Exclusive Human Milk Feeding in Infants With Single Ventricle Physiology

This is a single blinded (physician investigator), randomized, controlled trial to evaluate
growth velocity and clinical outcomes in infants with single ventricle physiology fed an
exclusive human milk diet during their initial hospitalization after birth and through the 30
days post-surgical repair feeding period or hospital discharge, whichever comes first.

Subjects will be randomized to one of two groups at birth or immediately following diagnosis
if prenatal care was not obtained prior to birth. Parents who decline participation by their
infants in the study will be asked to consent to data gathering on their infants who will be
treated and fed per institutional practice. The data on these individuals will be summarized
and evaluated descriptively in comparison with the actual trial results.

All patients will receive exclusive maternal human milk or donor human milk prior to
randomization. Once randomized, patients in Group One will receive an exclusive human milk
diet prior to the surgery and throughout the 30-day feeding period following surgical repair
or until hospital discharge, whichever comes first. Day 1 is defined as the day of the first
enteral feed post-surgery. Patients in Group Two (Control Group) will receive maternal human
milk or formula or donor human milk (per standard of care at each hospital) in the
pre-surgical period. In the post-surgical period the control group will receive human milk or
formula, as per feeding algorithm The primary objective of this study is to evaluate growth
velocity (weight velocity [g/kg/day] and weight z-score from World Health Organization (WHO)
growth charts) at 30 days after the initiation of feed post-surgery for infants with single
ventricle physiology who are fed an exclusive human milk diet from birth through the 30 day
feeding period following surgical repair or until hospital discharge, whichever comes first.

Inclusion Criteria:

1. Term infants (≥37 and 0/7 weeks gestational age) ≤ 7 days old with a diagnosis of
single ventricle physiology who are thought to require a single ventricle repair at
the time of enrollment.

2. Infant feeding was NPO or consisted of 100% human milk diet prior to enrollment

3. Parent(s) willing to sign informed consent.

4. Parent(s) willing to comply with study follow-up procedures.

5. Require surgical palliation within the first 1 month of life.

Exclusion Criteria:

1. Term infants >7 days old at the time of diagnosis.

2. <37 weeks gestation

3. Infants requiring cardio-pulmonary resuscitation prior to surgical repair.

4. Outborn infants who received enteral nutrition at the other institution prior to
surgical repair. If it is uncertain if infant received even 1 bottle or a small amount
of formula, infants will be excluded.

5. Major congenital abnormalities that could significantly affect survival such as:

1. Confirmed or suspected major genetic abnormalities (lethal or with extremely low
probability for survival).

2. Chromosomal abnormalities: Trisomies (13, 18, 21 etc.) deletions or
translocations (Turner/Williams Syndrome, DiGeorge, to name a few)

3. Major organ system abnormalities not related to a genetic syndrome that are
lethal or have extremely low probability for survival (i.e, bilateral kidney
intrinsic disease, pulmonary hypoplasia, Central Nervous System (CNS)
malformations: Arnold Chiari, myelomeningoceles, hydranencephaly, schizencephaly,
holoprosencephaly))

4. Heterotaxia

5. Metabolic disorders affecting growth: homocystinuria, methylmalonic acidemias,
propionic acidemias, urea cycle defects

6. Evidence of intracerebral hemorrhage (IVH) ≥ Grade 3

7. Any comorbidity or significant clinical event prior to enrollment, deemed by the
Investigator as likely to affect survival.

8. Requires Extracorporeal Membrane Oxygenation (ECMO) pre-operatively

9. Legally Authorized Representative(s) unwilling to comply with an exclusive human milk
diet either in the form of mother's milk, human milk-based human milk fortifier, human
milk based caloric fortifier or donor human milk during the initial hospitalization
period and through the 30 day feeding period after surgical repair or hospital
discharge, whichever comes first.