The Single Ventricle Reconstruction (SVR) Trial looked at how infants with single
ventricle heart defects did after the first stage of surgery (Norwood operation). Infants
enrolled in the trial got one of two kinds of shunts during the Norwood; a modified
Blalock-Taussig shunt (MBTS) or right ventricle to pulmonary artery shunt (RV-to-PA
shunt).

The purpose of this study is to learn if children (2-6 years of age) who were enrolled in
the SVR Trial do better with one of the two shunts (MBTS or RV-to-PA) in the years after
surgery.

Single Ventricle Reconstruction Extension Study (SVR II) - Pediatric Heart Network

Heart Defects

Among critical congenital heart lesions, hypoplastic left heart syndrome (HLHS) and
related single right ventricle (RV) anomalies are associated with the highest morbidity
and mortality. The first stage in palliation for patients with these defects is the
Norwood procedure. The essential components of the Norwood procedure include (1) an
atrial septectomy, (2) anastomosis of the proximal pulmonary artery to the aorta with
homograft augmentation of the aortic arch, and (3) establishment of a source of pulmonary
blood flow, with either a modified Blalock-Taussig shunt (MBTS) or the right ventricle to
pulmonary artery (RV-to-PA) shunt. In May 2005, the Pediatric Heart Network began a
multi-center, randomized trial, the Single Ventricle Reconstruction (SVR) Trial,
comparing outcomes in subjects with HLHS or other single RV anomalies palliated using the
Norwood procedure with either a MBTS or the RV-to-PA shunt. The primary outcome of this
trial was freedom from death or cardiac transplantation by 12 months post-randomization.

The Single Ventricle Reconstruction Extension (SVR II) Study is an extension of the SVR
Trial that compares clinical outcomes and RV performance following the RV-to-PA shunt
versus MBTS modifications of the Norwood procedure in subjects at 6 years
post-randomization. Data is collected annually between ages 2 and 6 years, as well as
before and after the Fontan surgery. Vital status and medical history is ascertained
annually until the last enrolled subject is 6 years old. Data is obtained through medical
record review, phone interview with the parent or guardian, electrocardiography (ECG),
core laboratory analysis of echocardiographic images and Holter monitors, and completion
of questionnaires regarding neurodevelopmental outcomes, behavior, health-related quality
of life, and family functioning. The primary outcome variable in SVR II will be freedom
from death or cardiac transplantation at 6 years post-randomization. All subjects who
were randomized in the SVR trial will be included in analysis of this endpoint.

Single Ventricle Reconstruction Extension Study (A Study Conducted by the Pediatric Heart Network)

-  RV ejection fraction by echocardiogram pre-Fontan and at 6 years.

  -  RV fractional area change pre-Fontan and at 6 years post-randomization.

  -  Severity of tricuspid regurgitation by echocardiogram pre-Fontan and at 6 years.

  -  Heart Failure Class pre-Fontan and at 3, 4, 5, and 6 years.

  -  Somatic growth pre-Fontan and annually from ages 2-6 years.

-  Diagnosis of ventricular arrhythmias by 6 years.

  -  Diagnosis of atrial arrhythmias by 6 years.

-  Behavior Assessment System for Children, Second Edition (BASC-2)

  -  Vineland Adaptive Behavior Scales

-  Pediatric Quality of Life Inventory (PedsQL)

  -  Children's Health Questionnaire Parent Form 50-Item (CHQ-PF50) Physical and
     Psychosocial Function Summary

Impact on Family Scale Functional States II-R

Single Ventricle Reconstruction Extension Study (SVR II) - Pediatric Heart Network

About this trial

Study ID

Status

Type

Placebo

Accepting

Not accepting

Trial Timing

What is this trial about?

What are the participation requirements?