Pediatric Heart Network
Congenital and acquired heart diseases occur frequently in infants, children, and young adults and are a major cause of death and long term disability. In the past 25 years, fewer than 40 randomized clinical trials have been performed in pediatric patients with congenital or acquired heart disease. Reasons for this include:
- Many cardiac care providers only see a limited number of children with any one type of heart disease
- Parents are not aware of available research studies, either because they do not live near a research site or have not been approached about the benefits of being in a research study
The Pediatric Heart Network (PHN) is a group of eight hospitals in the United States and Canada that conduct research studies in infants, children and young adults with heart disease. The PHN was created and funded in 2001 by the National Heart, Lung and Blood Institute to promote the efficient evaluation of novel treatment methods and
management strategies having potential benefit for pediatric patients with structural congenital heart disease, inflammatory heart disease, heart muscle disease, and arrhythmias. Cincinnati Children's Hospital is one of eight centers with experienced research teams that specialize in the care of patients with heart disease chosen to become part of the PHN. All centers do the same studies. This helps to increase the total number of patients in each study. More information about the Pediatric Heart Network, including information about research and about the current sponsored studies can be found at www.pediatricheartnetwork.com.
Cincinnati Children's Hospital is currently involved in 3 active studies sponsored by the Pediatric Heart Network:
Single Ventricle Reconstruction (SVR) Trial
The purpose of the SVR trial is to determine the best way to perform the initial surgery for hypoplastic left heart syndrome (HLHS), or heart defects similar to HLHS. In this congenital heart disease, the main pumping chamber of the heart (the left ventricle) and the main blood vessel that carries blood from the heart to the body (the aorta) do not develop properly and are too small to pump blood to the body. Instead, with the Norwood operation, the pumping chamber that usually pumps blood to the lungs (the right ventricle) can be used to pump blood to the body. This operation is done shortly after a child with HLHS is born.
Currently, there are two ways to perform the Norwood operation. At this point, we do not know which one results in better survival or better long term results (such as heart function over many years or long term brain development). Infants enrolled in this study will undergo the Norwood operation using one of the two modifications and will be followed over a 14 month period. Infants will undergo developmental testing in addition to their routine follow-up.
Contact Us About the Single Ventricle Reconstruction (SVR) Trial
To learn more about this study, you may contact our Research Nurse Coordinators, Lois Bogenschutz and Teresa Barnard or the SVR Trial Site Principal Investigator, Catherine Dent, MD, at 513-636-3891.
Infant Single Ventricle (ISV) Trial
The ISV trial is a trial of angiotensin converting enzyme (ACE) inhibition in infants with a single pumping chamber (ventricle). This study is being done to see if giving a medication called Enalapril (an ACE inhibitor) will improve growth and heart function in the first year of life, in infants who only have a single pumping chamber. This study should help provide valuable information to cardiologists caring for these infants. Infants enrolled in this study will receive either Enalapril or a placebo and will be followed over a 14 month period. Infants will undergo developmental testing in addition to their routine follow-up.
Contact Us About the Infant Single Ventricle (ISV) Trial
To learn more about this study, you may contact our Research Nurse Coordinators, Lois Bogenschutz and Teresa Barnard or the ISV Trial Site Principal Investigator, William Border, MBChB, at 513-636-3891.
Marfan Trial
The Marfan trial will compare the effect of beta blocker therapy (atenolol) to that of angiotensin II receptor blocker therapy (losartan) on the rates of aortic growth and progression of aortic regurgitation in patients with Marfan Syndrome (MFS). MFS is a systemic disorder of connective tissue due to mutation in the Fibrillin-1 gene. Cardiovascular pathology, including aortic root dilation, dissection and rupture is the leading cause of mortality in patients with MFS. Several studies have shown that beta blockers reduce the rate of aortic growth, but these effects have been small. Recent studies in a Fibrillin 1-deficient mouse model of MFS with a susceptibility to aortic dilation and dissection similar to that seen in humans with MFS demonstrated that treatment with losartan normalized aortic root growth and architecture, preventing aortic aneurysms and premature death Preliminary data from a small pilot trial suggests potential benefit of losartan use in humans with MFS.
Contact Us About the Marfan Trial
For more information about the Marfan trial, contact our Research Nurse Coordinators, Lois Bogenschutz and Teresa Barnard or Dr. William Border, PHN Marfan Trial site Principal Investigator at 513-636-3891.
