Friday, September 19, 2014

Research Explained: Sano vs. BT Shunt

Popularity of the National Pediatric Cardiology Quality Improvement Collaborative's (NPC-QIC) Research Explained is growing.  The third installment, brought to you by NPC-QIC's Research Committee, analyzed differences in the Sano and BT shunt used during the Norwood procedure - "Comparison of Shunt Types in the Norwood Procedure for Single-Ventricle Lesions", published in The New England Journal of Medicine in May 2010.

Our families often discuss this same subject, "Sano vs. BT" and are looking forward to your comments regarding this comparison.

Main Finding from this Study

While there has been great improvement in care for patients with hypoplastic left heart syndrome and other similar single ventricle lesions that require the Norwood procedure, these patients are still at great risk. When the Norwood procedure is performed there are 2 different ways blood can be supplied to the lungs. A right ventricular-to-pulmonary artery shunt (RVPAS) is placed directly from the right side of the heart to the pulmonary arteries by making a cut in the heart muscle. A modified Blalock-Taussig shunt (MBTS) is placed from an artery that supplies the head and arm to the pulmonary arteries. Each shunt has advantages and disadvantages. The MBTS may cause less blood to flow to the heart muscle through the coronary arteries, but the RVPAS may not allow the pulmonary arteries to grow well and makes a scar on the muscle of the heart. It was not known if one of these shunts were better than the other.

This study describes a comparison of two groups who were randomly assigned to get one shunt type or the other at the time of their Norwood surgery. The authors of this study concluded that in infants undergoing the Norwood procedure, survival without requiring a heart transplant was better at 1 year of age in those receiving a RVPAS than those receiving a MBTS.  

About this study

Why is this study important? 

This was a very important study because doctors at many different medical centers worked together to find answers to questions that could not be answered by one medical center alone. This is the first time that this type of study was successfully performed in congenital heart surgery, and has helped usher in a new era of cooperation between centers in doing research to improve outcomes in these patients. The number of patients at any single center would not be large enough to be certain that difference are not just based on random events or differences between individual patients, but rather based on an effect of one particular intervention or another.   Statistical calculations may be used to correct for individual differences, but can only be used when the groups of patients studied are large.  Many other studies have come from the information collected in this study because so many different medical centers worked together to contribute information. This study was also the first fair comparison between shunt types because patients were randomly assigned to a type of shunt.  This means that in such a large group, any difference seen between those with the two types of shunts is most likely due to the type of shunt.
In addition, this is a very large group of patients with this heart defect about whom we have a lot of very good baseline information.  It will be very valuable to continue to follow them over time, even for their entire lives. 

How was this study performed?   

This study enrolled patients from 2005-2008 who required the Norwood procedure at 15 medical centers in the United States and Canada. The patients were randomly assigned to one shunt type when they had their Norwood surgery. They were followed after surgery for 12 to 52 months depending on when they entered the study. The main comparison was how many from each group survived to 1 year of age without needing a heart transplant. In addition, they compared how many catheterization procedures each group underwent and the function of the hearts by echocardiogram (echo) for each group. 

What were the results of the research? 

o 275 patients with MBTS were compared to 274 patients with RVPAS.

o At 1 year of age the RVPAS had more patients alive without needing a heart transplant (74%) than the MBTS group (64%).  
  • When they followed the patients they could for longer than 1 year the difference between the groups disappeared.
  • At centers that did a lot of Norwood procedures every year there was no difference between shunt types even at 1 year.
o The group getting the RVPAS underwent more catheterization procedures than the MBTS group, mostly to perform interventions to increase the size of the pulmonary arteries.

o At the end of the study there was no difference between shunt groups in the function of their heart by echo. 
What are the limitations of this study?
This study went on for many years and patients were enrolled at different times so they were followed for different lengths of time. The main comparisons between groups were made when all patients were about 1 year old. This may not be long enough to know which shunt type is better.  Differences at 3 years were also examined and published recently1.  These data confirm that differences seen at 1 year did not continue. The number of patients alive without heart transplant was very similar between the two groups (67% for RVPAS vs. 61% for MBTS), even though cardiac function by echo was a little better in the MBTS group and those in the RVPAS group underwent more catheter procedures.  A further extension of this trial is ongoing to see what happens at 6 years old.

Differences in many features of patients might be studied, but deciding which are important (other than survival without heart transplant) is difficult.  As well, many differences in how patients are treated at different centers can be seen.  But since the patients were randomly sorted into groups for comparison based only on the type of shunt they received and not other aspects of care (such as using a certain medication), conclusions about other aspects of care would be very difficult.  
What are the takeaway messages considering the results and limitations of this study?
For the 1st year of life, survival without heart transplant is better in those who get a Norwood with RVPAS than those who get a MBTS. After that, there is not a clear advantage to have one shunt over another, but as they continue to collect more information over time, new knowledge may be gained. The group that had RVPAS had smaller pulmonary arteries and underwent more catheterization interventions to try to improve the size of their pulmonary arteries.  
1 Newburger JW, Sleeper LA, Frommelt PC, Pearson GD, Mahle WT, Chen S, Dunbar-Masterson C, Mital S, Williams IA, Ghanayem NS, Goldberg CS, Jacobs JP, Krawczeski CD, Lewis AB, Pasquali SK, Pizarro C, Gruber PJ, Atz AM, Khaikin S, Gaynor JW, Ohye RG; Pediatric Heart Network Investigators. Transplantation-free survival and interventions at 3 years in the single ventricle reconstruction trial. Circulation. 2014 May 20;129(20):2013-20.

Many thanks to NPC's Research Committee for continuing to assist parents in understanding research studies that relate to our children with HLHS.

NPC-QIC Research and Publications Committee 2014-2016

Jeff Anderson
, Chair, Cincinnati Children's Hospital Medical Center
Jean Ballweg, Le Bonheur Children's Hospital
Katie Bates, Children's Hospital of Philadelphia
Michael Bingler, Children's Mercy Hospitals and Clinics-Kansas City
Clifford Cua, Nationwide Children's Hospital
Nancy Halnon, Mattel Children's Hospital UCLA
Garick Hill, Children's Hospital Wisconsin
Colleen Melchiorre, Parent
Patrick O'Leary, Mayo Clinic
Sarah Ortiz, Parent
Matt Oster, Children's Healthcare of Atlanta
David Schidlow, Boston Children's Hospital
Julie Slicker, Children's Hospital Wisconsin
Karen Uzark, University of Michigan Congenital Heart Center


Tuesday, August 26, 2014

Parent Travel Scholarships - NPC-QIC 2014 Fall Learning Session!

Registration for the NPC-QIC 2014 Fall Learning Session is now open! Sisters by Heart and NPC-QIC want to send YOU to the Fall Learning Session November 7-8, 2014! Together, Sisters by Heart and NPC-QIC  are offering parent travel scholarships, valued at $500 each, to three parents to attend the learning session in Cincinnati, OH. If you are new to the HLHS world, haven’t heard of NPC-QIC before, or have questions about what NPC-QIC does, we have put together a little information for you.  The National Pediatric Cardiology Quality Improvement Collaborative’s mission is to improve the care and outcomes for children with cardiovascular disease. NPC-QIC’s current quality improvement project is working to improve survival and quality of life for infants with Hypoplastic left heart syndrome (HLHS) during the "interstage" period between discharge from their initial open heart surgery- Stage 1 Norwood - and admission for a Stage 2 biredirectional Glenn procedure. Fifty-five (55) centers from across the country have come together with parents to improve the care for HLHS babies.

Ask any parent who has attended a NPC-QIC learning session about their experience and their reaction will be the same; it is life changing. Natasha Sawyer, Sisters by Heart board member, shared her experience on her personal blog after attending her first learning session in January of 2013:

“The conference was more than I could have ever imagined it would be. I'm not sure what I expected, but it definitely wasn't this. Teams consisting of cardiologists, nurse practitioners, nurses, dieticians, therapists, social workers, and parents filled a large conference room and the excitement began. Over 100 people were in attendance, including 15 parents, representing centers from across the country. From the moment I walked in, I felt welcomed. I felt important. Doctors and team members expressed how thrilled they were to see so many parents in attendance and how crucial we are to the success of the collaborative.

Before the conference, I did not fully comprehend how amazing pediatric cardiologists and their team members are. Sure, I knew it took a special kind of person to work with babies and children with life-threatening heart defects, but until this weekend, I did not see it as more than just their "job". Please bare with me for a few minutes, I am still on an "emotional high" from the past two days and I am desperately trying not to jump up and down and shout for joy while I try my best to describe what I witnessed.. The members of the NPC-QIC have more passion for saving Hypoplastic Left Heart Syndrome babies than you can even begin to imagine. They are not satisfied with their current programs. They are not satisfied with the percentage of babies surviving from the first to second surgery, even though just 30 years ago these babies were being sent home to die. They are spending so much time and energy going above and beyond what is expected of them to change the future of children born like my son. They are changing the quality of life for these kids- kids and families they have yet to meet and have no tie to! I used to think Cardiologists were keeping their ideas to themselves, not wanting to share their "trade secrets" with anyone else. This weekend, I saw how open every team was to sharing their "secrets" with their colleagues. I watched cardiologists and team members from different centers take notes as another shared what is working for their center.

This weekend, I saw not only passion for caring for HLHS kids, but a fire to continue to make advancements and improvements for these children. I am so humbled by this experience, and feel forever indebted to these physicians and care team members who are helping our children continue to defy the odds. I cannot wait to share everything I have learned with my team's collaborative coordinator.” Natasha Sawyer, HLHS Mom,

Parents with a single ventricle child, who required a Norwood or Norwood varient surgery may attend the learning session and/or apply for a travel scholarship.  You do not need to be currently involved with your child's interstage clinic.

Sisters by Heart will be accepting travel scholarship entries until September 1, 2014. In order to be considered, please send an email with "Learning Session Scholarship" in the subject field to with the following information:

HLHS child's name and DOB
HLHS child's hospital
A brief paragraph on why you would like to attend

 If you are interested in attending and can make arrangements for childcare/time off work, please apply! This is such a wonderful opportunity to share your opinions, answer questions from a “professional” perspective as a parent, and most importantly be involved in helping to improve care for HLHS children.

Awarded scholarships will be announced via email by September 8, 2014. 

Please make sure to register for the event using the following link if you will be attending the Fall Learning Session!

We look forward to seeing you in Cincinnati in November!


Friday, August 22, 2014

Research Explained: Can the Left Ventricle Be Taught to Grow?

We're excited to share with the HLHS community, a second installment of "Research Explained" courtesy of the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC).  NPC-QIC's Research and Publication Committee reviewed a study published in November of 2012 - a subject we've covered previously - on Boston Children's Staged Left Ventricular Recruitment (SLVR) for borderline HLHS patients. 

We know many of you are interested in reading more about the SLVR program, and some of our Sisters by Heart families have and are currently undergoing SLVR in Boston. 

Special thanks to Colleen Melchiorre (Mom to Paul, HLHS) and Dr. David Schidlow for their dedication in bringing us the following "Research Explained."

The Journal of the American College of Cardiology published a study in November 2012:  “Staged Left Ventricular Recruitment after Single-Ventricle Palliation in Patients with Borderline Left Heart Hypoplasia.”

The abstract can be found at the following link:

The NPC-QIC Research and Publication Committee reviewed this article, and a summary of the findings can be found below.

Main Finding from this Study:
Most children with hypoplastic left heart syndrome (HLHS) require three surgeries in the beginning of their lives for survival: the Norwood, Glenn and Fontan surgeries.  This result of these surgeries is a heart that only has one pumping chamber (instead of the usual two chambers). For children with HLHS, the right ventricle pumps blood to the body.  The process of these surgeries all together is called “single-ventricle palliation”, or SVP.  (SVP is the term used in the article, but it might be more familiar to many readers as the “Fontan route”.)

SVP is recommended for most children with HLHS.  This is because the left ventricle is too small to help pump blood to the body.  In some children, however, the left ventricle is “borderline”, meaning that it might, with the help of multiple surgeries and other procedures designed to promote growth, be able to do the work of pumping blood to the body. 
The authors of this paper describe an approach at Boston Children’s Hospital for children with borderline hearts.  They show that for certain patients, it is possible to get the left ventricle to grow using a combination of different surgeries and other procedures.   Some of the patients who undergo these surgeries and procedures are able to have a “biventricular” circulation, meaning the left ventricle pumps blood only to the body and the right ventricle pumps blood only to the lungs.  This is more similar to a healthy normal heart.  Perhaps more importantly, this demonstrates that there is the potential for growth of the left ventricle in patients with borderline left heart structures.

About this study:
Why is this study important?

Many children with HLHS who undergo SVP do well.  Unfortunately, however, many babies and children do not survive SVP and others have medical problems related to the heart and other organs. This has prompted investigators to explore the possibility of getting the left ventricle to grow in the hope of keeping the left ventricle as the chamber that pumps to the body (this is called a biventricular circulation). These children could (we don’t know yet) have better survival and fewer medical problems than those that undergo SVP.

The authors of this paper describe ways of getting the left ventricle to grow; they call this “staged left-ventricular recruitment”, or SLVR.  SLVR procedures include surgeries and catheter-based procedures designed to promote blood flow through the left side of the heart.  This includes procedures on the mitral valve, the aortic valve, and within the left ventricle itself. These are all components of the heart that are affected by HLHS, and patients must meet certain qualifications of these components to be a candidate for SLVR.

How was this study performed? 

The researchers looked back at patients diagnosed with borderline heart at Boston Children’s Hospital between 1995 and 2010.  They compared 34 patients with borderline hearts who underwent traditional SVP, and 34 patients who underwent SLVR.    They compared the sizes of different left-sided heart structures.  Specifically, they compared the size of the mitral valve, aortic valve, and left ventricle. 

What were the results of the research?

o   At birth, the sizes of left heart structures were similar in both groups of patients, although the patients in the SLVR group tended to have a slightly larger left ventricle, and patients in the SLVR group were more likely to have had a procedure as a fetus (during the pregnancy) or shortly after birth to open a blocked aortic valve. 

o   Patients in the SLVR group did demonstrate growth in their left-sided heart structures.  Specifically, they had a larger mitral valve, aortic valve, and left ventricle than patients who had SVP.  The most growth was seen after the Glenn surgery.

o   12 of the 34 patients in the SLVR group were able to achieve a biventricular circulation.  18 of the patients had either Glenn or Fontan type hearts, and 1 underwent transplant.  Those with Glenn or Fontan circulation will either continue with SLVR or undergo SVP.

o   Patients who had a traditional SVP typically had 3 surgeries, whereas patients who underwent SLVR typically had 4 surgeries. 

o   Patients who had a small hole created in the top heart chambers to direct blood flow into the left ventricle were more likely to have growth of left-sided heart structures. 

o   There were 3 deaths (8%) in the SLVR group and 7 deaths (20%) in the SVP group.  Although this difference was noted, the number of patients in the study overall was too small to know if that difference was due to the way they were treated. 

What are the limitations of this study?

o   The study looked at a small numbers of patients.  This limits the ability to draw conclusions that can be applied to the general population.  Borderline patients encompass a wide range of patients with many different sizes of the left side of the heart. 

o   There may be aspects of the hearts in the SLVR that are different from the SVP group.  Patients in the SLVR group were more likely to have a procedure as a fetus or newborn to relieve blockage of the aortic valve.  This may mean that there are differences between the two groups despite the size of their left hearts being similar.

o   This study does not describe how the SVP and SLVR patients are doing clinically, either in the short or long term.  

o   Important questions that could be addressed in the future include:  

 §  What does the future hold for promoting the left side of the heart to grow?

 §  How are SLVR patients doing clinically, and what are their long-term outcomes? How do they compare to the SVP patients? 

 §  When is the best time to undergo SLVR?

What are the takeaway messages considering the results and limitations of this study?
o   This study shows promise for promoting growth of the left ventricle in patients with a borderline heart.  Future study is required to explore the best way to achieve that growth, but this study offers hope. 

o   In order to undergo SLVR, children must meet certain qualifications. It is important to realize that SVP is recommended for the most children with HLHS.  In a select group of patients with HLHS—those with a borderline heart—surgeries and other procedures to encourage the left heart structures to grow are possible.  Among such patients, some, but not all, will achieve a biventricular circulation.   

o   SLVR includes a variety of surgeries and catheter-based procedures on different parts of the left heart (the mitral valve, aortic valve, and ventricle), and most patients who undergo SLVR will have a Norwood and Glenn procedure prior to achieving a biventricular circulation. 

o   Compared with patients who undergo SVP, patients who undergo SLVR have larger left heart structures, and 12 out of 34 patients who underwent SLVR were able to achieve a biventricular circulation.     

o   More studies are required to understand the best way to promote left heart growth and to understand the short and long-term quality of life for patients with biventricular circulation.

The Bottom Line:

Doctors at Boston Children’s Hospital are working to grow the left side of the heart for some children with HLHS. These procedures are still being studied, but they seem to help the left heart grow for some children. In order for your child to qualify, he or she must meet certain criteria.   You may wish to talk to your cardiologist about SLVR, and whether your child may be right for it.  Dr. Emani (the author of the article reviewed here) can also be contacted at


Monday, August 18, 2014

CHD Kids and School Special Services

Summer is almost over and many HLHS children are headed to school - for the first time or as returning students. Trent Hamilton, HLHS dad and Middle School Principal, generously shared his wealth of information with Sisters by Heart on educational benefits and services for CHD children in the public school system. Thank you, Trent, for sharing this information with our families! 

Several people in our CHD support group have recently had questions about getting special services for the kids who are starting school this year. Specifically, we have been discussing the differences between Special Education services through the Individuals with Disabilities Education Act (IDEA) and Section 504 of the Rehabilitation Act of 1973 (Section 504), and which would be appropriate for their CHD child. As a school principal, I deal with both Section 504 and IDEA students on a daily basis. I hope that the following blog will help you make the right decisions to provide assistance and protection to your CHD child as they start the new school year.

Basically, the two laws are similar in that they both provide educational benefits to kids with disabilities, but they differ in their eligibility requirements and the types of benefits that are provided. The requirements for eligibility under IDEA are stricter than those under Section 504, but IDEA provides more benefits than does Section 504. Essentially, all students under IDEA are also protected under Section 504, but not all students under Section 504 are protected by IDEA. It is easier to place a student under Section 504 because it has less procedural criteria, but these students are generally provided less assistance and monitoring by the school. IDEA status is more difficult to get because there are more specific criteria and it has much stricter regulation, but it does provide more types and degrees of assistance and special services to the student. Section 504 protects people for their lifespan in many areas, including school, employment, and transportation, while IDEA only addresses the special education of students from ages 3 to 21.

Students are eligible for IDEA if they have a specific impairment or condition that adversely affects their educational performance. These impairments/conditions can include, but are not limited to: autism, learning disabilities, speech/hearing impairments, emotional disturbance, or other health impairments, but the condition MUST adversely affect their education. Students are eligible for Section 504 if they have a condition that substantially limits a major life activity, even if the condition does not affect their education. Major life activities include: walking, seeing, speaking, breathing, learning, and working. But Section 504 conditions do NOT have to have an educational affect.

IDEA requires that students be placed in the most appropriate learning environment for them individually. This can include regular or special education classes. It also requires that each student has an individualized education program (IEP), which provides specific modifications and accommodations to help the student succeed academically. Section 504 generally places students in a setting that is comparable to that of students who are not disabled. It also provides some accommodations, but these are usually used in the regular educational setting.

So, which type of services is right for your child? This depends on the level that your child’s CHD has affected them and their development. If your child’s CHD caused them to have any type of developmental or learning disability, then IDEA is probably the right program. If your child’s CHD slows them down physically, but has not affected their learning or development, then Section 504 is probably best. There are obviously exceptions to these, so be sure to consult your child’s school if you aren’t sure which is right for you.

You might be wondering why you need any type of service at all if your child doesn’t have any learning or developmental issues. As a principal and a CHD parent, I highly recommend that you pursue some sort of protection for your child at school. While your child may not need accommodations or special services in order to succeed academically, they may need some of the other protections that are provided under Section 504. One of these protections involves attendance. Many states require that students attend 90% of the available school days and, even if an absence is for approved medical reasons, a student can be retained for excessive absences. If a student has protection under Section 504, special accommodations can be made in the event of an extended illness or hospitalization. Also, your child may not experience severe educational deficits early in their school years, but if they do later in life, having a 504 plan can make it easier to get accommodations or even move to IDEA protection. Section 504 also makes it easier to get certain exemptions from activities, like running, lifting weights, etc. In addition to all of this, the Section 504 plan follows your child through school as a record and every new teacher is required to review it during the school year. This means that each new teacher will be notified of your child’s condition and know what your child needs.

I hope that this information helps you in making the right educational decisions for your CHD child. If you still have questions, I encourage you to speak to your school’s counselor or special education diagnostician. Again, your CHD child does not have to have either one of these services, but I assure you that my son (HLHS) will, at least, have a Section 504 plan.

Trent Hamilton

Heart Dad & Middle School Principal


Monday, August 11, 2014

Using Crisis to Create: The Asher James Foundation

In June, Sisters by Heart President, Stacey Lihn, presented at the Mended Little Hearts' National CHD Symposium.  Following, she was able to meet and talk with HLHS parents across the country.  Jen DeBouver is one of many parents that inspired Stacey, and we've asked Jen to share her journey and advocacy efforts - to educate parents on the risks of blood clots in our CHD community and how you might be able to help.

The Asher James Foundation
by Jennifer DeBouver

When I was pregnant with my son, my second child, I thought nothing could go wrong – not this time. I had just lost my daughter Olivia at 27 weeks. She no longer had a heartbeat the doctor told us. When it came around 26 weeks of pregnancy with Asher, I was a nervous wreck. My husband and I were at a 26 week check-up when the ultrasound tech left to get the doctor. I couldn’t believe something was happening again around the same time as Olivia. When the doctor returned, he told us that he wanted us to get a second opinion on Asher’s heart because he saw something but wanted to make sure.

We had an echo done and found out that Asher had Aortic Stenosis. The doctor gave us two options – wait until he was born in which his heart defect would develop into HLHS or have a fetal intervention. As a parent we wanted to do the best possible thing for our baby so we decided to do the fetal intervention. A week later we flew from Chicago to Houston to have the fetal intervention.

When we got to Texas Children’s, it was a full day of testing to see if we were a candidate for the fetal intervention. This is when we learned that in a week, Asher’s heart had gotten worse. He had developed coarctation of the arch and had leaks in his mitral and tricuspid valve. The cardiologist started crying and told us that our baby had no idea that he was sick. We met with the entire team and one of the doctors told us that without the fetal intervention, Asher wouldn’t make it to birth and that it was a Hail Mary that he would make it through the procedure. Asher was born on September 10, 2012, at 36 weeks – 9 weeks after the fetal intervention. After we had a very successful fetal intervention, we started calling Asher our little Fighter. Hours after being born, he had to have a cath procedure to re-inflate the balloon that was inserted during the fetal intervention. At 11 days old, he underwent his first surgery to fix his aortic arch.

Asher rocked every procedure and was doing amazing. But at 4 weeks old all that changed. One day Asher looked amazing and then the next he started looking red and puffy and continued getting puffier from there. After 2 days of being swollen, we found out that blood clots had developed in his jugular veins and arms, restricting blood flow from his head to his heart. The hematologist tried different medications on him, some seemed to work and some didn’t. She told us that they were using scaled down adult medications because there aren’t any medications designed for children. She also told us that there weren’t any foundations supporting blood clots in children.

Asher lived for 6 weeks before he lost his battle to these blood clots on October 23, 2012. After everything that his hematologist told us, I knew that I wanted to continue Asher’s fight by starting a foundation in his memory for children’s blood clots. So a few months later, my husband and I started the Asher James Congenital Heart Disease & Thrombosis Foundation. We wanted to let others know that their children were at risk for clots when hospitalized because we had no idea this could happen until it happened to us.

This year we held our first Children’s Blood Clot Awareness Day at Ann & Robert H. Lurie Children’s Hospital of Chicago to inform families and staff of blood clot awareness. We spoke on Capitol Hill to both Congress and the Senate on children’s blood clots and shared Asher’s story.

In the Fall, we are holding our first blood clot awareness walk. We know that Asher is still with us and is making a difference in children’s lives and we continue his fight through awareness, and one day, research in children’s blood clots. Find out more about our foundation at

Thank you, Jennifer, for sharing Asher's story and your courageous fight to raise awareness and answers for both the CHD community and all children at risk for thrombosis.  Feel free to "like" the Asher James Foundation on Facebook at and show Jen your support by commenting on the Foundation's page and/or sharing your story about how thrombosis/blood clots affected your CHD journey. 


Wednesday, July 2, 2014

HLHS Collaborative: June's Action Period Call

The Action Period call started as usual, with the typical data review.  For those who have allowed your center to share data with the Collaborative, it is collected for research and quality improvement.  To date there are 1304 patients in the registry.  Despite the typical data review, the focus was on the mortality rate because decreasing mortality is one of the primary goals.  Prior to the inception of the Collaborative in 2008, interstage mortality rates in the medical literature were around 10-15%.  The NPCQIC mortality rate at times seemed to hover around 10%.  However, when looking at all the data, it shows that the mortality from 2008 to 2014 is 8.1%.  Then came the big news, when looking at a more recent period, from January 2013 to May 2014 the Collaborative interstage mortality rate was 6%!  This was the first time that the Collaborative was able to statistically show a reduction in mortality.  The thought is that by working together, the involved centers were able to improve care processes that impact care.  There was excitement and celebration, but also a humbleness that 6% is still not good enough.  This is only the mortality rate for infants from Norwood discharge through the interstage period to Glenn surgery.  Moving forward there will be a continued focus on this interstage time period but also working with clinicians who care for these infants during this earlier time frame to find additional ways to decrease mortality.  It is always encouraging to see practitioners get excited about accomplishments and then get right back to work to find more ways to improve, especially when it directly impacts the care of your own child. 

 After the big news the focus shifted to a second main goal of the Collaborative, caloric intake.  The discussion revolved around how centers document caloric intake and weight gain for all infants they see.  Representatives from Advocate Children’s, Primary Children’s, and LeBonheur Children’s all shared ways that they track caloric intake and weight gain as well as who is responsible for doing so.  Advocate Children’s tracks it like other vital signs like blood pressure and heart rate to give an example of the importance this has. 

 The call ended with a number of announcements.  Nemours A.I. DuPont Hospital for Children in Wilmington, Delaware is the latest addition to the Collaborative bringing the total number of centers to 55.  The fall Learning Session will be on November 7 & 8 in Cincinnati.  The final announcement was that the Parent Page on the NPC-QIC website is under construction to help improve the resources available for parents.  After that all signed off to get back to improving care with a heartfelt thanks from all of the parents!


Monday, June 23, 2014

Pink for Paul!

From time to time, we here at Sisters by Heart are approached with fundraising ideas, ways to generate awareness about HLHS and support Sisters by Heart at the same time. We are always so touched and honored when heart moms choose to support our efforts. We were recently approached with a new fundraising idea, borne out of one mother's unique way of showing love for her special HLHS son, Paul. We were so touched by her story, and thrilled to participate in this fundraiser, Pink 4 Paul. This is Paul's story, as told by his mother, Colleen. Please be inspired along with us, and read on to hear how you can support Sisters by Heart and go Pink 4 Paul this summer.

No one teaches us how to grieve. There are certain theories, support groups, quotes and passages that are intended to guide us and for some they provide solace and a road map and for others they provide simple words of encouragement. I think society has a need for people to hurry up and get better in the face of all different types of issues. People have a tendency to rush to support folks in immediate crisis- think of all the casseroles the day of a funeral but in aftermath, the days and weeks and months that follow, we tend to expect people to move on with their lives and resume normal functioning. After getting an HLHS diagnosis I was supposed to go through the Norwood (hard) get through the Glenn (easy) and then resume normal functioning until the Fontan. I wish I was one of those people who immediately became better and gained amazing life perspective in the face of life-threatening illness but I was not- I am not. That does not mean that who I am at my core and what I value deeply has changed it just means that in spite of myself I can get really worked up when it comes to HLHS.

For me, things went like this: I was happy, in denial and a little bit information crazy when Paul was first born. A few months in I was mad. His Glenn was supposed to be easy and it wasn’t. I was supposed to feel better about everything and I wasn’t- I was feeling…WORSE. More sad, more anxious, more in the weight of this diagnosis than ever before. I was sort of in a fog with little glimpses of hope during Paul’s first year of life. I checked his oxygen saturations constantly, we went to the doctor all the time, I researched vigorously for a cure, for someone- something to make it better. I got irrationally mad at our doctors and had moments of total denial. Looking back I can see this was all about grief- RAW grief. My little boy has a very different type of heart and there is no denying it. A shift happened in our family that caused my husband to be home with the kids much more. He was running the ship with me and he did it very differently that I had.

My husband is the coolest guy I know and has an amazing calm to him. He has the ability to compartmentalize in a healthy way like no one else I have ever known. That’s a good thing since I have almost every feeling all the time  He taught me in the last year to see Paul as a little boy with a special heart, not as a ticking time bomb. He helped me see the forest for the trees so to speak. He helped me get through the fog in a way where the moments of joy started to over shadow the moments of panic. He didn’t sit me down and say “Colleen you should be feeling differently.” He just SHOWED me that parenting Paul was about parenting a little boy mostly and a heart kid sometimes.

Grief…What a word. Grief is an emotion that I will never lose in the face of HLHS but it is an emotion I have learned to manage. Here’s what I know right now- we have a beautiful two and half year old that can count to ten, knows most of his letters, is highly opinionated and really likes buddy fruit. We welcomed a little girl, Georgia Jane, on Valentines Day of 2014 and she is the queen of our little castle. Her heart is not perfect either, by the way. She has bicuspid aortic valve a very minor CHD but common for HLHS siblings (talk about re-visited grief but that’s a whole other blog). She is a sweet little ginger and Paul LOVES her. I fall more in love with my husband every single day. Our marriage is surviving and thriving in the face of this grief. It hasn’t been easy but we are making it, together. I have some amazing life long friendships because of HLHS and get to serve so many families by sitting with them in their pain and their grief when its needed. I am still who I was before HLHS. I have a huge crush on Gavin Degraw, get to work doing research for a lovely and highly patient company, I love to sing and dance, and I get an unhealthy joy from starbucks latt├ęs and am a sucker for Lifetime television.

I wrote this blog about two years ago and I still mean every single word. The HLHS community is a beautiful place to unintentionally bloom. It has been a gift and gorgeous blessing to know these children and families. I love them as my own and follow their journeys with a lot of hope.

One of my best friends in the world is named Diana. Her little boy Jude is the first HLHS child I ever met. When I was pregnant with Paul, Diana and Jude and I met for coffee and conversation. I thought she’d arrive with a baby in a wheelchair on oxygen and IV drip; instead, she arrived with a big cheeked tow head with quite the personality. I fell in love with him and her that day and have been privileged to love them everyday since.

Most of you know that HLHS children appear “blue” pre-Fontan, especially in their fingertips and toenails. Diana shared an idea with me to paint my toenails blue when I was pregnant with Paul. She had done it for Jude, citing that as long as his toes were blue, hers would be too. At that moment, before he was born, I made a promise to Paul that as long as his toes were blue, mine would be blue and I have kept that promise for the last 2 and a half years, painting my toenails with every blue polish there is.

Paul will have his Fontan surgery on July 28, 2014 and hopefully his fingernails and toenails will be more pink. To show support and to raise awareness for congenital heart disease, we are asking folks to go Pink 4 Paul!

Paint your toes and/or nails pink. Post your picture on our facebook page or Instagram #pink4paul and share with a caption of support. We will read all the messages to him during surgery.

You can purchase pink specific nail wraps and polish through our fundraiser with Jamberry Nails Independent Consultant Jenn W, between June 23rd and July 6th at We have also made custom wraps that you can purchase by emailing!! All proceeds will go to Sisters by Heart!
Custom Sisters by Heart Nail Wraps
This is the best way I know to place my grief. In the face of this upcoming surgery, I am trying to choose acceptance and synthesis as a means to get through this time. During the Fontan, I expect to grieve, freak out, yell, ugly cry, laugh and smile, and maybe I will enter the fog again. As sure as the sun will rise, I will get out of that fog-no matter what happens, no matter how long it takes, I will live and breathe again. I will not be paralyzed by grief forever.

I write often about the space between in life, the power of each other, and the importance of love without condition. Maybe this is my lesson…these are my lessons in this journey. Whatever your process, wherever you are, however you grieve it's ok and it will BE ok. Thanks for fighting for HLHS and for allowing me to be with you on your journey, and you on ours as we sit with grief; we have so much hope. Paul is proof that the quality of life for a child with HLHS can be amazing and I hope he continues to be that proof throughout the rest of his long long long life.

Dr Colleen Melchiorre is a researcher and retention specialist for Phired Up Productions, a speaker for active minds, and a Linked by Heart and NPCQIC research committee volunteer. Her most important purpose in life is being a wife and mother. Her families' HLHS journey is chronicled at