1. an L-citrulline bolus of 150 mg/kg at the initiation of cardiopulmonary bypass 2. the addition L-citrulline to maintain a steady state target concentration of approximately 100 μmol/L of L-citrulline during cardiopulmonary bypass 3. an L-citrulline bolus of 10 mg/kg 30 minutes after decannulation from cardiopulmonary bypass, followed immediately by a 9 mg/kg/hour continuous L-citrulline infusion or placebo for up to 48 hours post-first dose. The infusion rate will be adjusted (up or down titration of drug infusion) to achieve a target steady state concentration of 100 μmol/L. Infusion will be discontinued once invasive arterial blood pressure monitoring is discontinued or at 48 hours, whichever occurs first.
Primary Outcome Measures
NameTimeMethod
Post-operative need for mechanical ventilationTime in hours from separation from CPB until discontinuation of all mechanical ventilation including non-invasive support or Day 28, whichever occurs first

Mechanical ventilation is defined as invasive and non-invasive mechanical ventilation including bilevel positive airway pressure (BPAP), continuous positive airway pressure (CPAP)

Secondary Outcome Measures
NameTimeMethod
Use of inotropesMeasured from first use until discharge or Day 28, whichever occurs first

Duration of inotrope use (e.g., dopamine, dobutamine, milrinone, epinephrine, phenylephrine and/or norepinephrine).

IntubationFrom separation from bypass until discontinuation of intubation or Day 28, whichever occurs first

Length of time on intubation

Positive pressure ventilationTime in hours from separation from CPB until discontinuation of all non-invasive mechanical ventilation or Day 28, whichever occurs first

Length of time on non-invasive mechanical ventilation

Early extubationFrom end of surgery until 12 hours post-surgery

Frequency of extubation \<12 hours after surgery

Duration of hospitalizationFrom surgery until discharge from hospital or Day 28, whichever occurs first

Number of post-operative days until discharge from hospital

Use of vasodilatorsMeasured from first use until discharge or Day 28, whichever occurs first

Duration of vasodilator use (e.g., nitroprusside, nitroglycerin, and nicardipine)

Volume of chest tube drainageDuration of chest tube placement or Day 28, whichever occurs first

Total amount of chest tube drainage (mL)

Hemodynamic improvement (heart rate)1, 2, 4, 12, 24, and 48 hours post-dose

Changes in heart rate measurements.

Duration of chest tube placementFrom the end of the surgery to the time the chest tube is removed or Day 28, whichever occurs first

Total post-operative time chest tube is used

Hemodynamic improvement (systemic arterial blood pressure)1, 2, 4, 12, 24, and 48 hours post-dose

Changes in systemic arterial systolic and diastolic blood pressure measurements.

Hemodynamic improvement (oxygen saturation)1, 2, 4, 12, 24, and 48 hours post-dose

Changes in oxygen saturation measurements.

Hemodynamic improvement (central venous pressure)1, 2, 4, 12, 24, and 48 hours post-dose

Changes in oxygen saturation measurements.

Hemodynamic improvement (pulmonary arterial pressure)1, 2, 4, 12, 24, and 48 hours post-dose

Changes in PAP measurements (when available).

Arterial blood gasses (PaO2)Intra-operatively to Day 28

Changes in PaO2 measurements

Arterial blood gasses (PaCO2)Intra-operatively to Day 28

Changes in PaCO2 measurements

Arterial blood gasses (HCO3)Intra-operatively to Day 28

Changes in HCO3 measurements

Arterial blood gasses (pH)Intra-operatively to Day 28

Changes in pH measurements

Plasma levels of L-citrulline to assess PK-PD (exposure-response) relationshipPre-surgery, 6, 12, 24 and 48 hours after first dose

Measurement of plasma levels of L-citrulline

Health Economics: mechanical ventilationTotal over duration of hospitalization or to Day 28 whichever occurs first

Measured as cost per day and expressed as incremental cost per quality adjusted life year (QALY) gained

Health Economics: duration of hospitalisationTotal over duration of hospitalization or to Day 28 whichever occurs first

Measured as total cost of hospitalisation expressed as incremental cost per quality adjusted life year (QALY) gained

Adverse eventsPre-operatively until Day 28

Incidence of adverse events and serious adverse events

Incidence of refractory hypotensionFrom the end of surgery until 48 hours after first dose

Number of subjects with any refractory hypotension. Defined as a drop of \>20% in mean arterial pressure for \>30 minutes.

Clinical laboratory values (Blood Hemoglobin and Total Bilirubin)Intra-operatively, Days 1, 2 and 28

Absolute values and the absolute and percentage changes from baseline.

Clinical laboratory values (Blood Haematocrit)Intra-operatively, Days 1, 2 and 28

Absolute values and the absolute and percentage changes from baseline.

Clinical laboratory values (Red Blood Cell Count)Intra-operatively, Days 1, 2 and 28

Absolute values and the absolute and percentage changes from baseline.

Clinical laboratory values (White Blood Cell Count)Intra-operatively, Days 1, 2 and 28

Absolute values and the absolute and percentage changes from baseline.

Clinical laboratory values (Blood Platelet Count)Intra-operatively, Days 1, 2 and 28

Absolute values and the absolute and percentage changes from baseline.

Clinical laboratory values (Blood Sodium, Potassium, Calcium, Magnesium, Chloride)Intra-operatively, Days 1, 2 and 28

Absolute values and the absolute and percentage changes from baseline.

Clinical laboratory values (Blood Urea Nitrogen and Creatinine)Intra-operatively, Days 1, 2 and 28

Absolute values and the absolute and percentage changes from baseline.

Clinical laboratory values (Blood Alkaline Phosphatase, Aspartate Aminotransferase, Alanine Aminotransferase)Intra-operatively, Days 1, 2 and 28

Absolute values and the absolute and percentage changes from baseline.

Clinical laboratory values (Blood Lactate Dehydrogenase)Intra-operatively, Days 1, 2 and 28

Absolute values and the absolute and percentage changes from baseline.

Clinical laboratory values (Blood Activated Clotting Time)Intra-operatively, Days 1, 2 and 28

Absolute values and the absolute and percentage changes from baseline.

Trial Locations

Locations (10)

Children's of Alabama

🇺🇸

Birmingham, Alabama, United States

Children's Hospital of Colorado

🇺🇸

Aurora, Colorado, United States

Heart Center, Ann & Robert H. Lurie Children's Hospital of Chicago

🇺🇸

Chicago, Illinois, United States

Riley Hospital for Children at Indiana University Health

🇺🇸

Indianapolis, Indiana, United States

Cardinal Glennon Children's Hospital

🇺🇸

Saint Louis, Missouri, United States

Duke University Medical Center Surgical Office of Clinical Research (SOCR)

🇺🇸

Durham, North Carolina, United States

Cincinnati Children's Hospital Medical Center

🇺🇸

Cincinnati, Ohio, United States

Nationwide Children's Hospital- The Heart Center

🇺🇸

Columbus, Ohio, United States

Seattle Children's Research Institute

🇺🇸

Seattle, Washington, United States

University of Wisconsin-Madison

🇺🇸

Madison, Wisconsin, United States

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