Case Presentation - Management of Hypoplastic Left Heart Syndrome: A Team Approach

0:00 for this presentation i'm going to present a uh case of management of hypoplastic left heart syndrome and carry on the theme that we've already uh started during the early part of the sessions and go over how this is a team approach so as we showed earlier this is a fetus who came for an echocardiogram and was found to have hypoplastic left heart syndrome as we previously said there's a large right ventricle right here a large right atrium just this little sliver of left ventricle and a smaller left atrium so hypoplastic left heart syndrome consists of severe aortic stenosis or aortic atresia

0:47 and or severe mitral stenosis or atresia and no matter which results in a very small non-functional left ventricle with no way to get blood to the systemic circulation except the ductus arteriosus so that's what this cartoon shows a very small almost non-existent left ventricle with still the presence of this ductus arteriosus the fetal structure that connects the pulmonary artery to the descending aorta you could see that as in most cases the ascending aorta right here and the transverse aortic arch is also quite hypoplastic so after birth infants become acutely ill when the ductus arteriosus closes so this can be generally within the first

1:33 day sometimes at two or occasionally three days of life poor systemic cardiac output is caused by the anatomic obstruction of blood flow to the body so the real question is how do we get from the diagnosis to a healthy baby that goes home so the majority of prenatal uh diagnoses as we've sort of hinted at are made after the quote-unquote anatomy ultrasound or the ultrasound performed generally around 18 to 20 weeks for surveillance of fetal anatomy but sometimes it's later depending on um multiple factors that might influence the uh the maternal imaging or just sort of detective detection as we discussed i think prompt

2:20 referral for a fetal echocardiogram is generally the next step at the fetal echocardiogram we'll both confirm the diagnosis as well as figure out the anatomic details but almost more importantly we'll also begin the process of family counseling and education so in our program we'll generally do a repeat fetal echocardiogram around 26 weeks which is probably about a month later and then again at around 32 to 34 weeks and that that at that time will also um start the pre or the delivery planning like dr chapa hinted at before we generally will have a genetics consult if one has not been done before obviously the pregnancy needs continued maternal fetal medicine follow-up to assess fetal growth any

3:06 extra cardiac anomalies and assessments of fetal well-being and then we'll recommend and help plan delivery at the surgical center so in terms of prenatal goals the prenatal goals are really the comprehensive counseling um to detail the success of both the surgical strategy but also the short and long-term outcomes uh we'll provide prenatal options including termination if that's appropriate uh prenatal follow-up for the growth and the size of the left heart structures um and the aorta as well as the patency of the atrial septum as we discussed earlier that can be a critical issue at the time of birth we'll develop a comprehensive delivery plan and we'll help counsel the family for the

3:51 the issues that may occur in infancy as well as long-term care so at the 32-week visit we consider this often our sort of comprehensive counseling visit it's not always at 32 weeks sometimes before but the um the mother will generally get a fetal echocardiogram and continued cardiology counseling we'll have a maternal fetal medicine consult and an ultrasound if it's needed at that time a visit with the pediatric cardiac icu physicians who go over pre and post-op course and management with the neonatologist to discuss delivery room course with palliative medicine to figure out family support with a congenital cardiac surgeon to go over the details

4:37 of the surgery and the expected management tours of the facility as well as a delivery plan that will include any special issues or concerns delivery should be as we discussed ideally at the center where the definitive care of the infant can be provided that's not possible in some systems where there are freestanding children's hospitals and a sort of university or other type of um of situation that has to deliver the mother that's close by but there certainly are advantages to this all being in one site and as we discussed we have in our system our special delivery unit which it sounds like we'll discuss more later but in brief is a small and specialized labor and

5:24 delivery unit that is designed for high-risk mothers who need the entire facilities of the tertiary care hospital behind them or for infants who have some type of defects that need the full support of the children's hospital behind them prompt evaluation by neonatology and cardiology occurs after delivery and bonding time is given with the parents if the infant is stable the infant is then transferred to the pediatric cardiac icu so these babies get initially stabilized medically with prostaglandin which keeps the ductus arteriosus open our goal is actually to promote the fetal

6:10 circulation the same thing that was happening in the womb where they were fine and that is pulmonary the blood flows through the ductus arteriosus from the pulmonary artery to the aorta this allows for and increases systemic output or output to the body and ideally reduces pulmonary blood flow we minimize supplemental oxygen occasionally use hypercarbia or hypercapnia and try to promote some mild respiratory acidosis we promptly treat any metabolic acidosis with regard to the surgery i think we'll have more talks later but we cannot restore a quote-unquote normal circulation for these babies but the first stage is what we call a norwood repair which turns the right ventricle into the systemic or left ventricle

6:55 and turns the pulmonary valve into the aortic valve a shunt or a small tube then provides blood flow to the lungs the glenn and the fontane are the procedures that the child has to go through later generally at about six months and three to four years respectively the alternative management is a heart transplant that's usually not used primarily but for cases where the conventional surgery for whatever reasons is not successful so this cartoon just shows the norwood operation this is the case of the hypoplastic left heart syndrome the pulmonary artery is transected and the aortic arch opened up this main pulmonary artery is going to be anastomosed to the aortic arch and the arch rebuilt

7:43 to be of normal size which you could see right here here's the reconstructed aortic arch and then this shunt puts uh delivers some blood flow from the right ventricle to the pulmonary arteries these are the second and third stages which we talked about which i'm not going to go through in detail right now so with regard to postoperative care and discharge these babies have multiple medical needs to begin with they still have a single ventricle heart so as we said they're not fully corrected this is different than some forms of congenital heart disease that we treat even some forms of neonatal congenital heart disease that we treat there are increased metabolic demands of the heart and there's quite frequently extra cardiac anomalies or genetic syndromes which also

8:29 impact the well-being of the child very frequently they have feeding intolerance and abnormalities in that area developmental issues are much higher than the baseline population there's often need for additional medical procedures and medications and not to be overlooked as parent and caregiver stress and depression so we've created this journey board for the families to use when they are transferred to the step down unit this kind of goes over and highlights for the families as well as for the nursing staff all of the different steps that the baby needs to go through prior to discharge from the hospital many people like a graphic representation and it helps them sort of understand what it takes for the baby to get out of

9:15 the hospital we also have a home monitoring program for these and other high-risk babies the babies are discharged home with a baby scale a pulse ox a red flag action plan to let the caregivers know what might be an urgent situation or an emergency as well as a laminated card that has their diagnosis as well as some of their expected oxygen saturation emergency medications things like that that they can hang from their car seat and have with them if they need to go to an emergency room that's not part of our system or find themselves someplace else where they need emergency care we have the parent or guardian enroll in

10:01 my chart for the baby on on the baby's behalf and then we have an application through my chart where the parent can enter the baby's daily weight the daily pulse ox as well as the feeding volume this data goes directly to a flow sheet in epic the electronic health record and it's monitored daily by one of our nurse coordinators we then have comprehensive outpatient visits for these uh babies with a cardiologist a nurse coordinator and a dietitian so um unfortunately this is not always the situation that we have for these uh babies with such high risk heart problems but our goal is still to be able to go from here to a healthy baby at home thanks