Comparison of Two Types of Shunts in Infants With Single Ventricle Defect Undergoing Staged Reconstruction--Pediatric Heart Network

BACKGROUND:

Hypoplastic left heart syndrome (HLHS) and related single right ventricle anomalies are the
highest risk congenital cardiovascular malformations. Surgical repair begins with the
Norwood procedure during the newborn period, a stage II procedure at 4 to 6 months of age,
and Fontan procedure at 18 to 36 months. The Norwood procedure remains one of the highest
risk procedures in congenital heart surgery. A few small nonrandomized studies of a novel
approach to the Norwood procedure have reported improved outcomes. This new approach uses a
RV-to-PA shunt to provide pulmonary blood flow rather than the standard MBTS. This
multi-center, randomized clinical trial will evaluate early and intermediate-term outcomes
for patients undergoing a Norwood procedure with either the RV-to-PA shunt or the MBTS.

This study has been approved by the Institutional Review/Research Ethics Boards of all
participating clinical centers:

Hospital for Sick Children, Toronto, Canada

Children's Hospital Boston, Boston, MA

Columbia College of Physicians and Surgeons, New York, NY

Children's Hospital of Philadelphia, Philadelphia, PA

Duke University Medical Center, Durham, NC

Brody School of Medicine at East Carolina University, Greenville, NC

Wake Forest Baptist Medical Center, Winston Salem, NC

Medical University of South Carolina, Charleston, SC

Children's Hospital of Wisconsin, Milwaukee, WI

University of Michigan, Ann Arbor, MI

Cincinnati Children's Hospital Medical Center, Cincinnati, OH

Children's Hospital of Los Angeles, Los Angeles, CA

Egleston Children's Hospital, Emory University, Atlanta, GA

Congenital Heart Institute of Florida, University of South Florida, St. Petersburg, FL

Alfred I. duPont Hospital for Children, Wilmington, DE

DESIGN NARRATIVE:

This is a prospective, randomized clinical trial of the RV-to-PA shunt versus MBTS in
patients undergoing a Norwood procedure.

Inclusion Criteria:

- Diagnosis of hypoplastic left heart syndrome or related single, morphologic right
ventricle anomaly

- Planned Norwood procedure

- Informed consent of parent(s) or legal guardian

Exclusion Criteria:

- Single, morphologic left ventricle anomaly

- Preoperative identification of anatomy rendering either an MBTS or an RV-to-PA shunt
technically impossible

- Any major congenital abnormality (i.e., congenital diaphragmatic hernia,
tracheoesophageal fistula) or acquired extra-cardiac disorder (e.g., meconium
aspiration with need for high frequency ventilation, persistent renal failure
requiring dialysis) that, in the opinion of the investigator, could independently
affect the likelihood of the subject meeting the primary endpoint