Materiały źródłowe

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

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

  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. […] If hypoplastic left heart syndrome is found before birth, healthcare professionals usually recommend giving birth at a hospital with a cardiac surgery center. […] 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.

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

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

  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. […] Children with HLHS may require a heart transplant later in life. […] 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 the need for more heart surgery or a heart transplant.

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

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

  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. […] Catheters (tubes that carry medicine) are placed, in the umbilical blood vessels. This allows medications to be given and blood to be drawn for testing. An infusion of prostaglandin, a medicine that keeps the patent ductus arteriosus from closing, is started. This medicine keeps the pathway open for blood to reach the body from the right ventricle.

• #4 Hypoplastic left heart syndrome | Health Library | Memorial Health System

  https://www.mhsystem.org/health-library/con-20155127/

  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. […] 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. […] 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. Fluids through a vein. A baby might receive fluids through a tube inserted into a vein. These are called intravenous (IV) fluids. Feeding tube. Babies who have trouble feeding or who tire while feeding can be fed through a feeding tube.

• #5 Hypoplastic Left Heart Syndrome (HLHS) | Boston Children’s Hospital

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

  What are the treatment options for HLHS? […] A small number of babies with hypoplastic left heart syndrome may benefit from intervention before birth. Our highly trained cardiologists can use a needle or tiny balloon catheter to open abnormal heart valves or other obstructions. […] If fetal intervention is not an option for your baby, treatments after birth will focus on stabilizing your baby and may include: Medication to keep the patent ductus arteriosus (PDA) open, Intravenous fluids through a tube inserted into a vein, A feeding tube if your baby has difficulty feeding, Breathing assistance provided by a ventilator to ensure adequate oxygen, A balloon atrial septostomy procedure to create an opening in the wall between the upper chambers of the heart to improve mixing of oxygen-rich blood and oxygen-poor blood.

• #6 Pediatric Hypoplastic Left Heart Syndrome Treatment & Management: Approach Considerations, Medical Care, Surgical Care

  https://emedicine.medscape.com/article/890196-treatment

  If a diagnosis of hypoplastic left heart syndrome is suspected, start prostaglandin E1 infusion immediately to establish ductal patency and ensure adequate systemic perfusion. […] Correction of metabolic acidosis with sodium bicarbonate infusion is essential in early management. This therapy is futile if the ductus arteriosus remains constricted. […] Inotropic support is indicated only in severely ill neonates with concurrent sepsis or profound cardiogenic shock and acidosis. The administration of inotropes can adversely affect the balance between pulmonary and systemic vascular resistance. […] Consider diuretics to manage pulmonary overcirculation before surgery. Agents commonly used include furosemide and spironolactone. […] Consult a pediatric cardiologist, pediatric cardiovascular surgeon, genetic specialist if a chromosomal abnormality is suspected, and an interventional pediatric cardiologist.

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

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

  Treatment for hypoplastic left heart syndrome requires either a three-step surgical procedure called staged palliation or a heart transplant. […] Staged palliation is considered one of the major achievements of congenital heart surgery in recent years. The survival rate for children at age 5 is about 70 percent and most of these children have normal growth and development. This three-step surgery procedure is designed to create normal blood flow in and out of the heart, allowing the body to receive the oxygenated blood it needs. […] Heart transplant is another option for infants with hypoplastic left heart syndrome. However, suitable donor hearts for babies are often in short supply. […] Hypoplastic left heart syndrome sufferers will require life-long cardiac care as well as medication. They also will be more prone to heart valve infections, called endocarditis, and will need to take antibiotics before surgery or dental treatment.

• #8 About Hypoplastic Left Heart Syndrome | Congenital Heart Defects (CHDs) | CDC

  https://www.cdc.gov/heart-defects/about/hypoplastic-left-heart-syndrome.html

  Surgical repairs for HLHS are not a cure. […] Soon after a baby with HLHS is born, multiple surgeries done in a particular order are needed. These surgeries will help increase blood flow to the body and bypass the poorly functioning left side of the heart. The surgeries help the right ventricle become the main pumping chamber for blood to reach the body. […] These surgeries do not cure HLHS but help restore heart function. […] If the HLHS defect is very complex, or the heart becomes weak after the surgeries, a heart transplant may be needed. Infants who receive a heart transplant will need to take medicines for the rest of their lives. These medications may prevent their body from rejecting the new heart.

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

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

  There are three treatment options that have been used for children with hypoplastic left heart syndrome. […] 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 first surgery in the staged approach is known as the Norwood operation. It is usually done in the first week of life. With the Norwood operation, the right ventricle becomes the main ventricle pumping to the body. […] The next surgeries in the staged reconstruction plan are: The bi-directional Glenn procedure, done around 3 to 6 months of age, and the Fontan surgery. […] 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.

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

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

  Norwood procedure: Babies with HLHS need Norwood surgery within the first two weeks of life. During the procedure, surgeons: Reconstruct your child’s underdeveloped aorta to provide blood flow to their body. Place a shunt (tube) to reroute blood either from their right ventricle or aorta to their pulmonary arteries, which go to their lungs. Create a connection between the upper chambers of their heart. This drains oxygen-rich blood from their lungs to supply their body. […] Bidirectional Glenn shunt operation: At 4 to 6 months of age, babies need a second operation. During the Glenn procedure, surgeons: Remove the old shunt. Place a new shunt to attach your baby’s superior vena cava to their pulmonary arteries. The superior vena cava (SVC) is a large vein that carries oxygen-poor blood from their upper body to their heart. Use this shunt to reduce the strain on your child’s right ventricle by letting blood flow right into their lungs.

• #11 Hypoplastic Left Heart Syndrome (HLHS) | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/hypoplastic-left-heart-syndrome-hlhs

  The Fontan operation is based on connecting the veins from the body directly to the arteries to the lungs. […] It’s a more efficient connection because the tube is a very specific size, and the blood going through there is not turbulent. […] The surgery for hypoplastic left heart syndrome has evolved since the early 1980s and continues to evolve. […] So really, a child who survived all this in the late s is very different from a child having surgery today. So we have a moving target because we’re getting better at what we do.

• #12 Hypoplastic Left Heart Syndrome (HLHS) | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/hypoplastic-left-heart-syndrome-hlhs

  The second procedure, known as the hemi-Fontan or Glenn operation usually occurs within six months of birth. During this surgery the superior vena cava a large vein that carries deoxygenated blood from the upper body into the heart is disconnected from the heart and attached to the pulmonary artery. After this operation, deoxygenated blood from the upper body goes directly to the lungs without passing through the heart. […] This operation, called the Fontan procedure, occurs at approximately 1 1/2 to 3 years of age. During this surgery the inferior vena cava, a large vein that carries deoxygenated blood from the lower body into the heart, is disconnected from the heart and attached to the pulmonary artery. After this operation, all of the deoxygenated blood from the body goes directly to the lungs without passing through the heart.

• #13 Hypoplastic left heart syndrome: Prognosis and management options

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2817797/

  Hospital survival rates reported are 94% to 98% following the hemi-Fontan procedure and 86% to 94% following the Fontan procedure. […] Cardiac transplantation offers the potential for a normal cardiovascular physiology over the limitations of the single ventricle physiology achieved after multi-stage reconstruction. […] Management during the waiting period can also be challenging. […] Moderate hypoxemia is far better tolerated than hyperoxia in infants with HLHS, which results in hypoperfusion acidosis. […] Palliative or comfort care continues to be a common option chosen by the parents of HLHS infants. […] Surgical therapy provides hope for survival with this condition, but the long term prognosis is still unknown and more investigations on long term outcomes are warranted.

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

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

  Fontan procedure: Between 18 months and 4 years of age, babies need a final surgery. This procedure allows all blood returning from their body to go straight to their lungs instead of mixing in their heart. During a Fontan procedure, surgeons will connect your baby’s inferior vena cava (IVC) to their pulmonary arteries. Similar to the SVC, the IVC is the large vein that carries oxygen-poor blood from your child’s lower body to their heart. […] Sometimes, your baby’s surgeon may recommend a heart transplant instead of the three-part surgery series. However, babies with heart transplants need lifelong medications. […] Operating during pregnancy is not an option for treating hypoplastic left heart syndrome. A fetal surgeon may choose to operate only to correct some conditions that might be associated with poor HLHS outcomes in babies who are developing HLHS.

• #15 Hypoplastic Left Heart Syndrome (HLHS) | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/hypoplastic-left-heart-syndrome-hlhs

  Our current strategy for reconstruction of the heart for HLHS involves three stages, one done initially at birth, one at approximately three to six months of age, and the third stage at approximately two to three years of age. […] We like to do that surgery in the first week after birth, but it can be a day, two days, three days. It just depends on how stable the baby is. […] In HLHS, the aorta is usually too small. So we correct that by putting a patch on to enlarge it. […] In addition, you have to prevent blood from backing up inside the left side of the heart, so you have to cut out the partition between the two upper chambers of the heart. […] The third-stage operation is a variant of the Fontan operation. […] There are two types of Fontan operations. One is called the Lateral Tunnel Fontan, in which a baffle is placed inside the heart to redirect the blood flow from the lower part of the body to the lungs. The other is called the Extra Cardiac Fontan, where we actually use a tube that brings the blood outside the heart, up to the pulmonary arteries.

• #16 Hypoplastic left heart syndrome: MedlinePlus Medical EncyclopediaLock

  https://medlineplus.gov/ency/article/001106.htm

  Stage III, the final step, is called the Fontan procedure. The rest of the veins that carry blue blood from the body (the inferior vena cava) are connected directly to the blood vessels to the lungs. The right ventricle now serves only as the pumping chamber for the body (no longer the lungs and the body). This surgery is usually performed when the baby is 18 months to 4 years old. After this final step, the child is no longer cyanotic and has a normal oxygen level in the blood. […] Some doctors consider heart transplantation an alternative to the 3 step surgery. But there are few donated hearts available for small infants.

• #17 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/

  A recently published study in the Journal of Thoracic and Cardiovascular Surgery is a unique report of outcomes for infants treated using a staged surgical approach such as the “hybrid strategy.” […] 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.

• #18 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. […] 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. One treatment is a heart transplant. But it is very difficult to find a donor heart for a baby. For this reason, transplant is not often done as the first-line treatment. […] Surgery typically involves a series of at least 3 surgeries. In this very complex treatment, the surgeon redirects blood flow to the lungs and the body with several connections. The surgery is done in stages. The first surgery is done shortly after birth. The second is done at about ages 3 to 6 months. The third is done at about ages 18 months to age 4. 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.

• #19 Hypoplastic Left Heart Syndrome | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/23285

  During the echocardiogram for neonates suspected of having HLHS, the atrial septum will be carefully evaluated. In many cases, a catheter-based or surgical septostomy is required shortly after birth to relieve obstruction to left atrial flow. […] Survival for neonates with HLHS depends on a series of 3 palliative surgeries aimed at establishing a Fontan-type physiology, which separates systemic and pulmonary circulations. […] The first stage, performed within the first week of life, ensures initial stabilization. The second stage occurs around 4 to 6 months, and the third stage is typically completed around age 2. […] The stage 1 Norwood procedure, first described by Norwood et al in 1979, is the foundational surgical intervention for neonates with HLHS. […] The hybrid procedure offers an alternative to the Norwood procedure for neonates with HLHS who are high-risk surgical candidates, such as those with prematurity, low birth weight (<2 kg), or significant comorbidities. [...] The Fontan procedure is the final stage in the surgical palliation of single-ventricle congenital heart defects, such as HLHS. [...] Given the limited availability of donor hearts for newborns, the primary treatment for HLHS is a multistage palliative surgical approach.

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

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

  Most children with hypoplastic left heart syndrome need several surgeries. […] Many babies with hypoplastic left heart syndrome need a heart transplant. Children who have heart transplants need to take medicines for life to so that their bodies don’t reject the donor heart. […] 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.

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

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

  Cardiac transplantation in the newborn period is done as primary treatment for hypoplastic left heart syndrome. While transplantation replaces the abnormal heart of a child with hypoplastic left heart syndrome, this treatment is limited. The low number of newborn organs available for transplantation and the life-long need for anti-rejection therapy can cause concern. Although outcomes for transplantation are improving, and rejection is lowest in patients transplanted as newborns, the average life span of the transplanted heart is less than 15 years.

• #22 Pediatric Hypoplastic Left Heart Syndrome Treatment & Management: Approach Considerations, Medical Care, Surgical Care

  https://emedicine.medscape.com/article/890196-treatment

  No consensus has been reached in the approach to the treatment of neonates with hypoplastic left heart syndrome (HLHS). Supportive care, multistage surgical intervention (ie, Norwood, Glenn, and Fontan procedures) and cardiac transplantation are available options. A thorough explanation of each of these options, including their advantages and disadvantages, should be provided to the parents. […] Occasionally, some anatomic features favor one choice over the others. In the presence of severe tricuspid or pulmonary valve anomalies, the multistage surgical approach is not likely to be beneficial; cardiac transplantation is the only surgical choice. In most cases, the choice of treatment is based on the parents’ preference. While such a decision is being made, the infant should be stabilized.

• #23 Medical Therapies for Heart Failure in Hypoplastic Left Heart Syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9143150/

  Significant surgical and medical advances over the past several decades have resulted in a growing number of infants and children surviving with hypoplastic left heart syndrome (HLHS) and other congenital heart defects associated with a single systemic right ventricle (RV). […] However, cardiac dysfunction and ultimately heart failure (HF) remain the most common cause of death and indication for transplantation in this population. […] Importantly, prototypical adult HF therapies have been relatively ineffective in mitigating the need for cardiac transplantation in HLHS, likely due to several unique attributes of the failing HLHS myocardium. […] Here, we discuss the most commonly used medical therapies for the treatment of HF symptoms in HLHS and other single systemic RV patients. […] Additionally, we provide an overview of potential novel therapies for systemic ventricular failure in the HLHS and related populations based on fundamental science, pre-clinical, clinical, and observational studies in the current literature.

• #24 Pediatric Hypoplastic Left Heart Syndrome Medication: Prostaglandins, Diuretic agents, Cardiac glycosides, Inotropic agents, Afterload-reducing agents, Antiplatelet agents

  https://emedicine.medscape.com/article/890196-medication

  Before the Norwood procedure or cardiac transplantation in patients with hypoplastic left heart syndrome (HLHS), treat infants with prostaglandin E1 infusion, diuretics, inotropes, and afterload reduction. […] Inpatient medications include the following: Prostaglandin E1, Dopamine/dobutamine/milrinone, Furosemide (Lasix/Aldactone), Captopril/enalapril, Digoxin, Potassium chloride. […] Outpatient medications include the following: Furosemide (Lasix/Aldactone), Captopril/enalapril, Digoxin, Potassium chloride. […] Prostaglandin E1 promotes dilatation of the ductus arteriosus in infants with ductal-dependent cardiac abnormalities. […] These agents decrease preload by increasing free-water excretion. Decreasing preload may improve systolic ventricular function. […] These medications improve ventricular systolic function by increasing the calcium supply available for myocyte contraction.

• #25 Medical Therapies for Heart Failure in Hypoplastic Left Heart Syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9143150/

  In general, early recognition and treatment of HLHS-related complications are essential to improving patient outcomes. […] However, there are no Class I, Level A evidence-based treatments for HF associated with any form of CHD, and management of HF in patients with HLHS is particularly challenging due to their unique anatomy and physiology. […] Here, we review the most commonly used medical therapies for myocardial failure in single systemic RV HF as well as potential novel therapies for systemic ventricular failure in HLHS and related CHD populations. […] Current commonly used adult HF therapies have demonstrated mixed or inconclusive effects on HF risk and patient outcomes in the HLHS population. […] Reviewed below are the most commonly applied current therapeutic interventions for the treatment of HLHS HF.

• #26 Medical Therapies for Heart Failure in Hypoplastic Left Heart Syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9143150/

  Angiotensin-converting enzyme inhibitor (ACEi) therapy has been traditionally used in high-risk adult HF patients to delay pathological ventricular remodeling by blocking the Renin-Angiotensin Aldosterone System (RAAS). […] A double-blind, randomized study of the ACEi, Enalapril, in infants with SV who did not have HF failed to demonstrate any benefit in somatic growth, ventricular function, or HF prevention. […] Based on this study, prophylactic use of ACEi in SV is not recommended. […] While a handful of small retrospective case reports and studies have shown beneficial effects for SV HF with -Blocker therapy, a randomized controlled clinical trial in children with systolic HF comparing placebo to the non-selective -Blocker, carvedilol, demonstrated no benefit for SV patients. […] Moreover, it has been shown that -Adrenergic receptor adaptations are uniquely altered in the failing HLHS hearts compared to failing adult hearts, providing a potential explanation for the lack of efficacy of -Blockers in those with a systemic RV.

• #27 Medical Therapies for Heart Failure in Hypoplastic Left Heart Syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9143150/

  Digoxin is a cardiac glycoside, derived from the purple foxglove flower, and was one of the first drugs used for the treatment of HF and arrhythmias. […] A retrospective study of infants with HLHS in the National Pediatric Cardiology Quality Improvement Collaborative (NPCQIC) database demonstrated improved survival for those infants discharged home on digoxin after the Norwood procedure. […] However, there have not been prospective controlled trials of the use of digoxin for the treatment of SV HF. […] While the chronic use of phosphodiesterase-3-inhibitors (PDE3i), such as milrinone, is associated with increased mortality in adults with HF, this class of drugs is commonly used in children. […] Milrinone has been shown to improve HF symptoms in HLHS patients, including reducing frequency of HF-related emergency department visits and hospital admissions. […] PDE3i therapy in pediatric SV HF is commonly used as a bridge to transplant.

• #28 Medical Therapies for Heart Failure in Hypoplastic Left Heart Syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9143150/

  In the HLHS population, maintaining a low pulmonary vascular resistance is important for optimal circulation, particularly after Fontan palliation when blood flow to the lungs is entirely passive. […] Several studies conducted in post-Fontan patients treated with the endothelin rector antagonist, bosentan, observed improved exercise capacity and cardiac performance without significant adverse events including hepatotoxicity. […] Despite these encouraging results, this class of drugs is not commonly used in the treatment of SV HF. […] Congestive symptoms (edema, dyspnea, orthopnea) are a hallmark of HF and are secondary to increased extracellular volume and increased ventricular filling pressures. […] Diuretic therapy aims to mitigate the consequences of this volume expansion and is therefore commonly used for symptom management in patients with HF. […] While the use of diuretics is reasonable in patients with HLHS and congestive HF symptoms, once symptoms improve and euvolemia is achieved attempts at discontinuing or decreasing diuretic use can limit complications such as electrolyte derangements, worsening renal function, and fractures in small children.

• #29 Medical Therapies for Heart Failure in Hypoplastic Left Heart Syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9143150/

  Despite advances in medical and device therapy for the treatment of HF, morbidity and mortality remain high. […] Therefore, there is a continual interest in identification of new therapies for the treatment of adult LV or RV HF, which have the potential to subsequently benefit children with CHD, including those with HLHS.

• #30 Azthena logo with the word Azthena

  https://www.news-medical.net/news/20190724/Clinical-study-demonstrates-potential-of-regenerative-therapy-for-hypoplastic-left-heart-syndrome.aspx

  A phase I clinical trial is the first research monitored by the Food and Drug Administration that demonstrates the potential of regenerative therapy for hypoplastic left heart syndrome (HLHS) through collecting, processing and injecting an infant’s own stem cells directly into the heart at the time of surgery. […] The study focused on the safety and feasibility of stem cell treatments designed to strengthen the heart muscle of children with hypoplastic left heart syndrome, a severe congenital heart disease. […] Each patient underwent the first surgery and then received his or her processed stem cells during the second operation. This study was a first for using a cell-based therapy by direct injection during heart surgery in children. […] All 10 patients successfully underwent the second surgery with injection of stem cells directly into the heart.

• #31 Azthena logo with the word Azthena

  https://www.news-medical.net/news/20190724/Clinical-study-demonstrates-potential-of-regenerative-therapy-for-hypoplastic-left-heart-syndrome.aspx

  „We now have a reproducible protocol to utilize stem cell therapy in babies with HLHS. Our hopes are that this groundbreaking research will lead to stem cell therapy strengthening these babies’ hearts while delaying or even preventing the need for a heart transplant in some,” says Harold Burkhart, M.D., a pediatric cardiothoracic surgeon at OU Medicine in Oklahoma and first author on the paper. […] The HLHS Consortium is now conducting a larger phase IIb study with 50 infants. This study focuses on testing the ability of the stem cells to improve heart function.

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

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

  Despite the aforementioned advancements, patients with HLHS have considerable long-term morbidity following their Fontan operation, and early mortality is not uncommon. […] Another exciting development in the management of HLHS is fetal cardiac intervention, which has emerged as a possible means of altering the natural history of significant cardiac anomalies in utero, with the ultimate goal of improving survival and long-term outcomes.

• #33 Hypoplastic left heart syndrome (HLHS) | UH Rainbow Pediatric Heart Services | University Hospitals | University Hospitals

  https://www.uhhospitals.org/rainbow/services/congenital-heart/conditions-and-treatments/hypoplastic-left-heart-syndrome

  Fetal aortic valvuloplasty is performed in utero using ultrasound guidance and a catheter-based approach to gain access to the fetuss heart. It is normally recommended to take place between 20 and 29 weeks of pregnancy. […] If a baby with HLHS is born with an intact atrial septum, a balloon atrial septostomy may be performed shortly after birth. This cardiac catheterization procedure uses a balloon threaded through a catheter to create or enlarge an atrial septal defect, which allows for oxygen-rich blood to mix with oxygen-poor blood so that the body receives enough oxygen. […] If HLHS is not diagnosed until after birth, a baby will likely need three or more open-heart surgeries in order to repair this complicated heart defect, depending on the severity of the defect. […] The main surgical procedures used to treat HLHS include: Norwood surgery: This surgery takes place within the first week of life. The open heart procedure repairs the aortic arch to provide adequate blood flow to the body.

• #34 New Hope for Treating Heart Failure in Babies

  https://www.upmc.com/media/news/040522-babies-heart-treatment

  Almost one third of babies born with hypoplastic left heart syndrome, or HLHS, die from heart failure before their first birthday. […] Published today in Cell Stem Cell by scientists at the University of Pittsburgh School of Medicine, the study found that two commonly used medications, Viagra and an over-the-counter drug called tauroursodeoxycholic acid (TUDCA), restored processes that drive heart failure in cells derived from patients, opening potential avenues for new HLHS treatments. […] What causes heart failure in HLHS patients who die before one year of age is unknown, and the only treatment option is a heart transplant, which often is not possible. If we can find the cause, then there is hope for therapy. […] Having identified what was wrong at a cellular level, the researchers now had targets for therapies. They found that sildenafil, commonly known as Viagra, and TUDCA rescued the mitochondrial defect in heart cells from patients with severe disease.

• #35 New Hope for Treating Heart Failure in Babies

  https://www.upmc.com/media/news/040522-babies-heart-treatment

  We chose these drugs because we know they are safe and already approved in the clinic for other conditions, added Lo. This means it will take less time to get treatments to patients compared with developing entirely new drugs. […] For HLHS patients, this could mean new therapies that dont rely on heart transplant are on the horizon to treat heart failure.

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

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

  Hypoplastic left heart syndrome (HLHS) is an uncommon, complex congenital (present at birth) defect in which several structures in the hearts left side form incorrectly. […] Treatment for HLHS requires our highly specialized congenital heart surgeons to rework the baby’s circulatory system through a series of three open heart surgeries (Norwood, Glenn, and Fontan procedures) staged over a span of about three years. An alternative approach is heart transplantation, also an area of expertise at Children’s Wisconsin. […] For children undergoing Hypoplastic Left Heart Syndrome surgical treatment, the time between the first and second procedures, commonly known as the interstage period, is a critical stage in their care. […] The Herma Heart Institute at Children’s Wisconsin in Milwaukee is recognized for pioneering the first interstage home monitoring initiative of its kind for partnering with parents in caring for infants with HLHS and other single ventricle defects.

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

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

  After surgery, your baby will go to the Cardiothoracic Intensive Care Unit (CTICU). They may be on a breathing machine and have many tubes, wires, and equipment to keep them stable and make sure they are okay. Members of their health care team may make changes to your babys medicines and breathing machine often. This will happen the most in the first 24 to 48 hours after surgery. […] Other treatments your baby will probably get after surgery are: Antibiotic medicine to prevent infection, Fluids and nutrition through an IV or PICC line, Heart medicines by IV or by mouth, Chest drainage tube, Oxygen, Pain medicines, A tube in the nose to the stomach (nasogastric [NG] tube) to keep the stomach empty, Blood transfusions, when needed. […] 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. Here, your baby will recover, learn to eat, and start to breathe without oxygen. Your baby will go home once their oxygen levels are good and they are gaining weight. This can take at least 3 to 4 weeks after surgery or longer. The total time your baby will stay in the hospital depends on if they were born early or if there are other problems.

• #38 Hypoplastic Left Heart Syndrome (HLHS) in Children | Phoenix Children’s Hospital

  https://phoenixchildrens.org/specialties-conditions/hypoplastic-left-heart-syndrome-hlhs-children

  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.

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

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

  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 Norwood procedure is done in the first or second week of a baby’s life to allow the right ventricle do the work of the left ventricle — pumping blood to the body — and its normal job of pumping deoxygenated blood returning to the heart back to the lungs. […] The Glenn procedure typically is performed when the infant is 4 to 6 months old and is a step toward allowing the right ventricle to perform just the work of the left ventricle, pumping blood to the body. […] The Fontan procedure typically is performed when the child is 2 to 4 years old to reroute blood from the lower part of the body directly to the lungs and allow the right ventricle to replace the left 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.

• #40 Hypoplastic Left Heart Syndrome (HLHS): Symptoms & Causes | NewYork-Presbyterian

  https://www.nyp.org/pediatrics/heart/hypoplastic-left-heart-syndrome-hlhs

  Babies with hypoplastic left heart syndrome will die without surgery. If left untreated, a child would not be expected to survive longer than a few days or weeks after birth. […] The risk of HLHS complications or death are highest during the first stage of surgery. The second and third stages of surgery have an increased survival rate. […] Children who experience staged surgeries will continue to need special care and treatments throughout their life to ensure proper growth and development. […] As with other congenital heart disorders, children must continue regular follow-up visits with a pediatric cardiologist or heart specialist throughout their lifetime. […] The Children’s Hospital of New York retains world renowned pediatric heart surgeons whose expertise in treating children with congenital and acquired cardiovascular conditions is unsurpassed. […] Contact the Children’s Hospital of New York to schedule an appointment with a leading pediatric cardiologist or pediatric cardiovascular surgeon. Knowing the treatment options available to your child can alleviate some of your feelings of doubt and confusion.

• #41 Hypoplastic Left Heart Syndrome (HLHS) in Children | UMass Memorial Health

  https://www.ummhealth.org/health-library/hypoplastic-left-heart-syndrome-hlhs-in-children

  A baby with hypoplastic left heart syndrome needs surgery to survive. […] The first stage of the surgery has the highest risk for complications and death. Some special treatment centers that do many of these procedures have higher survival rates than centers where fewer procedures are done. Survival rates are higher with the second and third stages of surgery. […] 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. […] 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. Children with HLHS after surgery often have lower exercise tolerance.

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

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

  Depending on the success of surgery, individuals may lead functional lives for the rest of their days. Over time, the function of the right ventricle may worsen, and heart transplantation may be needed. […] 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.

• #43 Management of the Hypoplastic Left Heart Syndrome: From Fetus to Adult | Frontiers Research Topic

  https://www.frontiersin.org/research-topics/29743/management-of-the-hypoplastic-left-heart-syndrome-from-fetus-to-adult/magazine

  The hypoplastic left heart syndrome (HLHS) is arguably one of the most complex forms of congenital heart disease. It is universally fatal without intervention in the neonatal period and perhaps no other congenital cardiovascular condition poses as formidable a challenge when it comes to management. There is no cure and the most common strategy for survival involves a series of palliative surgical interventions resulting in a single right ventricle providing systemic output through a reconstructed aortic outflow, while a total cavopulmonary connection provides passive pulmonary perfusion. Since the first successful Norwood procedure in 1981, significant strides have been made in improving diagnosis and preoperative care, refining surgical techniques, and advancing postoperative management, with outcome data now available for nearly four decades. These show that neonatal survival has improved significantly, reaching close to 90% in more experienced centers, and that survival after the Fontan procedure, including reasonable quality of life, is possible for up to 20 years in as many as 80% of patients.

• #44 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

  Without surgery, babies with hypoplastic left heart syndrome will not live longer than a few days or weeks. […] The first stage of the surgery has the highest risk for complications and death. Some special treatment centers that do many of these procedures have higher survival rates than centers where fewer procedures are done. Survival rates are higher with the second and third stages of surgery. […] Some children will need a heart transplant to survive into adulthood. […] Your child will need regular follow-up care at a center specializing in this type of congenital heart care.

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