Materiały źródłowe

• #1 Caring for a Child with Hypoplastic Left Heart Syndrome: Parent and Medical Perspective

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8617359/

  This article, co-authored by parents of a child with hypoplastic left heart syndrome (HLHS) and his pediatric cardiology care team, discusses the parents experience of having a child with HLHS from diagnosis through living day-to-day with the heart condition as well as future directions. […] HLHS is one of the most severe forms of CHD with an estimated incidence of 0.160.36 per 1000 live births. Current management options for children born with HLHS include surgical palliation, primary transplantation, or hospice care. […] Regardless of surgical pathway chosen, these children require a minimum of three operative procedures with a high probability of multiple cardiac procedures throughout their lifetime. […] As stated by the parental question What is next?, the future is always in flux, but major areas of likely research will deal with improving neurodevelopmental and psychosocial outcomes in patients and families dealing with HLHS.

• #1 Hypoplastic left heart syndrome – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/hypoplastic-left-heart-syndrome/symptoms-causes/syc-20350599

  Hypoplastic left heart syndrome (HLHS) is a rare heart condition that a child is born with. That means it’s a congenital heart defect. In this condition, the left side of the heart doesn’t develop fully and is too small. So it can’t pump blood well. Instead, the right side of the heart must pump blood to the lungs and to the rest of the body. […] Treatment for hypoplastic left heart syndrome may include medicines, heart surgery or a heart transplant. Advances in care have improved the outlook for babies born with HLHS. […] But in babies with hypoplastic left heart syndrome, the left side can’t pump blood well. So they need medicine to keep these connections open and keep blood flowing to the body until they have heart surgery. […] With proper treatment, many babies with hypoplastic left heart syndrome (HLHS) survive. But they do need many surgeries and can have less energy and other challenges. Complications of HLHS may include: […] Children with HLHS may require a heart transplant later in life. […] Hypoplastic left heart syndrome care at Mayo Clinic.

• #1 Hypoplastic left heart syndrome: from comfort care to long-term survival | Pediatric Research

  https://www.nature.com/articles/pr2016194

  The management of hypoplastic left heart syndrome (HLHS) has changed substantially over the past four decades. […] Prior to the 1980s, there were no viable surgical options for these infants. Rather, these infants were provided supportive care and comfort measures, and most died shortly after birth. […] Fortunately, as a result of surgical and medical advances of the last four decades, many of these children now undergo a series of three operations that allow them not only to survive infancy but also to live on into early adulthood. […] Close outpatient surveillance has thus far been shown to be the most effective means of minimizing interstage attrition in children with HLHS and has therefore become a crucial component of their care. […] The interstage mortality was 0% after introduction of this home monitoring program at their institution and the improvement was sustained over a 10-y period.

• #1 Hypoplastic Left Heart Syndrome (HLHS): Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/12214-hypoplastic-left-heart-syndrome-hlhs

  Hypoplastic left heart syndrome (HLHS) is a heart condition that develops before birth (congenital). The left side of a baby’s heart doesn’t form right. This keeps blood from flowing through their heart in a normal way. Medicine can help shortly after birth, but babies with HLHS need a series of three surgeries at very young ages. […] Newborns with HLHS don’t always show symptoms immediately. Instead, symptoms may develop within a few hours or days of birth. […] Yes. First, your baby will need a medication called a prostaglandin. This keeps their ductus arteriosus open and functioning. Other medicines can help your baby’s heart work more efficiently. Your baby also may need help with breathing. […] After treatment for hypoplastic left heart syndrome, your child will need lifelong follow-up visits at least once a year with a cardiologist (heart doctor). These visits ensure that their heart, lungs and other organs continue to work properly.

• #1 Hypoplastic Left Heart Syndrome Diagnosis & Treatment | Cardinal Glennon

  https://www.ssmhealth.com/cardinal-glennon/fetal-care-institute/fetal-heart-program/heart-conditions/hypoplastic-left-heart-syndrome

  Most babies with HLHS can be delivered vaginally, unless there are obstetric indications for another mode of delivery. It is best for these babies to be delivered at a medical center where there is access to a team of pediatric cardiologists and neonatologists, along with an established Neonatal Intensive Care Unit (NICU). […] After delivery, the baby will need prostaglandin (PGE), an intravenous medication that keeps the patent ductus arteriosus (a normal connection present in babies in the womb) open. The patent ductus arteriosus (PDA) will allow the right ventricle to provide blood flow to the body. The baby will also be prepared for the first HLHS surgery, which typically occurs within a week of delivery. […] After the first surgery your baby may be fragile and have feeding difficulties because the heart is still working very hard. These babies will require a stay in the Neonatal or Pediatric Intensive Care Unit (NICU or PICU) until they overcome any feeding and breathing difficulties. Following these surgeries, your child will likely need lifelong follow-up visits with a cardiologist to monitor their progress. Children with HLHS whose hearts become weak may eventually need heart transplants.

• #1 Hypoplastic Left Heart Syndrome | Symptoms, Diagnosis & Treatment

  https://www.cincinnatichildrens.org/health/h/hlhs

  Hypoplastic left heart syndrome (HLHS) is one of the most complex cardiac defects seen in the newborn. It is one of the most challenging to manage of all congenital heart defects. […] The management of the newborn with hypoplastic left heart syndrome can be divided into the initial stabilization period and the operative / post-operative period. If the fetus has been diagnosed before delivery, stabilization measures are started immediately so the newborn does not become unstable. In newborns that are delivered and then later suspected of having hypoplastic left heart syndrome, stabilization begins while diagnostic tests are going on. The rapid stabilization of these infants must begin as soon as the diagnosis is suspected. […] Close monitoring is needed to find any organ dysfunction. It also helps to keep cardiopulmonary stability. Infants with this defect may be unpredictable and have sudden changes.

• #1 Content – Health Encyclopedia – University of Rochester Medical Center

  https://www.urmc.rochester.edu/encyclopedia/content?contenttypeid=90&contentid=p01798

  Hypoplastic left heart syndrome (HLHS) is a group of defects of the heart and large blood vessels. A child is born with this condition (congenital heart defect). It occurs when part of the heart doesn’t develop as it should during the first 8 weeks of pregnancy. […] For this reason, a baby with HLHS will not survive without surgery to fix the defect. […] Your baby will most likely be cared for in the neonatal intensive care unit (NICU). At first, they may get oxygen or be placed on a ventilator. This is to help with breathing. Your child may get IV medicine. The medicine helps the heart and lungs work. […] In most cases, surgery is used to treat hypoplastic left heart syndrome. […] Surgery typically involves at least a series of several surgeries. […] After the surgery, your baby will return to the pediatric intensive care unit (PICU) to be closely watched.

• #1 Hypoplastic Left Heart Syndrome – Pediatrics – MSD Manual Professional Edition

  https://www.msdmanuals.com/professional/pediatrics/congenital-cardiovascular-anomalies/hypoplastic-left-heart-syndrome

  Hypoplastic left heart syndrome (HLHS) accounts for 2 to 4% of congenital heart anomalies and is the second most common left heart obstructive lesion. […] All infants with hypoplastic left heart syndrome should be stabilized immediately in a neonatal ICU or pediatric cardiac ICU. Vascular access should be established rapidly via an umbilical venous catheter and/or peripheral IV, whichever is quicker. Prostaglandin E1 (beginning at 0.05 to 0.1 mcg/kg/minute IV) is infused to prevent closure of the ductus arteriosus or to reopen a constricted ductus. Neonates, particularly those who are critically ill at presentation, usually require tracheal intubation and mechanical ventilation. Metabolic acidosis is corrected via infusion of sodium bicarbonate. Severely ill neonates with cardiogenic shock may require inotropic drugs (eg, milrinone) and diuretics to improve cardiac function and control volume status.

• #1 Hypoplastic Left Heart Syndrome – Pediatrics – MSD Manual Professional Edition

  https://www.msdmanuals.com/professional/pediatrics/congenital-cardiovascular-anomalies/hypoplastic-left-heart-syndrome

  It is critical to keep pulmonary vascular resistance relatively high and systemic vascular resistance low to prevent marked pulmonary overcirculation at the expense of systemic perfusion. […] Endocarditis prophylaxis is recommended for at least 6 months after each surgical intervention and subsequently for as long as the patient remains cyanotic or has a residual defect adjacent to a surgical patch or prosthetic material. […] Definitive treatment requires staged operations.

• #1 Hypoplastic Left Heart Syndrome Part 2 | RN.com

  https://www.rn.com/blog/clinical-insights/hypoplastic-left-heart-syndrome/

  Additionally, the nurse should be vigilant for signs of inadequate systemic perfusion and low cardiac output; which may indicate a worsening in clinical status. Managing tachycardia, hypotension, decreased urine output, inadequate systemic perfusion, elevated temperature, low mixed venous oxygen saturation, metabolic acidosis, and increased serum levels of lactate have been related to better outcomes and decrease mortality. […] The complex nature of HLHS requires a multi-stage palliative repair. It is essential that nurses and other healthcare providers understand how the surgical interventions impact the patient’s cardiac anatomy, cardiac output, systemic blood flow, and perfusion.

• #1 Hypoplastic Left Heart Syndrome Part 2 | RN.com

  https://www.rn.com/blog/clinical-insights/hypoplastic-left-heart-syndrome/

  Unfortunately, hypoplastic left heart syndrome (HLHS) cannot be cured but must be palliated in such a way that the right side of the heart takes over the function of the nonexistent or severely deformed left heart. […] To decrease mortality and morbidity and ensure stronger right ventricular function the postop care of the HLHS patient must include strategies to optimize pulmonary and systemic perfusion and maximize cardiac output. […] The nurse is an essential component of postop care. Nursing interventions include a thorough assessment, providing adequate analgesia and sedation, avoiding hyperventilation, avoiding hypothermia, which increases PVR and SVR and interferes with clotting; and hyperthermia, which increases metabolic demand, increasing myocardial work and oxygen consumption.

• #1 The intensive care of infants with hypoplastic left heart syndrome | ADC Fetal & Neonatal Edition

  https://fn.bmj.com/content/90/2/F97

  Hypoplastic left heart syndrome (HLHS) is a continuum which can affect all left sided cardiac structures, from the mitral valve to the aortic arch. […] The primary goal of peri-operative care of the neonate undergoing stage 1 palliation (Norwood operation or its modifications) is to optimise systemic oxygen delivery and organ perfusion. […] In the preoperative period, systemic perfusion is critically dependent on a patent arterial duct, a non-restrictive inter-atrial communication, and good right ventricular function. […] Babies with HLHS require prostaglandin E to maintain ductal patency. […] Early intervention with prostaglandin maximises systemic perfusion, and optimises the condition of the infant before surgery. […] All neonates with HLHS should have continuous monitoring of their oxygen saturation.

• #1 The intensive care of infants with hypoplastic left heart syndrome | ADC Fetal & Neonatal Edition

  https://fn.bmj.com/content/90/2/F97

  Initial respiratory care of the neonate with known or suspected HLHS should be targeted at avoiding pulmonary overcirculation. […] The important contribution of haemoglobin to systemic oxygen delivery should not be underestimated. […] In the infant after stage 1 palliation, mixed venous saturation can be continuously monitored using a co-oximeter placed directly into the SVC at the time of surgery. […] Most patients after stage 1 palliation require inotropes to maintain cardiac contractility. […] Extracorporeal life support (ECLS) has been used to support many children with refractory cardiopulmonary dysfunction after surgery for congenital heart disease. […] Most infants require central venous access in the first days or weeks after stage 1 palliation, for drug delivery, and in some cases for parenteral nutrition.

• #1 Hypoplastic left heart syndrome: from comfort care to long-term survival | Pediatric Research

  https://www.nature.com/articles/pr2016194

  Their results have since led to the development of similar programs around the world. […] These programs typically provide families with an infant scale and pulse oximeter for their homes, and require regular phone interviews and clinic appointments aimed at detecting early warning signs before clinical deterioration can occur. […] Moreover, many centers have established interstage multidisciplinary clinics to provide regular comprehensive assessments including meticulous evaluation of nutritional status and oral-motor feeding skills by specialized dieticians and speech therapists. […] Despite these encouraging results, most centers restrict use of the hybrid procedure to their most fragile neonates and rely on the more traditional and established Norwood procedure for the majority of their children with HLHS. […] The hybrid procedure will likely need to be subjected to more rigorous study, akin to what was accomplished with the SVR Trial, before more widespread application occurs.

• #1 The Norwood Procedure (for Parents) | Nemours KidsHealth

  https://kidshealth.org/en/parents/norwood.html

  The Norwood procedure is a type of open-heart surgery for babies born with hypoplastic left heart syndrome. It is usually done in the first few weeks of life. […] Babies born with hypoplastic left heart syndrome need the Norwood procedure because their left ventricle and aorta are too small to pump blood to the body. The Norwood procedure lets the right ventricle pump blood to the body (usually it only pumps blood to the lungs). […] Babies who have the Norwood procedure usually spend 3 to 4 weeks in the hospital to recover. They get-around-the-clock care and monitoring. They also get medicines to help the heart and improve blood flow. They will keep taking some of these medicines at home. […] Children with hypoplastic left heart syndrome will need at least two more surgeries, the Glenn procedure (around age 36 months) and the Fontan procedure (around age 1836 months). […] Caring for a child after heart surgery can be overwhelming for any family. But youre not alone. The doctors, nurses, social workers, and other members of the care team are there to help you and your child.

• #1 Hypoplastic Left Heart Syndrome | Symptoms, Diagnosis & Treatment

  https://www.cincinnatichildrens.org/health/h/hlhs

  The most common treatment for hypoplastic left heart syndrome is staged reconstruction. Staged reconstruction is a series of operations, that are done to reconfigure the child’s cardiovascular system to be as efficient as possible. These surgeries do not correct the lesion. They are considered palliative. […] The Norwood surgery is one of the most complex and high risk procedures in the staged reconstruction surgeries for hypoplastic left heart syndrome. Current surgeries have led to a survival rate of 75 percent or better. […] Almost all children with hypoplastic left heart syndrome will need some cardiac medicines. All will need regular periodic follow-up visits with their cardiologist to look at their cardiac function and look for late complications such as arrhythmias.

• #1 Hypoplastic Left Heart Syndrome (HLHS) | Lurie Children’s

  https://www.luriechildrens.org/en/specialties-conditions/hypoplastic-left-heart-syndrome/

  The second stage is called a bi-directional Glenn, or hemi-Fontan operation, and consists of disconnecting the shunt to the pulmonary arteries and connecting the superior vena cava directly to the right pulmonary artery. […] The third stage of the procedures is known as a Fontan operation. This consists of connecting the inferior vena cava to the right pulmonary artery. […] The overall goals of the operation are to allow the right ventricle to pump only oxygenated blood to the body and to prevent or reduce mixing of the red and blue blood. […] At any time along your childs pathway from stage 1, 2 or 3, there may be unplanned events. Concerns may arise that will require the team to perform additional tests or procedures. […] The risks of open-heart surgery always include bleeding, infection, neurological injury and the need for cardiopulmonary bypass.

• #1 HLHS Surgery | Norton Children’s Louisville, Ky.

  https://nortonchildrens.com/services/cardiology/conditions/congenital-heart-disease/hypoplastic-left-heart-syndrome/

  Using the latest techniques, the board-certified and fellowship-trained specialists at Norton Children’s Heart Institute have the skills and experience to effectively rebuild a baby’s circulatory system with HLHS surgery, helping them to potentially live a full and productive life. […] HLHS surgery may involve a series of three surgeries. In some cases, a child with hypoplastic left heart syndrome may need a heart transplant because the case is so complex or because the heart becomes weak after HLHS surgery. […] The Glenn procedure allows blood returning from the upper part of the body to go directly into the lungs, bypassing the right ventricle. […] These surgeries don’t cure HLHS. The child will need regular follow-up visits with a pediatric cardiologist to monitor progress. When the child becomes an adult, they will need to see an adult congenital heart disease (ACHD) specialist for lifelong care. […] The Norton Children’s Heart Institute Fontan Clinic provides regular follow-up and monitoring to help spot any emerging issue early and treat it sooner, when more options may be available.

• #1 Hypoplastic Left Heart Syndrome | IntechOpen

  https://www.intechopen.com/chapters/81680

  The ductus arteriosus can be kept patent via the use of prostaglandin E1 (PGE1). […] The other means by which the ductus arteriosus can be maintained patent is through stenting of the ductus arteriosus. […] The post-operative management of the HLHS patient is dependent on which initial procedure is undertaken. […] The initial management of patients with Norwood MBTT shunt revolves again around achieving that fine balance between pulmonary and systemic flows. […] Both procedures require judicious monitoring and aggressive treatment of dysrhythmias. […] The management of patients post-operatively after a Hybrid procedure can be very similar to the way we manage patients post a Norwood procedure and MBTT shunt. […] The Glenn procedure is the second stage in the HLHS palliation. […] The third stage of palliation is called the Fontan procedure or total cavo-pulmonary anastomosis. […] The majority of Fontan patients will return from the operating room extubated. […] The post-operative Fontan patients require monitoring for high chest tube output and pleural effusions/chylothorax.

• #1 Hypoplastic Left Heart Syndrome (HLHS) in Children

  https://healthlibrary.osfhealthcare.org/Search/90,P01798

  Your baby will most likely be cared for in the neonatal intensive care unit (NICU). At first, they may get oxygen or be placed on a ventilator. This is to help with breathing. Your child may get IV medicine. The medicine helps the heart and lungs work. […] In most cases, surgery is used to treat hypoplastic left heart syndrome. Your baby’s cardiologist and cardiac surgeon will explain the risks and benefits. A heart transplant is another option. But it can be very difficult to find a donor heart for a baby. Also, the child will need to take medicines for the rest of their life to prevent rejection. […] Surgery typically involves at least a series of several surgeries. In this very complex treatment, the surgeon redirects blood flow to the lungs and the body through several connections. The surgery is done in stages. The first surgery is done shortly after birth. The second stage is done between ages 4 to 6 months, and the third stage is done between ages 18 months and 3 years. Another option for treatment of HLHS is a combination of surgery and cardiac catheterization. This is called a hybrid procedure. It accomplishes the goal of the first surgery without the need for placing your child on a heart-lung machine. This procedure is typically reserved for children who are at high risk, such as prematurity, low birth weight, and organ dysfunction.

• #1 Innovative hypoplastic left heart syndrome treatment offers hope – Children’s NationalSearchLink to: Youth with heart defects need a smooth transition to age-appropriate heart care, says AHALink to: Monika Goyal, M.D., recognized as the first endowed chai

  https://innovationdistrict.childrensnational.org/innovative-hypoplastic-left-heart-syndrome-treatment-offers-hope/

  Adopting a staged surgical strategy as the standard of care for medically fragile children with hypoplastic left heart syndrome (HLHS), a critical congenital heart defect, shows promise as an alternative care path for those who may not be ideal candidates for open heart surgery immediately after birth. […] This new surgical strategy gives the baby extra time to grow and allows doctors to collect detailed analysis of potentially treatable accompanying conditions. Furthermore, high-risk babies recover from birth trauma and have the chance to continue developing crucial organs before undergoing more traditional procedures for HLHS that require open-heart surgery with cardiopulmonary bypass. […] Advancing the standard of care for HLHS patients beyond the current best practice approach to improve outcomes for more newborns has been slow for several reasons, the authors note.

• #1 Hypoplastic Left Heart Syndrome (HLHS): Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/12214-hypoplastic-left-heart-syndrome-hlhs

  Most children with hypoplastic left heart syndrome will need heart medications. They’ll also need to take antibiotics before any other surgeries, including dental surgeries. These medications reduce the risk of endocarditis (a heart infection). […] Children born with this condition can live a healthy life with long-term monitoring from a cardiologist.

• #1 Hypoplastic Left Heart Syndrome (HLHS) in Children

  http://healthlibrary.chnola.org/MentalHealth/90,P01798

  After the surgery, your baby will return to the pediatric intensive care unit (PICU) to be closely watched. After the first surgery, you can expect your child to be in the hospital for several weeks. When your child is well enough to go home, they will most likely require oral medicines such as diuretics. […] Typically, after the first surgery, your baby will need frequent monitoring even when home. This may involve frequent phone calls with the medical team, daily checks of your baby’s oxygen levels, and daily weights. […] Babies and children who have the staged surgeries will need special care and treatment to support growth and development. These children may have physical developmental delays and will likely need extra support. […] In the long term, children with HLHS have an increased risk for complications such as heart failure and heart rhythm problems. They are also at risk for digestive and liver problems.

• #1 Hypoplastic left heart syndrome // Middlesex Health

  https://middlesexhealth.org/learning-center/diseases-and-conditions/hypoplastic-left-heart-syndrome

  After surgery or a transplant, a baby needs lifelong care with a heart doctor trained in congenital heart disease to watch for complications. Your child may need further treatment or other medicines for these complications. […] Some children may need to take antibiotics before certain dental or other procedures to help prevent infections. Ask your child’s healthcare professional if your child needs preventive antibiotics. Some children also may need to limit physical activity.

• #1 Hypoplastic left heart syndrome (HLHS) – Children’s Health Cardiology

  https://www.childrens.com/specialties-services/conditions/hypoplastic-left-heart-syndrome

  We offer all the care your child needs to enjoy their healthiest life possible. […] As experts in hypoplastic left heart syndrome, our team is well-positioned to guide you and your child through the various surgeries and to care for your child later in life. […] Your child needs lifelong monitoring by a cardiologist. […] When they become an adult, they can transition to our Adult Congenital Heart Disease Program. […] A child with hypoplastic left heart syndrome is at risk for developing an arrhythmia (abnormal heart rhythm), leaky heart valves or other heart problems that require treatment. […] Even after surgery, your child only has half of a working heart. […] Your child’s exercise capacity will be about two-thirds of normal levels. […] Although some physical activities may be safe, it’s best to follow the advice of your child’s cardiologist. […] Most children need to limit involvement in contact sports or other strenuous physical activities.

• #1 Hypoplastic Left Heart Syndrome (HLHS) in Children – Stanford Medicine Children’s Health

  https://www.stanfordchildrens.org/en/topic/default?id=hypoplastic-left-heart-syndrome-hlhs-in-children-90-P01798

  Babies and children who have the staged surgeries will need special care and treatment to support growth and development. These children often have physical developmental delays and will likely need extra support. […] Your child will need regular follow-up care at a center specializing in this type of congenital heart care.

• #1 Caring for a Child with Hypoplastic Left Heart Syndrome: Parent and Medical Perspective

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8617359/

  Current guidelines recommend early screening and intervention to maximize developmental outcomes. […] Future studies will need to determine how to best support not only the patient with HLHS but also those around the patient dealing with the lifelong impact of this diagnosis. […] It is only via the partnership between families and the medical field that progress will continue to be made to improve outcomes in this complicated patient population.

• #1 Hypoplastic Left Heart Syndrome (HLHS) in Children | Valley Children’s Healthcare

  https://www.valleychildrens.org/heart/conditions/hypoplastic-left-heart-syndrome-in-children

  Surgery typically involves at least a series of several surgeries. In this very complex treatment, the surgeon redirects blood flow to the lungs and the body through several connections. The surgery is done in stages. The first surgery is done shortly after birth. The second stage is done between ages 4 to 6 months, and the third stage is done between ages 18 months and 3 years. […] After the surgery, your baby will return to the pediatric intensive care unit (PICU) to be closely watched. After the first surgery, you can expect your child to be in the hospital for several weeks. […] Typically, after the first surgery, your baby will need frequent monitoring even when home. This may involve frequent phone calls with the medical team, daily checks of your baby’s oxygen levels, and daily weights.

• #1 Hypoplastic Left Heart Syndrome (HLHS) in Children

  https://healthlibrary.osfhealthcare.org/Search/90,P01798

  After the surgery, your baby will return to the pediatric intensive care unit (PICU) to be closely watched. After the first surgery, you can expect your child to be in the hospital for several weeks. When your child is well enough to go home, they will most likely require oral medicines such as diuretics. In addition, your baby may require assistance with feeding using a feeding tube. The healthcare provider may recommend pain medicine such as acetaminophen or ibuprofen to keep your child comfortable. Your child’s healthcare provider will discuss pain control before your baby goes home. […] Typically, after the first surgery, your baby will need frequent monitoring even when home. This may involve frequent phone calls with the medical team, daily checks of your baby’s oxygen levels, and daily weights. Many centers have specialized programs that help families and train them in how to care for the baby at home. If any special treatments are to be given at home, the nursing staff will make sure that you or a home health agency is able to provide them.

• #1 Hypoplastic Left Heart Syndrome (HLHS) | Prognosis & Treatment | Children’s Wisconsin

  https://childrenswi.org/medical-care/herma-heart/conditions/hypoplastic-left-heart-syndrome

  After each surgery, infants will return to the cardiac intensive care unit (CICU) to be closely monitored during recovery. […] Your child will be kept as comfortable as possible with several different medications; some which relieve pain, and some which relieve anxiety. The staff will also be asking for your input as to how best to soothe and comfort your child. […] If any special treatments are to be given at home, the nursing staff will ensure that you are able to provide them, or a home health agency may assist you.

• #1 Hypoplastic Left Heart Syndrome (HLHS): Overview and Hybrid Treatment | Nationwide Children’s Hospital

  https://www.nationwidechildrens.org/conditions/hypoplastic-left-heart-syndrome

  For all babies, growth and nutrition are an important part of their care. […] If your child cant safely eat enough by mouth in order to grow, they may need a G-tube. […] Your baby will go to the cardiac step-down unit (H4A) when they are off the breathing machine and can handle heart medicines and feedings. […] You will need to do CPR training in the Columbus Blue Jackets Family Resource Center before you leave. […] Your cardiologist will follow up with your babys heart needs even after they are discharged from the hospital.

• #1 Hypoplastic Left Heart Syndrome (HLHS) | Lurie Children’s

  https://www.luriechildrens.org/en/specialties-conditions/hypoplastic-left-heart-syndrome/

  Our care team is trained to assess and quickly manage these issues if they occur. […] Children with hypoplastic left heart syndrome require lifelong follow-up appointments with a pediatric cardiologist and ultimately transition to an adult congenital heart disease specialist. […] Research has shown that it is critically important to have an interstage home monitoring (IHM) program, that includes daily, at home assessments of oxygen saturations and weight between the stage 1 and stage 2 surgeries. […] Daily home pulse oximeter checks alert the team of any sudden or changing patterns in the oxygenation. […] Parents have been instructed to call if any one of these red flags is seen at home.

• #1 Hypoplastic left heart syndrome Information | Mount Sinai – New York

  https://www.mountsinai.org/health-library/diseases-conditions/hypoplastic-left-heart-syndrome

  If left untreated, hypoplastic left heart syndrome is fatal. Survival rates for the staged repair continue to rise as surgery techniques and care after surgery improve. […] Contact your health care provider right away if your infant: Eats less (decreased feeding), Has blue (cyanotic) skin, Has new changes in breathing patterns.

• #1 Hypoplastic Left Heart Syndrome Treatment | UVA Health Children’s

  https://childrens.uvahealth.com/services/heart/chd/hypoplastic-left-heart

  Finding out your baby has hypoplastic left heart syndrome (HLHS) can be devastating. […] At UVA Health Childrens, you’ll see top heart surgeons at the #1 childrens hospital in Virginia. […] Our experts perform a series of three surgeries to create new paths for oxygen-rich blood to reach your childs body and oxygen-poor blood to reach their lungs. […] After this surgery, your baby will stay in our NICU until they’re recovered. There, doctors trained on the specifics of newborn care will make sure that your baby’s recovery goes as smoothly as possible. There will be round-the-clock skilled nursing care, and of course, parents are a valued part of each child’s care team. […] Caring for an infant with a congenital heart defect requires planning. […] At UVA Health Children’s, you can trust your infant to our safe and secure neonatal intensive care unit, or NICU. We’ve earned national recognition for our excellence in caring for the tiniest babies.

• #1 Hypoplastic Left Heart Syndrome (HLHS) (for Parents) | Nemours KidsHealth

  https://kidshealth.org/en/parents/hypoplastic-heart.html

  Hypoplastic left heart syndrome (HLHS) is a problem that happens when the left side of a babys heart doesn’t form as it should. Its smaller than normal and cant pump enough blood to the body. After the baby is born, doctors can treat the problem with medicines and several surgeries. Some babies will need a heart transplant. […] To treat most babies with hypoplastic left heart syndrome, doctors: Give medicine called prostaglandin to keep the ductus arteriosus open so the right ventricle can continue pumping blood out to the body. Do three surgeries: the Norwood procedure, Glenn procedure, and Fontan procedure. These are done in order, and start in the first 2 weeks of life, before a baby goes home from the hospital. […] Children with hypoplastic left heart syndrome need intensive medical care from birth. Theyll have many follow-up doctor visits and tests, as well as surgery. At times this might feel overwhelming. You don’t have to go it alone. The doctors, nurses, social workers, and other members of the care team are there to help you and your child. Talk to any of them about resources that can help your family.

• #1 Hypoplastic left heart syndrome – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/hypoplastic-left-heart-syndrome/diagnosis-treatment/drc-20350605

  Adults who were born with hypoplastic left heart syndrome (HLHS) need to see a heart doctor trained in congenital heart disease in adults. Recent advances in surgical care have helped children with HLHS grow into adulthood. So it’s not yet clear what challenges an adult with the heart condition might have. Adults need regular, lifelong follow-up care to watch for changes in the condition.

• #1 Hypoplastic Left Heart Syndrome / HLHS | Fetal Care Center

  https://www.cincinnatichildrens.org/service/f/fetal-care/conditions/hlhs

  For a baby with HLHS and RAS, immediate treatment would include placing a catheter in the baby’s heart to create or enlarge the internal communication between the left and right atria and facilitate the flow of blood out of the lungs. This procedure is known as a catheter-based atrial septoplasty. […] Despite catheter based atrial septoplasty, newborns with HLHS and RAS have a higher mortality rate than those without RAS. This may be due to changes in the pulmonary blood vessels and heart tissue that result from chronic obstruction of blood flow from the lungs during fetal life. Decompressing the left atrium and improving shunting of blood from the left to right atrium could promote more normal development of the heart muscles and vessels and improve overall outcome for newborns. […] Strategies to provide immediate access to perinatal cardiac intervention, such as delivery of the baby in the cardiac catheterization laboratory, may minimize the severe instability, low oxygen levels and acidosis that occur in these newborns after birth.

• #1 The intensive care of infants with hypoplastic left heart syndrome | ADC Fetal & Neonatal Edition

  https://fn.bmj.com/content/90/2/F97

  Infants after stage 1 palliation require close and regular monitoring, with regular medical review. […] Prenatal diagnosis has allowed for more careful planning of delivery and immediate postnatal care of infants with HLHS. […] The key to the intensive care of infants after stage 1 palliation is to optimise systemic oxygen delivery, and to act early on any disturbances of this.

• #2 Hypoplastic Left Heart Syndrome – Pediatrics – MSD Manual Professional Edition

  https://www.msdmanuals.com/professional/pediatrics/congenital-cardiovascular-anomalies/hypoplastic-left-heart-syndrome

  Hypoplastic left heart syndrome (HLHS) accounts for 2 to 4% of congenital heart anomalies and is the second most common left heart obstructive lesion. […] All infants with hypoplastic left heart syndrome should be stabilized immediately in a neonatal ICU or pediatric cardiac ICU. Vascular access should be established rapidly via an umbilical venous catheter and/or peripheral IV, whichever is quicker. Prostaglandin E1 (beginning at 0.05 to 0.1 mcg/kg/minute IV) is infused to prevent closure of the ductus arteriosus or to reopen a constricted ductus. Neonates, particularly those who are critically ill at presentation, usually require tracheal intubation and mechanical ventilation. Metabolic acidosis is corrected via infusion of sodium bicarbonate. Severely ill neonates with cardiogenic shock may require inotropic drugs (eg, milrinone) and diuretics to improve cardiac function and control volume status.

• #2 Hypoplastic Left Heart Syndrome (HLHS) — THE NURSE NATALIE

  https://www.nicunursenatalie.com/notes/hypoplastic-left-heart-syndrome-hlhs

  Hypoplastic left heart syndrome (HLHS) is a severe congenital heart defect characterized by a small (underdeveloped) and functionally inadequate left ventricle. […] HLHS is often fatal without early intervention and immediate treatment. Medications may help stabilize the baby initially, but surgery will be absolutely necessary and vital for survival. Open-heart surgery is performed in order to redirect the oxygen-rich and oxygen-poor blood. […] After the baby is discharged, pediatric cardiologists will continue to follow him/her well into young adulthood, coordinating care as needed.

• #2 Hypoplastic Left Heart Syndrome (HLHS) | Conditions | UCSF Benioff Children’s Hospitals

  https://www.ucsfbenioffchildrens.org/conditions/hypoplastic-left-heart-syndrome-hlhs

  Babies with hypoplastic left heart syndrome may seem normal at birth but become severely ill soon after birth. […] Treatment for hypoplastic left heart syndrome requires either a three-step surgical procedure called staged palliation or a heart transplant. […] Hypoplastic left heart syndrome sufferers will require life-long cardiac care as well as medication.

• #2 Hypoplastic Left Heart Syndrome | Symptoms, Diagnosis & Treatment

  https://www.cincinnatichildrens.org/health/h/hlhs

  Hypoplastic left heart syndrome (HLHS) is one of the most complex cardiac defects seen in the newborn. It is one of the most challenging to manage of all congenital heart defects. […] The management of the newborn with hypoplastic left heart syndrome can be divided into the initial stabilization period and the operative / post-operative period. If the fetus has been diagnosed before delivery, stabilization measures are started immediately so the newborn does not become unstable. In newborns that are delivered and then later suspected of having hypoplastic left heart syndrome, stabilization begins while diagnostic tests are going on. The rapid stabilization of these infants must begin as soon as the diagnosis is suspected. […] Close monitoring is needed to find any organ dysfunction. It also helps to keep cardiopulmonary stability. Infants with this defect may be unpredictable and have sudden changes.

• #2 Hypoplastic Left Heart Syndrome (HLHS) in Children – Stanford Medicine Children’s Health

  https://www.stanfordchildrens.org/en/topic/default?id=hypoplastic-left-heart-syndrome-hlhs-in-children-90-P01798

  Your baby will most likely be cared for in the neonatal intensive care unit (NICU). At first, he or she may be placed on oxygen, and possibly even on a ventilator. This is to help with breathing. Your child may get IV medicine. The medicine helps the heart and lungs work. […] After the surgery, your baby will return to the intensive care unit (ICU) to be closely watched. After the first surgery, you can expect your child to be in the hospital for 3 to 4 weeks. When your child is well enough to go home, the healthcare provider may recommend pain medicine, such as acetaminophen or ibuprofen, to keep your child comfortable. Your child’s healthcare provider will discuss pain control before your baby goes home. […] If any special treatments are to be given at home, the nursing staff will make sure that you or a home health agency are able to provide them.

• #2 Hypoplastic Left Heart Syndrome (HLHS): Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/12214-hypoplastic-left-heart-syndrome-hlhs

  Hypoplastic left heart syndrome (HLHS) is a heart condition that develops before birth (congenital). The left side of a baby’s heart doesn’t form right. This keeps blood from flowing through their heart in a normal way. Medicine can help shortly after birth, but babies with HLHS need a series of three surgeries at very young ages. […] Newborns with HLHS don’t always show symptoms immediately. Instead, symptoms may develop within a few hours or days of birth. […] Yes. First, your baby will need a medication called a prostaglandin. This keeps their ductus arteriosus open and functioning. Other medicines can help your baby’s heart work more efficiently. Your baby also may need help with breathing. […] After treatment for hypoplastic left heart syndrome, your child will need lifelong follow-up visits at least once a year with a cardiologist (heart doctor). These visits ensure that their heart, lungs and other organs continue to work properly.

• #2 Hypoplastic Left Heart Syndrome | IntechOpen

  https://www.intechopen.com/chapters/81680

  Hypoplastic left heart syndrome (HLHS) is a complex congenital heart disease (CHD) involving hypoplasia of the left ventricle (LV), aorta (Ao), and mitral valve. […] The causes for such high morbidity and mortality are not well understood, but the majority of deaths are directly or indirectly related to cardiovascular causes. […] Most HLHS survivors suffer ongoing morbidity, with a substantial portion of these patients developing heart failure over time, as well as neurodevelopmental delay and neurocognitive impairment that can significantly degrade the health-related quality of life. […] Adequate pre-operative management of HLHS requires knowledge of the fetal circulation in this disorder. […] In the postnatal period, we need an adequate interatrial communication, widely patent ductus arteriosus and a balanced pulmonary vascular resistance (PVR) to systemic vascular resistance (SVR).

• #2 Hypoplastic Left Heart Syndrome (HLHS) | Lurie Children’s

  https://www.luriechildrens.org/en/specialties-conditions/hypoplastic-left-heart-syndrome/

  The second stage is called a bi-directional Glenn, or hemi-Fontan operation, and consists of disconnecting the shunt to the pulmonary arteries and connecting the superior vena cava directly to the right pulmonary artery. […] The third stage of the procedures is known as a Fontan operation. This consists of connecting the inferior vena cava to the right pulmonary artery. […] The overall goals of the operation are to allow the right ventricle to pump only oxygenated blood to the body and to prevent or reduce mixing of the red and blue blood. […] At any time along your childs pathway from stage 1, 2 or 3, there may be unplanned events. Concerns may arise that will require the team to perform additional tests or procedures. […] The risks of open-heart surgery always include bleeding, infection, neurological injury and the need for cardiopulmonary bypass.

• #2 Hypoplastic Left Heart Syndrome | IntechOpen

  https://www.intechopen.com/chapters/81680

  The ductus arteriosus can be kept patent via the use of prostaglandin E1 (PGE1). […] The other means by which the ductus arteriosus can be maintained patent is through stenting of the ductus arteriosus. […] The post-operative management of the HLHS patient is dependent on which initial procedure is undertaken. […] The initial management of patients with Norwood MBTT shunt revolves again around achieving that fine balance between pulmonary and systemic flows. […] Both procedures require judicious monitoring and aggressive treatment of dysrhythmias. […] The management of patients post-operatively after a Hybrid procedure can be very similar to the way we manage patients post a Norwood procedure and MBTT shunt. […] The Glenn procedure is the second stage in the HLHS palliation. […] The third stage of palliation is called the Fontan procedure or total cavo-pulmonary anastomosis. […] The majority of Fontan patients will return from the operating room extubated. […] The post-operative Fontan patients require monitoring for high chest tube output and pleural effusions/chylothorax.

• #2 Single Ventricle Defects | American Heart Association

  https://www.heart.org/en/health-topics/congenital-heart-defects/about-congenital-heart-defects/single-ventricle-defects

  In hypoplastic left heart syndrome (HLHS), the heart’s left side — including the aorta, aortic valve, left ventricle and mitral valve — is underdeveloped. […] The baby often seems normal at birth but comes to medical attention within a few days of birth as the ductus closes. The baby may appear ashen, have rapid and difficult breathing and have difficulty feeding. This heart defect is usually fatal within the first days or month of life unless it’s treated. […] Although this defect isn’t correctable, some babies can be treated with a series of operations, or heart transplantation. Until an operation is performed, the ductus arteriosus is kept open by intravenous medication. […] Children with HLHS require lifelong follow-up by a cardiologist for repeated checks of how their heart is working. Virtually all children with HLHS will require heart medicines, heart catheterization and additional surgery. […] Children with HLHS are at increased risk for developing endocarditis. Ask your pediatric cardiologist about your child’s need to take antibiotics before certain dental procedures to help prevent endocarditis.

• #2 Hypoplastic Left Heart Syndrome (HLHS) in Children

  https://healthlibrary.osfhealthcare.org/Search/90,P01798

  Babies and children who have the staged surgeries will need special care and treatment to support growth and development. These children may have physical developmental delays and will likely need extra support. Some children will need a heart transplant to survive into adulthood. They will also need regular follow-up care at a center specializing in this type of congenital heart care. […] After the surgeries, children will need special care and to be monitored for complications.

• #2

  https://link.springer.com/article/10.1007/s40119-021-00246-2

  As stated by the parental question What is next?, the future is always in flux, but major areas of likely research will deal with improving neurodevelopmental and psychosocial outcomes in patients and families dealing with HLHS. […] Current guidelines recommend early screening and intervention to maximize developmental outcomes. […] Future studies will need to determine how to best support not only the patient with HLHS but also those around the patient dealing with the lifelong impact of this diagnosis.

• #2 Caring for a Child with Hypoplastic Left Heart Syndrome: Parent and Medical Perspective

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8617359/

  Current guidelines recommend early screening and intervention to maximize developmental outcomes. […] Future studies will need to determine how to best support not only the patient with HLHS but also those around the patient dealing with the lifelong impact of this diagnosis. […] It is only via the partnership between families and the medical field that progress will continue to be made to improve outcomes in this complicated patient population.

• #2 Hypoplastic Left Heart Syndrome (HLHS) (for Parents) | Nemours KidsHealth

  https://kidshealth.org/en/parents/hypoplastic-heart.html

  Hypoplastic left heart syndrome (HLHS) is a problem that happens when the left side of a babys heart doesn’t form as it should. Its smaller than normal and cant pump enough blood to the body. After the baby is born, doctors can treat the problem with medicines and several surgeries. Some babies will need a heart transplant. […] To treat most babies with hypoplastic left heart syndrome, doctors: Give medicine called prostaglandin to keep the ductus arteriosus open so the right ventricle can continue pumping blood out to the body. Do three surgeries: the Norwood procedure, Glenn procedure, and Fontan procedure. These are done in order, and start in the first 2 weeks of life, before a baby goes home from the hospital. […] Children with hypoplastic left heart syndrome need intensive medical care from birth. Theyll have many follow-up doctor visits and tests, as well as surgery. At times this might feel overwhelming. You don’t have to go it alone. The doctors, nurses, social workers, and other members of the care team are there to help you and your child. Talk to any of them about resources that can help your family.

• #2 Hypoplastic Left Heart Syndrome Part 2 | RN.com

  https://www.rn.com/blog/clinical-insights/hypoplastic-left-heart-syndrome/

  Unfortunately, hypoplastic left heart syndrome (HLHS) cannot be cured but must be palliated in such a way that the right side of the heart takes over the function of the nonexistent or severely deformed left heart. […] To decrease mortality and morbidity and ensure stronger right ventricular function the postop care of the HLHS patient must include strategies to optimize pulmonary and systemic perfusion and maximize cardiac output. […] The nurse is an essential component of postop care. Nursing interventions include a thorough assessment, providing adequate analgesia and sedation, avoiding hyperventilation, avoiding hypothermia, which increases PVR and SVR and interferes with clotting; and hyperthermia, which increases metabolic demand, increasing myocardial work and oxygen consumption.

• #3 Hypoplastic left heart syndrome // Middlesex Health

  https://middlesexhealth.org/learning-center/diseases-and-conditions/hypoplastic-left-heart-syndrome

  A baby born with hypoplastic left heart syndrome (HLHS) needs urgent treatment. Treatment can include many surgeries or a heart transplant. Medicines and other therapies are used to manage symptoms before heart surgery. […] Talk with your child’s healthcare professional about treatment options for your child. […] The medicine alprostadil (Prostin VR Pediatric) is used to keep the ductus arteriosus open. It typically closes in all babies soon after birth. But in babies with hypoplastic left heart syndrome, the ductus needs to stay open so that blood can go to the rest of the body. […] While waiting for surgery or a heart transplant, a baby with hypoplastic left heart syndrome may be given medicine and have these treatments: Breathing help. Babies who have trouble breathing may need help from a breathing machine called a ventilator.

• #3

  https://link.springer.com/article/10.1007/s40119-021-00246-2

  As stated by the parental question What is next?, the future is always in flux, but major areas of likely research will deal with improving neurodevelopmental and psychosocial outcomes in patients and families dealing with HLHS. […] Current guidelines recommend early screening and intervention to maximize developmental outcomes. […] Future studies will need to determine how to best support not only the patient with HLHS but also those around the patient dealing with the lifelong impact of this diagnosis.

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