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


Friday, June 13, 2014

Why There are No "Brothers by Heart"

The following post is brought to you by... two Heart Dads!  Perfect timing for Father's Day. 

A huge thanks to Travis Lewis and Trent Neely for their candid and honest thoughts and perspectives on being a heart dad and, as the title states, "Why There are no 'Brothers by Heart.'"  So, who are Travis and Trent?

(Trent, Avery, and Ellery)
Trent Neely
Daughter:  Avery (HLHS) - almost 3, Ellery (HH) - 5
Fontan:  Scheduled for early July
Occupation:  Teacher (Anatomy & Physiology, Health & PE)
Favorite kid activity:  Being the tickle monster
Favorite ice cream flavor to share:  Cookies & Cream
Lives in:  Columbus, OH
Hospital:  Nationwide Children's 
Currently reading:  The Tale of the Dueling Neurosurgeons
Favorite kids movie:  The Goonies
Coping mechanism:  Bad jokes
NFL Team:  Bengals

(Travis and Cael)
Travis Lewis
Son: Cael ( HLHS) - just turned not so terrible two 
Fontan:  Scheduled for early July
Occupation:  Physician Assistant and Athletic Trainer in a pediatric office 
Favorite kid activity:  playing outside 
Favorite ice cream flavor to share:  Moose Tracks 
Lives in:  Butler, PA ( just north of Pittsburgh) 
Hospital:  Children's Hospital of Pittsburgh
Currently reading:  Any Given Monday 
Favorite Kids Movie: Charlie Brown Christmas
Coping mechanism:  Coffee
NFL Team:  Steelers

Why There are No Brothers by Heart
by Travis Lewis and Trent Neely

Calling all dads… Calling all dads… Does anybody else hear crickets?  It didn’t take long for us to notice a particular phenomenon in the heart community.  Now we dads aren’t the most observant creatures in the world (unless we’re talking about fantasy football or baseball stats), so the observation had to be pretty obvious.  Sisters by Heart has hundreds of affiliated moms, there are Heart Mom Facebook groups with 1,000+ female members, parent groups are predominately women, and a recent study about how to present a prenatal diagnosis had a 95% survey completion by the fairer sex.  So, where are all the dads?  And why is there not a support group like Sisters by Heart for Dads?

In order to understand this occurrence, it is important to first understand a few key differences between women and men.  In this case, evolution is necessary to provide some insight.  Historically, women have been the primary caregivers for children, and raising children is no easy task.  As caregivers, socializing and bonding became an important survival technique; just ask any mom experiencing the terrible twos.  Sometimes it feels as if a small army is required to attend to a child’s needs, and for help, many women turn to other moms.  On the other hand, men are responsible for providing security and a source of nourishment.  A group of men discussing their latest cave improvement techniques while hunting only tended to scare off the large animals they were hoping to bring home for dinner that night.  As a result, we men tend to internalize more often. 

This tendency of women to “tend and befriend” and men to “provide and protect” has resulted in variations in brain development of both men and woman.  These variations can be coupled with the fact that women carry and then deliver a baby nine months later, while the male’s “job” could be a mere couple of minutes.  In addition, males and females are socialized differently.  Women are taught from a young age to be caring and compassionate and males are taught to be tough and emotionless.  On the surface, we, the tougher species, appear to be devoid of emotions. However, this is hardly the case.  We just deal with our emotions differently.  Recent studies have shown that a Congenital Heart Disease diagnosis may hit the mom harder initially, but the stress tends to linger longer with dad.  Because of their ability to socialize and bond, moms come to terms with the diagnosis faster and then shift their attention to how to care for a fragile infant.  Dads unfortunately, do not typically reach out for help in the same way and often internalize the stress. As a result, the stress generally does not dissipate on it is own and may manifest itself in different forms for heart dads.

Another reason for the invisible nature of dad, is a result of one of the greatest powers in the universe.  We all are exposed to it from birth, but we cannot fully appreciate it until we have children of our own.  This super power is “maternal instinct” and it turns a regular, ordinary woman into SUPERMOM.  We knew it as a child from our own mothers, but it isn’t until our wives or girlfriends step out of their hospital shaped phone booths with a baby that we truly begin appreciate this power.  Seemingly regular women are rapidly transformed into an unstoppable force that has supersonic hearing, can soothe a crying infant, and change diapers with a single hand; all while sleep deprived.  Regrettably, this metamorphosis can turn a new dad into a bubbling idiot only capable of putting baby formula into the coffee maker.  This dynamic shift is rapid and extreme, frequently leaving dad in the back seat of the car that he is accustomed to driving. 

As fathers of children with a complex heart defect, who still may not have appropriately dealt with the stress of the diagnosis, we are still scared and insecure about how to react.  We too are thrust into this new situation and we want to do everything we can to help.  However, it appears to come more naturally to mom. This is threatening to our masculinity, so we often do not exercise our ability to reach out for help.  The last thing we want to do is make a mistake, because in this game, mistakes can be tragic.  So we retreat to what we know - providing and protecting.  When considering the idea of one parent choosing  to stay home to care for a child, it is often mom.  Dad continues to work in an attempt to make a larger financial contribution to the family. We feel this is our way to provide a sense of security for our family and to make up for all the shortcomings we perceive when compared to SUPERMOM.

So we ask again, where are all the dads?  Well, in three of the social media groups that exist specific to dads, there are a grand total of less than 100 of us involved. This would make it appear that we all ran for the hills.  Some do, but most go about winning their father-of-the-year award quietly.  We take the kids to the doctor “because our wives told us to,” hoping to retain and deliver the correct information so we don’t have to tuck our tails.  We go day to day holding onto similar stress and anxiety that moms do, but we don’t have the same social bonding ability to help cope with those feelings.  The good news though is that the brain can change. Many dads are becoming more involved in the day-to-day care of their children. Although moms still hold onto a larger percentage of the primary care giver role, dads are beginning to do their share.  Dads can also respond positively to bonding through social groups. Moms can help by encouraging their middle aged, balding child to reach out to other dads through social groups.  This will help to reassure us that we aren’t the helpless creatures we feel like, knowing that we aren’t alone.  Dads, join in the conversations, express emotions and ask questions. It may not be easy, but it will help.  Maybe it will improve family dynamics or it might even get us out of the doghouse. Who knows, perhaps we’ll develop some of our own super powers…But we won’t know until we try.   


Tuesday, May 13, 2014

What If...

Several weeks ago, Sisters by Heart reached out to our HLHS community asking families to submit photos of their heart kids with their care team members to be used in a special project. We are so excited to finally be able to share this special video project that debuted at the Spring National Pediatric Cardiology Quality Improvement Collaborative (NPCQIC) Learning Session!  
The video features the song “What If” by Five For Fighting; to say the lyrics are powerful would be an understatement. 

It is safe to say we expected the crowd to react positively to the video, but what happened next came as a complete shock and truly moved the entire room (parents, cardiologists, nurses, dieticians, quality improvement experts, etc.) to tears.  Dr. Gail Wright, upon watching the “What If” video, wrote down her raw, emotional thoughts to parents, then courageously read them to a room filled with nearly 200 participants:

What If….?
What if…
               I told you we are not in control?
What if…
               I told you that there are not answers?
What if…
I told you that I wish there was really a Journeyboard, rather than wide open space?
What if…
               I told you I don’t know?
What if…
               I told you I don’t understand why this happens either?
What if…
               I told you I can’t make that part better?
What if…
I told you a bunch of knuckleheads won’t cough up the money to educate the nurses who are taking care of your baby tonight and that fighting for that is sucking the very marrow out of me?
What if…
I told you your life has changed forever today, but somehow you will keep walking forward?
What if…
I asked you how we can go forth together not knowing what’s going to happen?
What if…
I told you take a picture now in the CVICU so we can laugh together at his high school graduation?
What if…
I looked you in the eye and told you that your son is a miracle and is why I really do believe in miracles  ?
What if…
I told you there are literally 300 people today who will be holding your baby in their hands with their focused attention and deepest intentions?
What if…
I tell someone that taking care of babies with half a heart has taught me how to live whole-heartedly?
gail e wright, MD
April 26, 2014
Upon viewing the “What if?” video by Sisters By Heart NPCQIC Learning Session
Cincinnati, Ohio

This - yes this - is a prime example of openness and transparency amongst parents and clinicians.  These types of interactions and candid discussions will help reshape the way pediatric medicine is practiced.  Parents and clinicians, coming together as partners, to move mountains.  What better place to start than with pediatric cardiology?  What better time to start than now?

Please feel free to share this post with your cardiac center leaders; let them know you're committed to working as a partner to transform the practice of pediatric cardiology on a broad scale. 

Many thanks to Gail Wright - for taking a giant leap forward and improving upon our work together, as partners, to move mountains for HLHS children - and beyond.  


Wednesday, April 2, 2014

HLHS Collaborative: March's Action Period Call

The National Pediatric Cardiology Quality Improvement Collaborative (NPCQIC) is preparing for its spring Learning Session - where parents and practitioners come together to discuss a range of topics and collaborate on how to transform care and identify best practices for HLHS infants.

If you've ever wondered what goes on backstage with NPCQIC, March's Action Period Calls focused on some of the "behind the scenes" work within the collaborative.   The call began with details about the monthly NPCQIC e-newsletter received by participating cardiac centers and parents.  There is some concern regarding the rate at which the newsletters are opened/read, however, each month, 80-90% of centers have at least one member utilizing the newsletter.  Centers and participants discussed with NPC leaders how the information shared via e-newsletters might be more effective.

Conversation quickly shifted to planning and preparations for the Learning Session taking place April 25th and 26th in Cincinnati. Centers will be busy doing their homework to prepare and sharing tools that others can learn from.
Both practitioners and parents are excited for the upcoming Learning Session, as parent participation has nearly doubled in the last six months and the parent voice will be paramount to driving discussion.  If you're an HLHS parent and interested in joining NPC-QIC Learning Sessions, please contact your cardiac center for details.  A list of participating cardiac centers can be found HERE.  If your center is not currently participating in the collaborative and you'd like to see their name on the list of "Participating Sites," please email with your name and care center.

For parents attending the spring Learning Session, an orientation call will be held next week to help newly attending parents feel comfortable in NPCQIC's process and in working with dedicated groups of medical professionals. 

For those who have attended a Learning Session in the past, please share your experience and for first timers, what questions do you have about what to expect?   Not able to attend? Don’t worry, updates will be provided via Twitter, ‪#‎NPCQICLS‬.

March's calls ended with an “unveiling” of the reorganized Collaborative SharePoint site. This site manages and stores NPCQIC's important information - tools that centers share and data for each participating cardiac center. The site is not available to parents - except for those on the Parent Leadership Team, but keeping with the theme of parent engagement, NPC involved a parent in the reorganization process.  The site looks much cleaner and is easier to navigate.

While we don't always see "behind the scenes," there is much work going on within NPCQIC, all of which assists centers to collaborate and engage in improved care for our children.

We're very much looking forward to seeing many of you in Cincinnati in a few short weeks!  Until then.... virtual heart hugs.


Wednesday, March 5, 2014

HLHS Collaborative: February's Action Period Call

February's Action Period Call updates are brought to you by Trent Neely and Rivkah Reichmann:

Action packed summarizes this month’s call!  Each of the two calls began with a presentation of recent research derived from NPC-QIC's multi-institutional data set.
On Thursday's call, Dr. David Brown from Boston began with discussing a recent study regarding the association between Prenatal Diagnosis (PD) and Morbidity. Previous studies yielded variable results, indicating some benefits, as well as some downfalls. The recent data is now indicating more benefits to PD, such as decreased valve regurgitation, ventricular dysfunction, and shorter ventilator time post-surgery. One area that was not included in the study was parent stress. Parents were able to jump in and provide their experiences with PD and the ability to prepare for hospitalization and the interstage period.  

On Monday's call, Dr. Matt Oster from Atlanta discussed a study regarding home monitoring. There was a discussion of some data on home monitoring. While the data reviewed didn't seem to correlate daily/weekly home monitoring with decrease in mortality or readmission rates, it did seem to impact weight gain. Those with daily home monitoring had better weight gain than those without home monitoring. The nurses and doctors on the call really wanted to hear from the parents -- they were interested in knowing whether home monitoring added to parents' anxiety and stress levels, or helped alleviate anxiety and stress. A good discussion was had of some of the benefits of home monitoring, and some of the challenges.  The study, in particular, did carry it's own limitations, including omitting hybrid patients, excluding those with no echo data, and not having access center specifics.

Following discussion on these NPC studies, teams were updated on efforts made by the Research and Publications Committee. The Parent Leadership Team requested a summary of findings from a recent study on the relationship between Prenatal Diagnosis, birth location, and mortality. This is a valuable tool to help parents understand research studies.  The first "Research Explained" can be found on the SBH blog and NPC-QIC's research page.  Based on the response from this first review, NPC will continue "research explained."  If you have come across a study or abstract relevant to HLHS and NPC's work, please feel free to email a link to and we'll pass it along to the Committee.  Additionally, NPC's Research & Publications Committee is adding two parent representatives to assist in reviewing manuscripts/abstracts and research explained projects.

Each call finished with review of survey results obtained from centers aimed at exploring various interstage practices.  Some of the discussion surrounded center practices regarding red flags, interstage care, and frequency of follow up care. The discussion flowed into a discussion of reasoning behind frequency of visits and proximity to center.  Some discussion was had regarding becoming more transparent in sharing data and protocols and the fortunate timing of NPC's new Transparency Workgroup.

According to parents on the call, February's AP Call was probably the most engaging call of the past year. 
Please keep the discussion going by providing your thoughts and experiences on the following research topics:
Prenatal Diagnosis:  Were you prenatally diagnosed? Was this beneficial to help you prepare or was it too much stress? How did either experience help you with care? 
Interstage Monitoring: Did your center offer daily or weekly home monitoring? Did you find it helpful? More stressful? Less stressful? If you didn't have home monitoring, did you wish you did, or did you feel calmer without the stress of worrying about home monitoring?
Thank you for your continued engagement and your participation.  Each comment shared improves the experience and outcome for future HLHS families.  We look forward to meeting many of you at April's NPC-QIC Learning Session in Cincinnati!


Tuesday, February 18, 2014

Research Explained: Factors that Affect Survival of HLHS Infants

Often times, research studies are published in medical journals that relate to our HLHS community.  Some of these studies spread rapidly through social media sites and become a topic of conversation amongst HLHS parents.

At the request of NPC-QIC's Parent Leadership Team, their Research and Publication Committee reviewed a recent article that gained quite a bit of traction with HLHS families, nationwide. The study was published in the October, 2013 edition of Circulation:  “Prenatal Diagnosis, Birth Location, Surgical Center, and Neonatal Mortality in Infants with Hypoplastic Left Heart Syndrome.

NPC-QIC's Research and Publication Committee reviewed the study and provided the following summary for parents, or "Research Explained" as we like to call it. 

Key Findings: Prenatal Diagnosis, Birth Location, Surgical Center, and Neonatal Mortality in Infants with Hypoplastic Left Heart Syndrome
The journal Circulation published a study in October 2013:  “Prenatal Diagnosis, Birth Location, Surgical Center, and Neonatal Mortality in Infants with Hypoplastic Left Heart Syndrome.”
The NPC-QIC Research and Publication Committee has reviewed this article and a summary of the findings can be found below.
Main Finding from this Study:
The authors of this study concluded that infants with HLHS born closer to a surgical center that performs cardiac surgery on infants have a higher survival rate than infants born far from a surgical center. 
About this study:
·        Why is this study important?

Over the past several decades there has been dramatic improvement in surgical survival in infants born with HLHS. However, ~20% of infants born with HLHS still die within the first months of life.   We are constantly looking for ways that we can improve the survival of infants with HLHS, trying to identify improvements we can make in the way we care for these infants. This study attempts to identify risk factors that we might be able to address to improve this survival.

·        How was this study performed? 

The researchers in this study used information from the Texas Birth Defects Registry and identified 463 infants with HLHS born in Texas from 1999-2007. The researchers then looked at where these infants were born, and what the distance and driving travel time was between their birth hospital and the closest surgical hospital that performed stage 1 surgical palliation (Norwood procedure). The researchers then looked for a relationship between this distance and the likelihood of survival of these infants. 

·        What were the results of the research?

o   Data for a total of 588 infants with HLHS born in Texas between 1999-2007 was available in this Registry.

o   Several factors were found to be associated with better or worse survival for infants born with HLHS:

§     The researchers concluded that infants born far from a surgical hospital (more than 90 minutes driving distance away) have worse survival.

§     Infants with a birth weight <2.5 kg had worse survival.

§     Infants who had surgery at a surgical hospital who performed more Norwood surgeries had slightly better survival after that surgery.

o      While prenatal diagnosis was not by itself associated with better or worse survival, prenatal diagnosis is a very important factor related to distance from the surgical hospital; 66% of infants who were born less than 10 minutes from the surgical hospital were diagnosed prenatally as compared to only 13% of the babies born more than 90 minutes from the surgical hospital.

·        What are the limitations of this study?
Studies that use databases like the Texas Birth Defects Registry are nice because they allow researchers to look at a large number of patients.  This is especially helpful when trying to learn about patients with rare problems like HLHS.  However, one of the problems with research using databases is that there often is incomplete information about the patients. The following are some of the other things that limit the interpretation of findings from this study.
o      Survival was better in infants born in the more recent time period (2003-2007) than in the older (1999-2002) period. More infants were diagnosed prenatally in the more recent time period (49% versus 25.5%) and that number has likely continued to increase in recent years.

o      More infants living more than 90 minutes from the surgical center were Hispanic (30% of mothers born in Mexico) and lived in poverty, suggesting they may have had more limited access to care, including lack of prenatal diagnosis. These socio-demographic factors were not included in the study of factors that may affect survival.

·        What are the takeaway messages considering the results and limitations of this study?

There are many factors that affect survival of infants born with HLHS.  There are some findings from this study that may allow us to improve survival of these infants moving forward.

·        This study did note worse survival in infants with HLHS born far from surgical hospital.  However, there are likely other factors that influence that finding, including the changes in surgical practices over time and socio-economic factors that may influence survival.

·        Prenatal diagnosis is important because it may reduce mortality if mothers living far away can deliver close to a cardiac surgery center.  Prenatal care is also important because we know that infants with HLHS who are born prematurely or with a low birth weight (<2.5kg) have worse survival. We should be doing everything we can to make sure that pregnancies receive adequate prenatal care and appropriate referrals.

·        We need to continue to understand the relationship between surgical volume (the number of specific procedures that are performed at a surgical hospital) and the survival before and after surgery in infants with complex heart problems like HLHS.  Some studies, including this one, have suggested that the more surgeries that are done, the better the survival.  Collaborative work, like that going on in NPC-QIC will be required to understand the answer to this question. 

"Research Explained" is an example of the type of collaborative work you can expect to see between  NPC-QIC and parent partners in the pediatric cardiac community.  We look forward to sharing additional studies and projects which engage and empower HLHS patients and families.