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Hypoplastic Left Heart Syndrome: Key Insights for Pediatricians · Pediatrics in Practice: A CME Podcast

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  Michael Smith, MD (Host): Welcome to Pediatrics in Practice, A CME podcast.
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  joining me today is Pza Doctor, a medical doctor from Children's Mercy.
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  Today we're diving into an important topic, hypoplastic left heart syndrome,
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  key insights for pediatricians.
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  Thanks for joining me today.
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  I want to dive right into this and ask you what are the key prenatal indicators
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  of hypoplastic left heart syndrome?
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  Pezad Doctor, MD: Thanks for having me on this podcast.
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  in terms of prenatal diagnosis, as the name suggests, hyperplastic left
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  heart syndrome is usually when the left side of the heart, That is mainly
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  the left ventricle, mitral valves.
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  The aorta is, small and underdeveloped.
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  And.
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  Sometimes the spectrum can be that it can be completely absent.
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  It's usually detected, prenatally these days when there is a routine antenatal
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  scan done at the ob GYNs office.
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  And when there's always a suspicion that they cannot see all the four chambers of
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  the heart, that's when, these children or these fetal moms are refer to cardiology
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  where a fetal cardiologist, performs the study and, confirms the diagnosis.
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  Michael Smith, MD (Host): So how can pediatricians support
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  families during this process?
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  This is a hard diagnosis to hear, right?
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  So any, tips.
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  Ideas you can give pediatricians, for helping their families go
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  through that diagnostic process.
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  Pezad Doctor, MD: yes.
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  it's extremely stressful situation from the time that there may be something
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  wrong with the baby's heart in neutral, and by the time they see the fetal
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  cardiologist, it could be sometimes the next day or it could be sometimes weeks.
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  And in the meantime, the parental anxiety can be, really,
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  striking and nerve wracking.
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  So, in terms of coordinating care with their.
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  Primary pediatrician that they know of from their other siblings, or if
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  they have someone, in mind who is close to their, location to the area.
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  It's definitely helpful for the pediatrician to talk to them about
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  the basic nuances of what this, condition means for the child.
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  And what are the.
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  few palliative surgeries that this child may need eventually, in the future.
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  but just to summarize that This is, a stressful situation for the parents
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  and having a pediatrician, to, uh, explain them, the nuances of
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  this diagnosis, is really important.
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  Michael Smith, MD (Host): What is the prevalence of
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  hypoplastic left heart syndrome?
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  Pezad Doctor, MD: It is one of the rare congenital heart defects.
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  Overall, we maybe see like one in, every 5,000 alive births.
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  in terms of all the congenital heart diseases, it's one in a hundred.
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  So, let's say out of five or 10,000 live births, one may have hypoplastic left.
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  Michael Smith, MD (Host): So what should pediatricians know about the stage?
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  Surgical approach to this And what role do they play in
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  coordinating care between surgeries?
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  Pezad Doctor, MD: in Sharon with this condition, there is, drastic
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  changes in their hemodynamics throughout their palliative surgeries.
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  they mainly have three surgical palliations, starting from a
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  normal procedure That is soon after the birth in the newborn period.
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  it's to ensure, a stable source of systemic and pulmonary blood flow.
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  So since the left ventricle is underdeveloped, the right ventricle is
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  fashioned to supply both the circulations.
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  after that procedure, the children, at about six months of age undergo the next
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  operation called the Glen Procedure.
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  so in between those first stage and second stage palliative, is called the
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  Interstage period, which is really, high-risk mortality in this age period.
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  So it's important to maintain, Good balance between the systemic
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  and pulmonary blood flow.
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  And the best way is by assessing their oxygen saturations.
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  So we guide pediatricians to make sure when they routinely see these children
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  every other week with the cardiologists, that's been the practice, during the
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  end-stage period to make sure they're gaining weight so that we know that
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  there is good systemic blood flow.
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  They're feeding well, And the oxygen saturations are where we expect
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  them to be between 75 and 85%.
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  Michael Smith, MD (Host): So gaining weight, watching oxygen saturation,
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  Those are the key things during this very important time period.
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  Pezad Doctor, MD: Exactly.
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  so we have, parents note down how much feeds they have been taking.
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  Sometimes fa failure to feed or feeding intolerance can also suggest, compromise.
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  Michael Smith, MD (Host): If a child comes out of this like very important
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  phase, does the outcome become better?
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  Pezad Doctor, MD: Yes, absolutely.
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  previously, in this inter staged, mortality was close to 30%.
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  but due to, implementing of home monitoring programs and,
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  you know, with the help of CLO coordination with the pediatrician
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  And the cardiologist, we have.
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  Been able to significantly bring down this mortality, to close to now 2%.
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  as you said, once they have that, second stage where it's a more
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  stable form of pulmonary blood flow, the mortality, is much lower.
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  Michael Smith, MD (Host): So 30% down to 2%.
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  that's significant.
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  Pezad Doctor, MD: Absolutely.
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  it's, all thanks to this close monitoring that we do in these children
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  along with the care of pediatricians.
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  Michael Smith, MD (Host): Yeah, I wanna talk about that.
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  So the main app that's used now is called Champ, right?
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  That's a, remote monitoring app.
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  How is that, guess you're saying that's had like some of the biggest.
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  Impacts and outcomes.
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  Huh?
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  Pezad Doctor, MD: Yes, absolutely.
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  So initially, children, when they would be discharged from the hospital, they would
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  sometimes be, not clear understanding of what is normal for these children.
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  You know, these children are, as I said, very, high acuity,
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  at least for decompensation.
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  So with this monitoring, some people call it, so Children's Mercy kind of
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  pioneered this champ, application where, parents are given An iPad and there is a
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  portal where they can have to document the child's weight, the oxygen saturations,
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  the feedings, And we closely monitor with the entire sham team on how they're doing.
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  if there is any concern, uh, them.
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  Parent has about their child at home during this end-stage period, they can
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  quickly shoot us a message and it comes as a page to our on-call cardiologist.
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  we also now have the ability to record videos if they think the
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  child is very blue, the child is grunting, the child is, we can all,
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  yeah, we can remotely access that.
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  Michael Smith, MD (Host): and of course that helps you.
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  It just speeds up the recognition of an issue that there's something
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  going on, that's fascinating.
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  what are realistic expect expectations?
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  For growth, development activity levels in children with HLHS how
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  do pediatricians play a role in, facilitating that counseling families?
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  can you dive into that a little bit?
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  Pezad Doctor, MD: Absolutely.
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  once they have the Fontan operation, That's the third
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  final stage of their palliation.
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  That happens usually at three to four years of age.
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  so you can imagine that by the age of four years they've had multiple
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  heart surgeries, some of which requiring a heart lung machine,
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  long ICU stays, cat procedures.
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  and This is just a routine.
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  if there are complications that may even, have more cath procedures.
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  So, children's neurodevelopmental, That we have found that especially
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  in older kids who are studying, we have found learning disabilities.
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  We have found attention deficits And also an increased risk of,
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  mental health issues like anxiety, depression in these groups.
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  So understanding that evaluating that, at the right time is
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  really appropriate so that, we can guide them through therapies.
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  talking about the physical activity, we.
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  Know that since these children have one ventricle, which is doing the job
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  of both initially, And then the final palliation is where we want the pulmonary
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  blood flow to go passively to the lungs.
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  So the children's exercise capacity is reduced, but we encourage pediatricians
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  and, through our Fontan clinic we have a multidisciplinary clinic where we closely
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  monitor these children and continue to encourage them to participate in sports.
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  Michael Smith, MD (Host): What about supporting families emotionally not just
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  the patient, but the parents, siblings.
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  how can pediatricians work through that?
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  Pezad Doctor, MD: it's a very long journey, at the end of the road.
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  And then, despite doing all these palliations, there is a lot of
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  long-term issues these children can encounter and, I think supporting.
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  First understanding what the parents, are hoping for their child
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  to, finally succeed in whether it is, final graduation or it's the
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  being able to participate in sports or, they're having other issues.
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  identifying them early on, where they have a close, bond with their pediatrician.
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  and noticing that will really help us kind of guide them in the right.
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  So, I mean, I really appreciate the pediatrician's role in identifying
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  these, high stress or behavioral concerns in children and their parents.
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  Michael Smith, MD (Host): You know, when it comes to these,
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  Air, congenital conditions.
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  one of my favorite questions is talking a little bit about promising innovations,
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  are there any things that are being studied, clinical trials that are going
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  on that you would like to share with pediatricians that are kind of exciting?
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  You?
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  Pezad Doctor, MD: Yeah, there are a few, actually.
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  the one That is really exciting is the, reenergized trial That is
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  going on and still actually enrolled.
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  participants.
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  So the idea is that, as I mentioned earlier, these children
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  with fonta and palliation do not have adequate exercise capacity.
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  And, when compared to children with a healthy heart, their capacity is
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  about 40 or 50% of that, that we use the oxygen uptake as a on exercise
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  stress test, as a marker of that.
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  And we have identified that a certain cohort of these, children with fontans
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  have in fact near normal exercise capacity and are able to, perform as,
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  any other child of that age group.
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  And we found that these children, have been enrolled in
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  competitive sports, athletics.
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  they're pursuing, postgraduate education and they have more muscle mass.
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  So the theory is that since the fontan palliation is a method of sending
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  passive blood flow to the lungs and augmenting cardiac output, perhaps their
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  activity level, the secondary muscle in their legs is acting as that bump that
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  they're deficient of in their heart.
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  And.
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  That is supplying more blood to their lungs, more cardiac output, and
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  they're able to perform very well.
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  so coming to this clinical trial, they are actually providing an exercise
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  prescription to these children.
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  and, every, three weeks, they expect them to do certain activities for an hour.
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  And then, they're again reassessing their exercise capacity and seeing if
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  that, helps improve their exercise capacity as well as reduce any long-term,
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  issues with their other organs, like liver failure, lymphatic failure.
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  Michael Smith, MD (Host): that's fascinating to me.
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  that's an amazing, exciting, thing that's coming down the line.
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  how do you see this playing out in the future?
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  when does this become something that we'll be able to talk more
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  about with the average patient with hypoplastic left heart syndrome.
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  Pezad Doctor, MD: This is really a great, breakthrough we have, through
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  some retrospective studies, identified these findings that, you know,
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  children with more muscle mass act.
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  Are overall doing well, and now we need to implement a more randomized
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  control trial to make sure the cause effect is appropriate.
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  we had other studies in the past where, one of the other clinical trials was
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  fuel study where they gave pulmonary vasodilators and tried to see if
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  there's increased excess capacity in this children with very limited like.
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  Statistical significance.
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  So I think this approach would be really, amazing to see if we are able
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  to improve the quality of life overall.
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  So more to come on this.
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  Michael Smith, MD (Host): Yeah.
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  Very interesting.
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  I'm sure you'll be watching all of this closely.
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  This was great information.
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  thank you so much.
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  For coming on the show today and sharing your insights for more information,
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  please visit CM KC link slash CME podcast.
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