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Hypoplastic Left Heart Syndrome (HLHS)

  • If a prenatal ultrasound has revealed that your baby will be born with hypoplastic left heart syndrome (HLHS)—or if your infant has been diagnosed with HLHS—an understanding of the condition will help you plan for, and cope with, this rare and complex congenital (present at birth) heart defect.

    While this condition is very serious, children with HLHS can undergo therapy at Boston Children's Hospital—shortly after birth or in some select situations, while your baby is in utero.

    In the infant and child with a normal heart…

    Oxygen-poor (blue) blood returns to the right atrium from the body, enters the right ventricle and is pumped through the pulmonary artery into the lungs to receive oxygen. The oxygen-rich (red) blood returns to the left atrium from the lungs, passes into the left ventricle and is pumped out to the body through the aorta.

    HLHS normal heart

    In HLHS…

    • Most of the structures on the left side of the heart are too small and underdeveloped (hypoplastic) to provide enough red blood flow for the body’s needs.
    • The small left ventricle, which needs to be large enough and strong enough to pump blood out to the body, simply can’t function effectively.
    • Other left heart structures can also be underdeveloped in varying degrees—including the mitral valve, the aortic valve and the aorta itself.

    How Boston Children’s Hospital approaches HLHS

    The experienced surgeons in our Advanced Fetal Care Center and Cardiac Surgery Department understand how distressing a diagnosis of HLHS can be for parents. Ranked #1 for heart care and heart surgery in 2010, Children’s takes an innovative approach to treating HLHS—including the world’s largest and most experienced program for fetal cardiac intervention.

    You can have peace of mind knowing that Boston Children’s surgeons treat a high volume of some of the most complex pediatric heart conditions in the world, with excellent success rates. We’re home to some of the world’s foremost pediatric physicians and researchers. And we provide families with a wealth of information, resources, coordination and support—before, during and after your child’s treatment.

    With our compassionate, family-centered team of caregivers providing expert treatment, follow-up and aftercare, you and your baby are in the best possible hands.

    HLHS @ Boston Children's featured on National Public Radio

    Listen as Boston Children's cardiologist Wayne Tworetzky, MD, describes our unique expertise in a special treatment for some cases of HLHS—fetal intervention, or operating on the baby's heart while she's still in utero. The NPR radio segment is called, "Stitch in Time: Fixing a Heart Defect Before Birth." 

    Screening neurodevelopment problems 

    Children who’ve had surgery for heart disease as infants are at greater risk of neurodevelopmental problems. By school age, they tend to have more academic, behavioral and coordination difficulties than other children. Boston Children’s Cardiac Neurodevelopment Program—one of just a handful in the United States—provides expert screening, evaluation and care for infants, children and teens with congenital heart disease who are at risk for neurodevelopmental problems. Screening begins soon after your child’s first cardiac surgery and continues as your child grows to make sure she’s hitting her developmental milestones. 

    Hypoplastic left heart syndrome: Reviewed by Wayne Tworetzky, MD
    © Boston Children's Hospital, 2010
     

  • Hypoplastic left heart syndrome (HLHS) is one of the most challenging congenital heart defects to care for. But Boston Children's Hospital is a world pioneer in innovative HLHS care—including fetal cardiac intervention—and our team is well-qualified to care for your child.

    We are known for our science-driven approach. We’re home to the world’s most extensive pediatric hospital research enterprise and we partner with elite health care and biotech organizations around the globe. But as specialists in innovative, family-centered care, our physicians never forget that your baby is precious, and not just a patient.

    What characterizes HLHS?

    HLHS Heart

    Hypoplastic left heart syndrome is one of several congenital (present at birth) heart defects in which the heart has only one fully functioning ventricle (single ventricle defect).

    In HLHS, most of the structures on the left side of the heart are too small and underdeveloped (hypoplastic) to provide enough red blood flow for the body’s needs. The most critical defect in HLHS is the small left ventricle, which needs to be large enough and strong enough to pump blood out to the body. If it’s too small, it simply can’t function effectively.

    Other left heart structures can also be underdeveloped in varying degrees, including:

    • the mitral valve, which controls blood flow between the left atrium and left ventricle in the heart
    • the aortic valve, which regulates blood flow from the left ventricle or main pumping chamber into the aorta
    • the aorta, the largest artery in the body, which supplies oxygen-rich blood to the body and is one of the two great arteries in the heart

    In HLHS, despite the severity of the heart defect, the fetus or newborn is able to survive because of two naturally occurring “holes in the heart,” which allow for the communication of blood between the left and right sides of the heart. These are the:

    • patent ductus arteriosus (PDA)a blood vessel (the ductus arteriosus) that connects the two great arteries (the aorta and the pulmonary artery) and usually closes soon after birth. As long as the ductus remains open (patent), blood can pass from the right ventricle and pulmonary artery to the aorta and body, allowing some oxygen-rich blood to circulate.
       
    • patent foramen ovale (PFO): The foramen ovale is a small opening between the right and left atria, normally present in utero, but which usually closes shortly after birth. With HLHS, it’s important that the foramen ovale stay open (patent) as this allows blood returning from the lungs to cross from the left atrium to the right atrium and out to the body via the right ventricle and pulmonary artery.

    In HLHS, blood returning to the right atrium from the lungs must pass through the PFO to reach the right side of the heart. The right ventricle must then do “double duty”—pumping blood to the lungs through the pulmonary artery and to the body through the PDA.

    Once the PDA begins to close (a natural occurrence), the baby will become extremely sick (go into “shock”) due to lack of blood supply to the body. At this point, treatment is necessary for the baby to survive.

    How is HLHS diagnosed?

    • prenatal, or fetal, diagnosis: made by fetal ultrasound. When you go for your routine fetal ultrasound, your doctor should be able to identify:
      • a four-chamber heart
      • two valves entering the heart
      • two valves and blood vessels exiting the heart

    HLHS can be diagnosed on fetal ultrasound when the person performing the ultrasound notices that the left heart structures are small. If HLHS is suspected, you should be referred to a fetal pediatric cardiologist for a fetal echocardiogram (a specialized fetal cardiac ultrasound).

    • postnatal diagnosis: If HLHS wasn’t diagnosed prenatally, HLHS is usually diagnosed after the newborn baby shows signs of distress.

    What is the value of diagnosing HLHS prenatally?

    A diagnosis of HLHS through prenatal cardiac ultrasound enables us to plan to treat your newborn immediately after delivery. The family and medical team should plan for delivery at, or next to, a cardiac center that’s equipped to care for the newborn, such as:

    • a children’s hospital with an adult or obstetric hospital attached or very near
    • a medical center that has adult/obstetric care, as well as neonatal and pediatric cardiac services

    What are the symptoms of HLHS?

    At first, a newborn with HLHS may appear normal. But symptoms usually develop in the first few hours or days of life. Lack of vital blood flow causes the baby to go into profound shock, endangering the brain, liver, kidneys and other vital organs. Symptoms may include:

    • rapid breathing or shortness of breath
    • rapid heartbeat or pounding heart
    • poor suckling and feeding
    • cold extremities (poor perfusion)
    • blue color of the skin, lips and nailbeds (cyanosis)
    • lethargy

    How do you treat HLHS?

    • fetal cardiac intervention: A small number of prenatally diagnosed fetuses with HLHS may benefit from fetal intervention.
    • stabilization and intervention of the newborn: If the diagnosis of HLHS is known before birth, the neonatal and cardiac teams will begin caring for the newborn with HLHS immediately after birth. A few newborns will need to have a procedure to open up a too-small foramen ovale immediately after birth (performed in the cardiac catheterization laboratory), but most will be stable and require mostly close observation in the cardiac intensive care unit.

    An intravenous medication called prostaglandin (PGE), to keep the ductus arteriosus from closing, will be started and administered until the surgery.

    • staged surgical interventions: All children with HLHS will undergo at least three surgeries within the first three years of life.

      There are three variations of the first surgery (“Stage I”). Which variation is used will depend on the surgeon’s preference, the baby’s size, and the exact anatomic features of the heart. These surgical options are:
    • the Norwood operation
    • the Sano modification
    • the hybrid procedure

    The second surgery is called the bidirectional-Glenn shunt, and the third is called the Fontan. Together, they make up a series of surgeries called the Fontan Sequence. The goals of these surgeries are to:

    • enable the fully-functioning right ventricle to do the work normally done by two ventricles
    • separate the blue (low-oxygen-content) blood (pulmonary circulation) from the red (well-oxygenated) blood (systemic circulation)

    Will my child be OK?

    Open heart surgery at Boston Children’s has the one of the highest success rates in the United States among large pediatric cardiac centers. However, your child will need at least three staged surgeries in his first few years of life.

    Note: Infants who’ve had initial surgery for a single ventricle defect are typically enrolled in Boston Children’s Home Monitoring Program between the baby’s Stage I and Stage II operations in the Fontan sequence. For more, see “Boston Children’s Home Monitoring Program for your child” in Treatment & care.

    What about later in life?

    Children with hypoplastic left heart syndrome will need follow-up care throughout their lives to ensure that their hearts continue to function adequately. Most children will also need heart medication(s). Complications going forward can include arrhythmias (abnormal heart rhythms), heart failure, blood clots, and a few other rare problems.

    Your cardiologist will help you create a long-term care program as your baby grows into childhood and his teen years. Families with a child with HLHS will have an ongoing relationship with their cardiologist. We will treat your child if complications arise, and we’ll advise on daily-life issues such as exercise, activity levels, nutrition and pregnancy precautions.

    Causes

    It’s important to understand that as parents, you’ve done nothing to cause HLHS and its associated defects. Nothing you have ingested or have been exposed to environmentally has been linked to having a child with HLHS. Familial cases have been reported, but the genetic link has yet to be clarified.

    HLHS is the result of underdevelopment of sections of the fetal heart during the first eight weeks of pregnancy. But it’s not understood why some babies’ hearts don’t develop normally. Congenital heart defects usually occur sporadically (by chance), with no clear reason for their development.

    Symptoms

    At first, a newborn with HLHS may appear normal. But symptoms usually develop in the first few hours or days of life. Lack of vital blood flow causes the baby to go into profound shock, endangering the brain, liver, kidneys and other vital organs. Symptoms may include:

    • rapid breathing or shortness of breath
    • rapid heartbeat or pounding heart
    • poor suckling and feeding
    • cold extremities (poor perfusion)
    • blue color of the skin, lips and nailbeds (cyanosis)
    • lethargy
       

    When to seek medical advice

    Call your health care provider immediately if your baby or child is having difficulty breathing or is breathing rapidly, has a bluish color, tires easily, has cold hands and feet, is drowsy or unresponsive and is uninterested in eating.

    Who’s at risk

    Congenital heart defects usually occur sporadically (by chance), with no clear reason for their development. So, it’s difficult to predict who’s at risk. Familial cases have been reported, but the genetic link has yet to be clarified.

    Complications

    Children who have had HLHS reconstruction surgeries can be susceptible to arrhythmias, heart failure and blood clots as they go forward. And the potential does exist for needing a heart transplant in early or mid-childhood.

    Long-term outlook

    In many cases, the Fontan reconstructive surgeries now can palliate (not cure) HLHS, so the outlook for your child is positive. Children who have undergone HLHS reconstruction can be susceptible to arrhythmias, heart failure, blood clots and a few other complications. And the potential does exist for needing a heart transplant in early or mid-childhood.

    Your cardiologist will help create a long-term care plan as your baby grows into childhood and his teen years. Most people who have had congenital heart disease repair have an ongoing relationship with their cardiologist; we will consult and treat your child if complications arise, and we’ll advise on daily-life issues such as exercise, activity levels, nutrition and pregnancy precautions.

    For teens

    As a teen with a congenital heart defect, you have a lot to cope with. Besides the typical issues any teenager faces—from social acceptance to body changes and more—you also have to deal with medical appointments and procedures, some delay of your natural wish for independence, the consciousness of feeling different and personal responsibility for maintaining your good health.

    If you as a teen (or as the parent of a teen) feel overwhelmed, depressed or anxious through this time, speak to your doctor to get help.

    What you can do at home

    Consult your child’s cardiologist for guidance on specific steps for follow-up care at home. And as your baby recovers and grows, be sure to follow a regular program of well-baby/well-child checkups.

    Prevention

    It’s important to understand that as parents, you’ve done nothing to cause HLHS and its accompanying defects, and there are no precautions you could have taken to prevent it. Congenital heart defects usually occur sporadically (by chance), with no clear reason for their development.

    HLHS Glossary

    • Advanced Fetal Care Center (AFCC) at Children’s: the only comprehensive fetal care center in New England, and one of just a few in the United States. The AFCC is the first and most experienced center performing fetal cardiac interventions—treating abnormalities such as HLHS in utero to reduce potentially fatal concerns after delivery.
       
    • aorta: one of the heart’s two great arteries. In a normal heart, the aorta arises from the left ventricle and pumps oxygen-rich blood out to the body. In HLHS, the aorta can be too undersized to function adequately.
       
    • cardiac catheterization: provides detailed visual information and measurements about the structures inside the heart, cardiac catheterization is a procedure that can be performed on a fetus, a baby, a child or an adult. Catheterization can be diagnostic-only, diagnostic and therapeutic, or diagnostic and interventional.
       
    • cardiac/cardio-: pertaining to the heart
       
    • cardiac intervention: a minimally invasive procedure, such as cardiac catheterization or angioplasty, to diagnose and treat heart disease
       
    • cardiac magnetic resonance imaging (MRI): a non-invasive diagnostic tool using 3-D imaging technology produced by magnets to accurately determine the blood flow and functioning of your child's heart
       
    • cardiac surgery: surgical procedure performed on the heart
       
    • cardiac surgeon: doctor who performs surgery on the heart. A pediatric cardiac surgeon performs surgery on the hearts of infants and children.
       
    • cardiologist: doctor who diagnoses and treats heart problems non-surgically. A pediatric cardiologist treats infants and children with heart problems.
       
    • The Center for Families at Children’s: dedicated to helping families find the information, services and resources they need to understand their child’s medical condition and take part in their care
       
    • cyanosis (cyanotic): blue color of skin, lips and nailbeds caused by lack of oxygen-rich (red) blood circulating in baby’s bloodstream—a major symptom of HLHS
       
    • congenital heart defect: heart defect present at birth. The heart usually forms in the first eight weeks of fetal development. It's thought that most congenital heart defects develop during this period.
       
    • diagnosis: medical determination of illness or disease based on physical examinations and advanced technology diagnostic testing tools
       
    • echocardiogram (echo, cardiac ultrasound): a diagnostic tool that evaluates the structure and function of the heart using electronically recorded sound waves that produce a moving picture of your child’s heart and heart valves. The ultrasound can detect an HLHS defect.
       
    • electrocardiogram (ECG, EKG)a diagnostic tool that evaluates the electrical activity of your child’s heart. An EKG is usually the initial test for evaluating the causes of symptoms and detecting heart abnormalities, including HLHS.
       
    • fetal cardiac intervention: Advanced catheterization treatment of the baby’s heart while still in utero.
       
    • fetal echocardiogram (fetal ultrasound): a diagnostic tool that uses sound waves to evaluate the structure and function of the baby’s heart while still in utero. The ultrasound can detect an HLHS defect in the fetus.
       
    • fetal magnetic imaging (MRI): a non-invasive diagnostic tool that uses 3-D imaging technology produced by magnets to accurately determine the blood flow and functioning of your child’s heart in utero. Drawing on our expertise in pediatric imaging, Children’s is setting a new standard in fetal MRI.
       
    • Fontan sequence: a series of three palliative operations performed on children whose congenital heart defect results in just one full-sized functioning ventricle (single ventricle defect), including HLHS.  They are the Norwood (and its alternatives), the bi-directional-Glenn Shunt and the Fontan Operation. The Fontan surgeries are usually performed at intervals starting at a few months and ending at a few years of age.
       
    • heart valves: valves (tricuspid, pulmonary, mitral and aortic) that regulate uni-directional blood flow into and out of the heart chambers. In HLHS, the mitral and aortic valves are undersized.
       
    • hypoplastic left heart syndrome (HLHS): a congenital (present at birth) heart defect in which most of the structures on the left side of the heart—and especially the left ventricle—are too small and underdeveloped to provide enough red blood flow for the body’s needs
       
    • interventional cardiologist: a doctor who specializes in performing minimally invasive procedures, such as cardiac catheterization or angioplasty, to diagnose and treat heart disease
       
    • symptoms: the presenting reasons why a child needs medical attention. A symptom’s characteristics—including but not limited to onset, quality, triggers and severity—help diagnosticians to determine an illness or disease, or to determine what further testing is needed to determine the illness or disease.
       
    • palliative: aiming to reduce the severity and impact of a disease, rather than cure it
       
    • patent ductus arteriosus (PDA): a blood vessel (the ductus arteriosus) that connects the two great arteries (the aorta and the pulmonary artery) and usually closes soon after birth. As long as the PDA remains open (patent), blood can pass from the right ventricle and pulmonary artery to the aorta and body, allowing some oxygen-rich blood to circulate.
       
    • patent foramen ovale (PFO): The foramen ovale is a small opening between the right and left atria, normally present in utero, but which usually closes shortly after birth. With HLHS, it’s important that the foramen ovale stay open (patent), as this allows blood returning from the lungs to cross from the left atrium to the right atrium and out to the body via the right ventricle and pulmonary artery.
       
    • pulmonary artery: one of the heart’s two great arteries. In a normal heart, the pulmonary artery arises from the right ventricle and carries oxygen-poor blood to the lungs, where it receives oxygen.
       
    • pulmonary artery banding: a repair in which a band is secured around the pulmonary artery to limit blood flow to the lungs. Pulmonary banding is an aspect of the hybrid procedure, one of the surgical options for treating HLHS.
       
    • shunt: an artificial connection of blood vessels in order to redirect blood or other fluids. In pediatric cardiology and cardiac surgery, this usually refers to a Gore-Tex (a synthetic material) tube connected between the aorta or one of its branches and the pulmonary artery.
       
    • single ventricle defect (SVD): one of several congenital (present at birth) heart defects in which the heart has only one fully functioning ventricle. Hypoplastic left heart syndrome (HLHS) is a single ventricle defect.
       
    • sporadic: occurring by chance, occasionally, not inherited 
  • For the baby in utero

    Most pregnant women have fetal ultrasounds in the course of their pregnancies. If a basic (level one) ultrasound shows the possibility of heart abnormalities, a more detailed cardiac ultrasound (fetal echo) can correctly diagnose your baby’s heart defect. In addition, an advanced general (level two) ultrasound at Boston Children’s Hospital's Advanced Fetal Care Center (AFCC) can detect other anomalies, if these are present:

    • fetal echocardiography (ultrasound, fetal echocardiogram, fetal echo): electronically recorded sound waves that produce a moving picture of the heart and heart valves. A diagnosis of HLHS through prenatal cardiac ultrasound is key to planning for:
      • fetal cardiac intervention: In some select situations, our doctors can treat the baby’s heart while still in the womb.
         
      • Alternatively (or in addition), we can plan in advance for effective delivery of the baby, and for his immediate stabilization once he’s born. Upon the baby’s birth, our AFCC team brings together all the pediatric and obstetric specialists necessary for critical care, as well as support services for families.
    • fetal magnetic resonance imaging (MRI) enables us to acquire additional important anatomic information. Drawing on our expertise in precise pediatric imaging, the AFCC is setting a new standard in fetal MRI.

    For the newborn baby

    Some combination (not necessarily all) of the following medical tests will be used to diagnose HLHS and its related defects in a newborn:

    • echocardiography (ultrasound): The ultrasound on the newborn’s heart will reveal critical information about the extent of the HLHS abnormality—including the size and anatomy of the left ventricle, heart valves and other structures.
       
    • electrocardiogram EKG (EKG, ECG): An EKG evaluates the electrical activity of your child’s heart.
       
    • cardiovascular MRI (magnetic resonance imaging): A heart MRI is a non-invasive test using 3-D imaging technology produced by magnets to accurately determine the blood flow and functioning of your child's heart.
       
    • cardiac catheterization: provides detailed visual information and measurements about the structures inside the heart. Cardiac catheterization can be diagnostic-only, diagnostic and therapeutic, or diagnostic and interventional.

    Keep family and friends up-to-date during your child’s treatment by creating a free Boston Children’s Carepage


    Boston Children’s Heart Care Center 

    The Heart Care Center at Boston Children’s is one of the largest pediatric heart programs in the United States. Our staff of more than 80 pediatric cardiac specialists cares for thousands of children and adults with congenital and acquired heart defects each year, from simple to complex cases. We have experience treating rare heart problems—with results that are among the best in the world.

  • Boston Children's Hospital is known for successfully pioneering cardiac treatments. A large part of our success comes from our commitment to research—and to advancing the frontiers of what's possible in cardiac medicine through the world's largest research program at a pediatric institution.

    Shaping new advances in fetal and newborn care, our researchers work just steps away from our physicians and patients—giving doctors a vital jump-start on applying lifesaving research discoveries to real-life patient care.

    Fetal (prenatal) cardiac intervention

    Our physicians have pioneered refinements to this complex intervention, and we're the world's largest and most experienced center performing fetal cardiac interventions. Patient families come from around the world for treatment with this new approach.

    Fetal cardiac intervention is appropriate in certain select situations. If your child is a candidate, the procedure will be coordinated by your team at the Advanced Fetal Care Center (AFCC). Children's pediatric interventional cardiologists can use a needle or tiny balloon catheter to treat HLHS, as well as certain other fetal cardiac abnormalities. Balloon catheters can be inflated to open abnormal heart valves or other obstructions.

    After the baby is born, if he develops HLHS, he will have a series of three surgical interventions between a few months and a few years of age. See Fontan Sequence in this section.

    Immediate post-birth treatments

    If fetal intervention is not an option for your baby, the AFCC will plan and coordinate his postnatal care to ensure the best possible outcome for him and the family. As soon as your baby is born, Children's will begin treating him, so that we may ultimately put your child on the path to good health.

    How we treat your child depends on the extent of the disease and other variables, but initial treatments in the cardiac ICU typically include:

    • IV (intravenous) medication to keep the PDA open
    • intravenous fluids
    • for a few babies, breathing provided by a mechanical device
    • balloon atrial septostomy (usually not necessary if an atrial septal defect [ASD] is present): creates an opening in the atrial septum (wall) between the upper chambers of the heart (the left and right atria) to improve the mixing of oxygen-rich (red) blood and oxygen-poor (blue) blood
    • A few unusually ill patients will require support using a heart/lung bypass machine that circulates oxygenated blood through the baby's body

    Three staged surgeries: the Fontan Sequence

    In treating HLHS, cardiac surgeons perform three palliative procedures called the Fontan Sequence. The goals of these surgeries are to:

    • enable the fully-functioning right ventricle to do the work normally done by two ventricles
    • separate the blue (low-oxygen-content) blood (pulmonary circulation) from the red (well-oxygenated) blood (systemic circulation)
       
    • Stage I: the Norwood Procedure: Developed at Children's, the Norwood Procedure is usually performed in the first week of the baby's life. This procedureconnects the right ventricle to the aorta so that its flow will be delivered to the body through branches of the aorta.

      This procedure usually involves rebuilding a small aorta and connecting it to the ventricle. Blood flow to the lungs is provided through a tube, called the modified Blalock-Taussig Shunt, which extends from a branch of the aorta to the pulmonary artery.

      Two alternative procedures to the Norwood Procedure are sometimes performed, based on surgical preference, the size of the child and the anatomy of the defects. These are:

    the Sano modification: places a conduit between the pulmonary artery and the right  ventricle instead of the modified Blalock-Taussig Shunt

    the hybrid procedure: combines cath lab techniques, such as stenting, with surgical techniques. In this procedure, the interventional cardiologist and the cardiovascular surgeon work together.

    The hybrid procedure establishes a stable outflow by:

    • implanting a stent in the ductus arteriosus, connecting the pulmonary artery to the aorta
    • banding the branch pulmonary artery to restrict some of the pulmonary blood flow

    The hybrid procedure typically takes between 60 and 90 minutes, rather than the six-hour, more invasive Norwood. It allows for delaying the major reconstruction until Stage II, when the baby will be stronger and will have built up some immune defenses.

    The other advantage of this procedure is that it does not require the baby to be on the heart-lung machine during the surgery, which may reduce the risk of certain complications.

     Glenn shunt HLHS

    • Stage II: bi-directional Glenn Shunt: usually performed between three and eight months of age. The baby's lungs have matured enough so that blood flows through more easily, and ventricular force is no longer necessary.

      The bi-directional Glenn shunt is a direct connection between the superior vena cava and the pulmonary artery, diverting half of the blue blood directly to the lungs without the help of the ventricle.
       
    • Stage III: Fontan procedure (operation): With further lung maturity in the baby's first few years, the vessels of the lungs can now accommodate all the body's blue blood, allowing the Fontan procedure to be done.

      The procedure connects the inferior vena cava to the pulmonary artery by creating a channel (“baffle”) through the heart, or a tube alongside the heart, to direct its flow to the pulmonary artery. Now all the blue blood flows passively to the lungs, and the single ventricle pumps exclusively red blood to the body.

    A word about heart transplants: A very few institutions perform heart transplants as a primary therapy, rather than the staged Fontan procedures. At Boston Children's, we perform transplants only if patients don't respond to other surgery. Such an occurrence at Boston Children's is very unusual.

    Coordinated post-procedure care from Boston Children's AFCC

    Before, during and after each stage of your child's treatment and recovery, the AFCC's caring staff will help you to coordinate care services, appointments, counseling, social services and travel and lodging arrangements.

    At home: caring for your child after HLHS treatment

    Your child's cardiologist will follow your baby's progress closely, and will offer recommendations for post-operative follow-up care—including a nutritional program to encourage weight gain, an oral hygiene program to prevent infection, and an appropriate exercise routine.

    We'll also help you to create a longer-term care program as your baby matures into childhood and his teen years. Families who have a child with HLHS will have an ongoing relationship with their cardiologist. We will consult and treat if complications arise, and we'll advise on daily-life issues such as exercise, activity levels, nutrition and pregnancy precautions.

    As your baby recovers and grows, be sure to follow a regular program of well-baby/well-child checkups. And to the greatest extent possible, encourage your child to live normally. Even if some physical activities are limited, your child and your family can enjoy a full life together.

    Boston Children's Home Monitoring Program for your child

    An infant with single ventricle anatomy needs support with shunt-dependent blood flow between his Stage I and Stage II surgical repairs (see surgery descriptions above on this page).

    The results of the Stage I surgery have improved—with nearly 90% of infants who are cared for in experienced centers discharged home after the first stage. So we can now focus new attention on reducing the known mortality of 10 to15 percent for these infants between their Stage I and Stage II surgical repairs.

    Research shows the vital importance of a Home Monitoring Program, including daily at-home assessments of oxygen saturations and weight between the Stage I and Stage II surgeries.

    Checking your baby's daily weight:

    • alerts you and your child's providers to dehydration
    • enables appropriate immediate intervention
    • decreases the risk of sudden death at this very fragile stage in your infant's multiple-surgery sequence

    During this period between the Stage I and Stage II surgeries, your child's pediatric cardiologist and pediatrician will be in close contact with you as your child's primary home caregiver. (After your child's Stage II repair, this intensive level of home monitoring will no longer be necessary.)

    In Boston Children's Home Monitoring Program:

    • You'll be given detailed guidelines and goals for your child's:
    • growth
    • weight gain
    • oxygen saturations
       
    • You'll be instructed to call your child's cardiologist if his goals for growth, weight gain and oxygen saturation aren't met, or if:
      • your child is breathing harder, faster or is fussy beyond his baseline
      • you have any questions or concerns
         
    • You'll be supplied with a pulse oximeter and a baby scale and shown how to use both instruments. You'll measure your child's saturations twice a day and weigh the baby daily—checking against given guidelines and goals.
       
    • You'll record your baby's weights, oxygen saturations and general observations in a daily log, and will share this log with the pediatrician/cardiologist during check ups.
       
    • You'll consult your pediatrician if your child:
    • is experiencing nausea/vomiting/diarrhea
    • is not having sufficient wet diapers
    • is feverish
       
    • You'll have weekly calls with your designated pediatric nurse practitioner, who will consult with you, and who will notify your child's cardiologist of any concerns that arise.
       

    As he grows: your child's long-term outlook

    Babies with HLHS will need three surgeries in their first few years of life; and children will need follow-up care throughout their lives to ensure that their hearts continue to function adequately. They are never entirely cured of their cardiac defect, and will probably need to take heart medications.

    The most positive outcome is that a child can undergo these surgeries and not have any symptoms going forward. On the other hand, there are children whose hearts struggle with their single ventricle.

    Boston Children's cardiologists will follow your child's recovery and progress, and will provide further treatment, if needed. There is a risk of complications, including arrhythmias, heart failure and blood clots. And the potential does exist for needing a heart transplant in early or mid-adulthood.

    Thanks to sophisticated diagnoses and treatment innovations, babies with HLHS who would not have survived a mere 20 years ago now have a good chance for a full life. And the medical science on HLHS and other congenital heart defects continues to evolve and improve.

    Coping and support

    Before, during and after each stage of your child's treatment and recovery, our Advanced Fetal Care Center (AFCC) staff will help you coordinate care services, appointments, counseling, social services and travel and lodging arrangements.

    AFCC caregivers will acquaint you with the Boston Children's Center for Families, where you can find family-to-family support groups and an array of services.

    Also, our Boston Adult Congenital Heart and Pulmonary Hypertension Service (BACH) is an international center for excellence, providing long-term inpatient and outpatient care and advanced therapies for people with congenital heart disease as they progress to adulthood.

    Watch some of Boston Children's frequently used cardiac procedures in action 

  • Boston Children’s Hospital is a world pioneer in the development and use of innovative fetal cardiac intervention to treat hypoplastic left heart syndrome (HLHS). Thanks to Boston Children’s research and refinements of technique, our Advanced Fetal Care Center offers the world’s largest and most experienced fetal intervention program for HLHS.

    In addition, a significant amount of the groundbreaking cardiac research being conducted at Children’s aims to refine and advance open heart surgery and catheterization procedures to correct congenital heart defects in newborns and young children.

    Cardiac surgery research

    Members of the Boston Children’s Cardiac Surgery Research Laboratory—a multidisciplinary team of basic and applied research investigators who hold faculty appointments at Harvard Medical School—are studying the mechanisms of heart disease and new treatments for children with congenital heart defects.

    Cardiology research

    Boston Children’s is a world leader in opening new avenues of “translational research,” bringing laboratory advances to the bedside and doctor’s office as soon as possible. Senior medical staff members of the Department of Cardiology participate in clinical research activities, and many do laboratory research, as well.

    Learn more about current projects in heart research.

    Innovations

    Reawakening the heart’s regenerative capacities

    The problem: Kids with congenital heart defects sometimes suffer from heart failure, meaning their hearts can’t pump adequately. And since heart muscle has very little growth capacity after birth—nowhere near enough to fix a severe cardiac injury—heart transplant is often the sole treatment option.

    Innovative solution: Researchers in Children’s Cardiovascular Program have discovered a growth factor, called neuregulin1 (NRG1), that can reawaken the heart’s regenerative capacities, and may be able to strengthen the heart after a heart attack and in children and adults with heart failure.

    NRG1, which is involved in the initial development of the heart and nervous system, spurred heart-muscle growth and recovery of cardiac function when injected systemically in rats. Preparations are underway to test the growth factor in humans. Doctors now envision a time when patients may receive monthly infusions to build up their hearts.

    Creating new ways to perform surgery

    Problem: When surgeons perform heart surgery on a baby, they need to open the infant’s chest and stop her heart—an inva­sive, lengthy procedure that can cause life-threatening complications. Pedro del Nido, MD, chief of Cardiac Surgery at Boston Children’s, had to perform surgery on his tiny patients using this method, or come up with a way to improve it.

    Innovative solution: Del Nido decided to develop a way to perform surgery on a still-beating heart. But he needed two things that didn’t exist: superior imaging tools that could show the structures inside the heart while it’s beating, and tiny instruments to perform the intricate surgery.

    So, he bor­rowed technology from the videogame industry and developed stereo-rendered 3-D ultrasound imaging that allows surgeons to see inside the beating heart as a hologram.

    Del Nido also designed new instruments. One is a millimeter-sized tool that extends into the heart through needle-sized incisions. Using a joystick controller and real-time imaging, a surgeon can now navigate through the beating heart’s chambers to remove blockages, repair faulty valves and close leaks.

    The other new instrument is a cardioport device that allows instruments to be safely introduced into the cardiac chambers with­out the usual risks of blood loss or an air embolism.

    cardioportResults: Del Nido’s 3-D tool appears not only to provide superior imaging, but also to yield faster surgery times. Researchers using it to operate on pigs with congenital heart disease performed the procedure 44 per­cent faster than before. Dr. Del Nido’s cardioport will soon be tested in clinical trials and will facilitate further development of similarly novel instruments for heart repair.  Del Nido’s newly-developed cardioport
    will someday make possible faster, less invasive heart surgery.

    History of innovation

    In 1938, Boston Children’s cardiac surgeon Robert Gross, MD, performed the world’s first successful surgery to correct a child’s heart defect. Since that time, we have gained recognition around the globe for our leadership in pediatric cardiology and continue to make critical advances in the field. In 2014, U.S.News & World Report named Boston Children’s cardiology and cardiac surgery programs the best of any pediatric hospital in the country.

  • Avery Toole's Gift of Life

    The day after Avery Toole was born, an echocardiogram revealed that she had a serious and rare heart defect called hypoplastic left heart syndrome. Her mother, Cheryl, is a nurse in the Neonatal Intensive Care Unit at Boston Children's Hospital knew her only chance for survival was immediate open-heart surgery.

    Avery needed immediate open-heart surgery at Boston Children's, where her mother, Cheryl Toole, RN, MS, is a nurse manager. The newborn underwent the delicate procedure at just 5 days old, recovering well. Avery's parents were thrilled to bring their baby home, though they knew she had a long road ahead. Treating HLHS requires a series of surgeries. Avery had her second surgery at 6 months, followed by nine other operations, including leaky valve repairs.

    By March, 2009, repairs could no longer save Avery's heart—she needed a new one. Doctors placed the 5-year-old on a Berlin Heart, a mechanical device that does the heart's work, while she waited for a donor. Avery spent 54 days on the Berlin Heart, struggling to gain strength for surgery.

    Avery's life was saved by the heart that was donated by an amazingly generous family from Texas who had just lost their child in a car accident.

    My baby’s congenital heart defect

    By Casey BoltonParker patient HLHS

    During a routine ultrasound I had in the 20th week of my pregnancy, I learned that my soon-to-be-born son, Parker, would be born with a complex congenital heart defect (CHD) called hypoplastic left heart syndrome (HLHS).

    Parker couldn’t be born in our home state of New Hampshire because none of the local hospitals were equipped to handle his CHD, so we scheduled an induced birth in Boston, and almost immediately after birth Parker was brought to Children’s Hospital Boston’s Cardiac Intensive Care Unit (CICU).

    Parker’s first 24 hours of life were so unstable that doctors weren’t sure he’d make it. His condition was so severe that even as his parents we weren’t allowed to see him in the cardiac ICU until they could stabilize him with an emergency catheterization.

    Parker had to undergo his first heart intervention at just a few hours old. Then, at only seven days old, he underwent his first open heart surgery. His chest remained open after the surgery because his heart was so swollen; finally it was closed on Christmas Day. We spent that Christmas hoping for the best, but he was not adjusting well to the change.

    Parker

    Due to Parker’s condition, doctors said he required either three open heart surgeries or a full heart transplant. We choose the three surgeries because Children’s had a reputation of successfully performing surgery in that way.

    Originally, Parker was supposed to come home between the first and second stage of his heart operations, but unfortunately he didn’t do well after the first surgery and needed to remain hospitalized with the support of the hospital equipment and medicine in order to survive to the second stage.

    Parker had the second open heart surgery on March 3, 2008. This was one of the earliest stage II surgeries performed by cardiac surgeon Dr. Sitaram Emani.

    Thanks to everyone at Children’s, after nearly 15 weeks in the hospital, Parker was finally discharged and able to come home for the first time in his life.

    Parker is now home and he receives eight different medications, 16 times throughout the day. His next open heart surgery will be sometime between Thanksgiving and Christmas of this year. Eventually, Parker may need a heart transplant, but we are hopeful this isn’t for years to come.

    parker

    Because of Parker’s condition we have learned to cherish each and every moment. Life is no longer about milestones, but about the moments. The difference between good days and bad days no longer seems so important; we are just thankful for the days. We are forever grateful for the first-class care our son has received at Boston Children’s; we truly felt he couldn’t have been in better hands.

    The entire staff was amazing, and the dedication I felt from Dr. Sitaram Emani and Dr. David Brown goes beyond words. We were at Boston Children’s for 95 straight days and I felt like they were always available to us for assistance, guidance or to answer our questions. They never made me feel rushed or that my concerns weren’t valid.

    Seeing their dedication to and passion for helping children made me want to make sure all future parents of children with heart conditions are aware of the amazing care their baby can receive from Boston Children's Hospital.

    Parker

    When I first received Parker’s diagnosis of HLHS, I had no idea what a congenital heart defect was, never mind what HLHS was. When I left the doctor’s office that morning in tears, the only thing I had was a tissue with hypoplastic left heart syndrome written on it.

    Through our personal journey I’ve learned there just isn’t enough awareness or funding for research for CHDs. I’m amazed that the cardiologist we first saw in New Hampshire who diagnosed my baby couldn’t provide any information about his condition. I think most of my fears stemmed from the lack of education on the CHD and fear of the unknown.

    As a result I have made it a life goal to raise awareness for CHDs so other expectant parents aren’t as lost as I was. I’ve contacted many people, medical professionals and organizations in attempts to open the lines of communication about the dangers CHD.

    Thanks to work I’ve done with New Hampshire Governor John Lynch, this past February 14 was declared CHD Awareness Day in my home state. Currently, I’m working with New Hampshire State Representative Lynne Blankenbeker on having a pulse oximetry test (which could help detect signs of CHD in babies and save lives) added to medical screenings of all newborns born in New Hampshire.

    While I’m glad to have the opportunity to be a part of the fight against CHD, I am most thankful for the work and dedication of Dr. Emani, Dr. David Brown and the entire staff at Boston Children's Hospital. They have given my son a chance at life, and I could never fully repay them—I am truly blessed to have Parker and my thanks go out to Boston Children’s for keeping him in my life.

    About Hannah

    Hannah patient HLHS

    Hannah Englert was born with hypoplastic left heart syndrome (HLHS), a rare and serious congenital heart defect. Her parents knew about her condition when Hannah’s mom, Denyse, was pregnant with Hannah, so they arranged to temporarily leave their Louisiana home so Denyse could have the baby at a hospital near Boston Children’s Hospital. That way, Hannah could be immediately transferred to Children's for treatment.

    At just two days old, Hannah had her first open heart surgery at Boston Children’s. Over the next few years, the Englerts flew back to Boston Children’s for Hannah’s follow-up surgeries, sometimes staying for weeks or months at a time. Last September, they arrived for her third—and hopefully final—heart surgery. Since the procedure was complex, Hannah has been slow to bounce back, but “her little personality is getting close to what it was at home,” says Denyse.

    Despite the hardships caused by Hannah’s condition and treatment and spending so much time away from their Louisiana home, the Englerts have used Hannah’s health crisis to help others. They’ve partnered with the Louisiana Pediatric Cardiology Foundation to form a support network for parents of children with HLHS. “I want to help as much as I can,” Denyse says.

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