Materiały źródłowe

• #1 Hypoplastic Left Heart Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK554576/

  Hypoplastic left heart syndrome (HLHS) is a rare but severe congenital heart defect characterized by underdevelopment of left-sided heart structures, including the left ventricle, aortic valve, mitral valve, and ascending aorta. […] Diagnosis often occurs prenatally through fetal echocardiography, with postnatal clinical findings including cyanosis, weak pulses, and signs of low cardiac output as the ductus closes. […] Despite advancements in care, HLHS patients face significant risks of morbidity and mortality, including heart failure, arrhythmias, and thromboembolic events, with long-term survival dependent on specialized medical and surgical interventions. […] Infants born with HLHS depend on a patent ductus arteriosus and interatrial communication for survival until they can undergo surgical intervention.

• #2 Hypoplastic left heart syndrome – Wikipedia

  https://en.wikipedia.org/wiki/Hypoplastic_left_heart_syndrome

  Hypoplastic left heart syndrome (HLHS) is a rare congenital heart defect in which the left side of the heart is severely underdeveloped and incapable of supporting the systemic circulation. Early signs and symptoms include poor feeding, cyanosis, and diminished pulse in the extremities. […] Closing of the ductus arteriosus in a heart that is severely underdeveloped on the left results in cyanosis and respiratory distress which can progress to cardiogenic shock and death. Early symptoms might include poor feeding or cyanosis that does not respond to oxygen administration. Peripheral pulses may be weak and extremities cool to the touch. […] If left untreated, patients with HLHS die within the first weeks of life while 70% of those that undergo three-staged palliative surgery reach adulthood. After surgery, children with HLHS typically experience neurodevelopmental as well as motor delay and are at an increased risk of heart failure as adults.

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

  https://healthlibrary.overlakehospital.org/library/testsprocedures/neurological/90,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. […] The following structures are usually affected: Mitral valve. This valve controls blood flow between the left atrium and left ventricle. Left ventricle. This is the lower left chamber of the heart. It pumps blood to the body. Aortic valve. This valve controls blood flow from the left ventricle into the aorta, and then to the body. The aorta is the major blood vessel that delivers blood to the body. Aorta. This is the large artery leading from the left ventricle to the body. […] The left ventricle is normally very strong, so it can pump blood to the body. When it is small and poorly developed, it can’t pump any or enough blood out to the body. For this reason, a baby with HLHS will not survive without surgery to fix the defect.

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

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

  Infants and babies with HLHS usually develop symptoms shortly after birth. The following are the most common symptoms of hypoplastic left heart syndrome. However, each child may experience symptoms differently. HLHS symptoms may include: […] Cyanosis (blue color of the skin, lips, and nailbeds) […] Pale skin […] Sweaty or clammy skin […] Cool skin […] Heavy and/or rapid breathing […] Fast heart rate. […] The symptoms of hypoplastic left heart syndrome may resemble other medical conditions and heart problems. Always consult your child’s physician for a diagnosis. […] Perhaps the most critical defect in HLHS is the small, underdeveloped left ventricle. This chamber is normally very strong and muscular so it can pump blood to the body. When the chamber is small and poorly developed, it will not function effectively and cannot provide enough blood flow to meet the body’s needs. For this reason, an infant with hypoplastic left heart syndrome will not live long without surgical intervention.

• #5 Hypoplastic Left Heart Syndrome – MD Searchlight

  https://mdsearchlight.com/heart-health/hypoplastic-left-heart-syndrome/

  Hypoplastic left heart syndrome (HLHS) is a birth defect of the heart where the left side is underdeveloped. This includes parts such as the mitral valve, left ventricle, and the aortic valve, which are crucial for the heart to properly function. […] HLHS, short for Hypoplastic Left Heart Syndrome, is typically identified in babies before they are born through the use of an ultrasound. Most of the time, babies who have this syndrome are born full-term with a normal weight. They often seem healthy in the initial hours of their lives as their ductus arteriosus, a blood vessel that’s part of typical fetal circulation, remains open during the first day or so following birth. […] If a baby fails the pulse oximetry screen, or if they start showing symptoms like poor blood circulation, unusually fast heart rate, disturbed acid balance in the body, low blood pressure, and weak pulses, they should be referred to a cardiologist immediately. These symptoms often start appearing when the ductus arteriosus begins to close, affecting the blood flow in the body.

• #6 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. […] In a child with hypoplastic left heart syndrome, the structures on the left side of the heart are very underdeveloped. […] Newborns with hypoplastic left heart syndrome will have lower-than-normal oxygen saturations. This is because all the blood from the lungs mixes in the single right ventricle before being pumped out of the lungs and body. […] Cyanosis, or blue coloration of the skin, may be the first clue to a serious cardiac condition. […] Sleepiness, poor feeding, and worsening respiratory distress may be seen as the ductus arteriosus closes. […] Without treatment, hypoplastic left heart syndrome is fatal. This happens within the first hours or days of life.

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

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

  Few congenital heart malformations have raised as many surgical, ethical, social and economic issues as the therapy for infants diagnosed with hypoplastic left heart syndrome. […] Before the 1980s, this complex malformation was associated with 95% mortality within the first month of life. […] Untreated, the prognosis for the infant is certain death. […] The clinical presentations of HLHS occur most frequently between days 1 and 3 of life, and include respiratory distress with tachypnea and mild cyanosis, shock, and less commonly, severe cyanosis. […] Most infants die within the first two weeks of life, with an average age at death of 4.5 days. […] Some patients with HLHS, however, can survive beyond sixty days, without any surgical intervention through the development of pulmonary hypertension.

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

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

  In most cases, children with hypoplastic left heart syndrome appear healthy at birth. […] When a baby is not diagnosed during pregnancy, symptoms may develop within the first few hours or days of life: Rapid breathing or shortness of breath, Rapid heartbeat or pounding heart, Poor suckling and feeding, Cold extremities (poor perfusion), Blue color of the skin, lips, and nailbeds (cyanosis), Weakness. […] If your baby was not diagnosed during pregnancy, it will become apparent within the first few hours to days that your baby has a heart defect.

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

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

  Babies born with hypoplastic left heart syndrome may seem normal at birth but become severely ill soon after birth. […] Symptoms include: Grayish-blue skin color, Rapid, difficult breathing, Poor feeding, Cold hands and feet, Lethargy. […] Without treatment, babies afflicted with hypoplastic left heart syndrome can die within the first days or weeks of life. […] This heart defect is usually fatal within the first days or month of life unless treated.

• #10 Hypoplastic Left Heart Syndrome – Pediatrics – Merck Manual Professional Edition

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

  Symptoms of hypoplastic left heart syndrome appear when the ductus arteriosus begins to close during the first 24 to 48 hours of life. Subsequently, the clinical picture of cardiogenic shock (eg, tachypnea, dyspnea, weak pulse, pallor, cyanosis, hypothermia, metabolic acidosis, lethargy, oliguria or anuria) rapidly develops. […] The patient often presents with a history of poor feeding, increased work of breathing, pale or gray coloration, and lethargy. Physical examination shows a very active precordium with a marked parasternal lift associated with very poor peripheral perfusion, cool extremities, bluish gray skin color, and absent or barely palpable pulses. […] Severe metabolic acidosis, often worsening if supplemental oxygen is administered, is characteristic of hypoplastic left heart syndrome. […] Symptoms of cardiogenic shock (eg, tachypnea, dyspnea, weak pulse, pallor, cyanosis, hypothermia, metabolic acidosis, lethargy, oliguria or anuria) appear when the ductus arteriosus begins to close during the first 24 to 48 hours of life, becoming more pronounced if supplemental oxygen is given.

• #11 Hypoplastic Left Heart Syndrome Diagnosis & Treatment | Mount Sinai – New York

  https://www.mountsinai.org/locations/childrens-heart/conditions/hlhs

  Hypoplastic left heart syndrome (HLHS) is a serious congenital heart defect (present at birth) that affects the left side of the heart. HLHS occurs when the left side of the heart does not form fully. As a result, the left side of the heart, which pumps blood, is unable to do its job. HLHS interrupts the normal flow of oxygenated blood to your baby’s body. […] When your baby is born with HLHS, there may be no symptoms at first, since the blood is able to get to the body through a blood vessel that all babies are born with, the patent ductus arteriosus (PDA). Your baby’s only symptom may be a low oxygen level, which is difficult to detect by how your baby looks. […] However, once the PDA closes, which it usually does within the first hours or days of life, your baby’s body will not receive enough blood. Symptoms of HLHS that usually develop may include: Problems breathing, Weak pulse, Ashen or bluish skin color.

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

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

  Hypoplastic left heart syndrome occurs when parts of the left side of the heart (mitral valve, left ventricle, aortic valve, and aorta) do not develop completely. The condition is present at birth (congenital). […] At first, a newborn with hypoplastic left heart may appear normal. Symptoms may occur in the first few hours of life, although it may take up to a few days to develop symptoms. These symptoms may include: […] Bluish color to the skin (cyanosis) due to low oxygen level in the blood […] Cold hands and feet (extremities) […] Lethargy […] Poor pulse […] Poor suckling and feeding […] Pounding heart […] Rapid breathing […] Shortness of breath. […] A bluish color in the chest or abdomen, lips, and tongue is abnormal (called central cyanosis). It is a sign that there is not enough oxygen in the blood. Central cyanosis often increases with crying.

• #13 Pediatric Hypoplastic Left Heart Syndrome: Background, Pathophysiology, Etiology

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

  Hypoplastic left heart syndrome is a uniformly lethal cardiac abnormality if not surgically addressed. […] Hypoplastic left heart syndrome typically presents within the first 24-48 hours of life. Presentation occurs as soon as the ductus arteriosus constricts, thereby decreasing systemic blood flow, producing shock, and, without intervention, causing death. […] The most common presentation is visceroatrial situs solitus with D-ventricular loop and atrioventricular and ventriculoarterial concordance, as well as levocardia. Rarely, dextrocardia and heterotaxy may be present. […] Following the Norwood procedure (stage I), overall success (survival to hospital discharge) is approximately 75%. […] Hypoplastic left heart syndrome has the greatest mortality rate among all coronary heart conditions. […] Without surgery, hypoplastic left heart syndrome is uniformly fatal, usually within the first 2 weeks of life.

• #14 Hypoplastic left heart syndrome — Knowledge Hub

  https://www.genomicseducation.hee.nhs.uk/genotes/knowledge-hub/hypoplastic-left-heart-syndrome/

  Hypoplastic left heart syndrome occurs when the left side of the heart is underdeveloped: the mitral and/or aortic valve is narrow or blocked, the left ventricle is underdeveloped, the aorta is small and underdeveloped and there is an atrial septal anomaly. […] The clinical features of hypoplastic left heart syndrome are: dyspnoea; a high-pitched noise during inhalation; cyanosis; congestive heart failure early in life; poor feeding; frequent vomiting; lethargy; and life-threatening complications such as shock and hepatomegaly, which can occur if the condition is left untreated. […] The progression and severity of hypoplastic left heart syndrome depends on whether the ductus arteriosus remains patent or not. The ductus arteriosus allows blood to pass from the right ventricle to the aorta, bypassing the left side of the heart. It normally closes soon after birth, but initial treatment in babies with hypoplastic left heart syndrome involves keeping it open until corrective surgical measures can be completed.

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

  https://healthlibrary.sanjuanregional.com/BreatheEasy/90,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. […] Babies with hypoplastic left heart syndrome usually get symptoms shortly after birth: […] Blue color of the skin, lips, and nails (cyanosis) […] Pale skin […] Sweaty, clammy, or cool skin […] Trouble breathing […] Fast heart rate […] Cold feet […] Poor pulses in the feet […] Poor feeding. […] The symptoms of HLHS may look like other health conditions and heart problems. Your child needs to see a healthcare provider for a diagnosis. […] Hypoplastic left heart syndrome is a group of problems that affect the heart and large blood vessels. […] Babies usually get symptoms shortly after birth. […] Babies with HLHS need surgery to survive. […] Most babies will need a series of surgeries during their first 2 to 3 years of life. […] After the surgeries, children will need special care and to be monitored for complications.

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

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

  The left ventricle is normally very strong, so it can pump blood to the body. When it is small and poorly developed, it can’t pump any or enough blood out to the body. For this reason, a baby with HLHS will not survive without surgery to fix the defect. […] Babies with hypoplastic left heart syndrome usually get symptoms shortly after birth: Blue color of the skin, lips, and nails (cyanosis), Pale skin, Sweaty, clammy, or cool skin, Trouble breathing, Fast heart rate, Cold feet, Poor pulses in the feet, Poor feeding. […] The symptoms of HLHS may look like other health conditions and heart problems. Your child needs to see a healthcare provider for a diagnosis. […] 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. […] 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.

• #17 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. […] A baby born with hypoplastic left heart syndrome may have: fast breathing, blue or grayish coloring of the skin and nails, trouble feeding, low energy and activity, fewer than normal wet diapers. […] In hypoplastic left heart syndrome, the left ventricle is too small. The aorta, which takes the blood to the body, is small too. The heart cant pump enough blood to the body.

• #18 Hypoplastic Left Heart Syndrome – Seattle Children’s

  https://www.seattlechildrens.org/conditions/hypoplastic-left-heart/

  Most babies with HLHS develop symptoms within the first days after birth if they do not get the medical care they need. […] Common symptoms include: Blue or purple-tinged skin, lips or fingernails (cyanosis) or skin that looks mottled, grayish or paler than your baby’s usual skin color. […] Being more tired than is normal. […] Trouble feeding. […] Fast breathing or working hard to breathe. […] Cold arms and legs. […] Weak pulse. […] Because the left ventricle cannot pump enough blood to the body, the right ventricle must do all the work. […] In a newborn, this can work for a short time. […] The other risk for babies with this syndrome is that their right ventricle must do so much work. Over time, this can cause heart failure.

• #19 HLHS in Infants | Children’s Hospital Colorado

  https://www.childrenscolorado.org/conditions-and-advice/conditions-and-symptoms/conditions/hypoplastic-left-heart-syndrome/

  Children with hypoplastic left heart syndrome (HLHS) develop a blue or gray tint to their skin. This condition is called cyanosis. The blue color can become more intense when an infant cries. […] Other hypoplastic left heart syndrome symptoms to look for include: Low or faint pulse, Trouble nursing and feeding, Fast breath or shortness of breath, Extreme tiredness, Loud heartbeat. […] Hypoplastic left heart syndrome is a congenital heart defect, meaning kids are born with the disease. With this condition, the only way for blood to get to the body is if it can pass back through the heart from the left atrium to the right atrium and ventricle.

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

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

  Hypoplastic left heart syndrome is a type of single ventricle defect that is present at birth. In a child with hypoplastic left heart syndrome, the left ventricle, a pumping chamber, is too small to pump blood with oxygen to the body. Only the right ventricle (pumping chamber) is able to pump blood to the body. In addition, the first part of the main blood vessel between the heart and the body (aorta) is very small. As a result, your child’s organs and body don’t get the blood and oxygen they need to function properly. […] An infant with hypoplastic left heart syndrome may appear healthy at first. Symptoms typically appear within the first hours or days of life as oxygen levels and blood circulation drop. […] Pale or blue skin (cyanosis) from low oxygen levels. Heavy, rapid breathing. Fast heart rate. Cold feet. Weak pulse throughout the body.

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

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

  HLHS might not cause trouble for the first few days of life while the PDA and the foramen ovale remain open. However, they may quickly develop signs and symptoms after these openings close, including: […] Problems breathing […] Fast heart rate […] Weak pulse […] Ashen or bluish skin color. […] Surgical repairs for HLHS are not a cure. Infants with HLHS may have lifelong complications. They will need routine checkups with a heart doctor to monitor their progress.

• #22 Hypoplastic Left Heart Syndrome – Causes, Symptoms, Diagnosis & Treatment

  https://www.medindia.net/health/conditions/hypoplastic-left-heart-syndrome.htm

  The symptoms of HLHS manifest within a few hours or a few days (1 to 3 days) after birth; they typically become prominent once the ductus arteriosus closes. The child may suffer from cardiovascular shock (extremely low blood pressure) and collapse. […] The symptoms include: skin feels cold and clammy, bluish discoloration of the skin called cyanosis, abnormality in breathing, rapid pulse that is weak, poor feeding, as a result of which tube feeding may be required in the initial days. […] The changes in the systemic circulation could result in metabolic acidosis and death. The average age of death for infants dying in the first 2 weeks is 4.5 days. Certain infants can survive for more than 60 days without any surgery, but mortality is 100% in the absence of surgery.

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

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

  Hypoplastic left heart syndrome (HLHS) is a severe congenital heart defect in which the left side of the heart is underdeveloped. […] The following symptoms of hypoplastic left heart syndrome may be present at birth or several days later: Blue or purple tint to lips, skin and nails (cyanosis), Difficulty breathing, Difficulty feeding, Lethargy (sleepy or unresponsive). […] It’s a very severe form of heart disease, and if not treated medically and then surgically early after birth, almost every infant will die within the first few weeks of life. […] Most babies with hypoplastic left heart syndrome born before the 1980s had no chance of survival. […] Many children are now in their early teens with this kind of heart condition, and they’re doing quite well. […] 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. […] Only time will tell what the long-term outcome really is.

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

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

  Signs of shock for babies include: […] Cold, clammy, pale skin […] Bluish-gray lips […] Rapid or weak pulse […] Difficulty breathing, or slow or shallow breaths […] Dull stare in the eyes. […] 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. […] Children who experience staged surgeries will continue to need special care and treatments throughout their life to ensure proper growth and development. […] There is an increased risk for heart failure and heart rhythm issues. […] This condition causes a child to become easily fatigued, therefore limiting their participation in exercise.

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

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

  Hypoplastic left heart syndrome is a critical illness for an infant and soon after their birth they will exhibit signs of the condition. […] Symptoms for HLHS include: […] Cyanosis. This condition is caused by lack of oxygenation. The lips and gums of a person will become grayish-blue in color. […] Breathing. The baby will have rapid or difficulty breathing […] Feeding. The infant will have poor feeding […] Cold hands and feet. Because of the lack of blood circulation and oxygen reaching the extremities, the baby might have cold hands and feet […] Weak pulse. Infants may show signs of heart failure or heart disease […] Extreme sleepiness. Due to lack of oxygen, infants with HLHS may experience extreme sleepiness. […] If the openings between the left and right sides of the heart do close for babies with hypoplastic left heart syndrome, they could go into shock or die.

• #25 Azthena logo with the word Azthena

  https://www.news-medical.net/health/Causes-and-Symptoms-of-Hypoplastic-Left-Heart-Syndrome.aspx

  Hypoplastic left heart syndrome (HLHS) is one of the most serious congenital heart anomalies, with an incidence of about 0.016-0.036% in North America, and has a mortality of 100% if untreated. […] The symptoms with which they present occur within the first three days of life, typically after the first day. They include: Breathlessness, Rapid shallow breathing, Pounding heartbeat, Weak pulse, Mild cyanosis or a bluish-gray discoloration of the lips and around the mouth, Signs of shock such as sweating and a cold, clammy skin. […] The normal changes in blood flow that happen in the first few days of life cause infants with HLHS to become acutely ill as they are deprived of the compensatory shunt between the right and the left side of the heart. […] Thus the perfusion of the heart and of the coronary vessels is reduced, leading to hypoxia, metabolic acidosis, and shock. Death usually occurs within 2 weeks in affected infants.

• #26 Hypoplastic Left Heart Syndrome (HLHS) – Patient Worthy

  https://patientworthy.com/hypoplastic-left-heart-syndrome-hlhs/

  Hypoplastic left heart syndrome (HLHS) is a birth defect that affects normal blood flow through the heart. As the baby develops during pregnancy, the left side of the heart does not form correctly. Hypoplastic left heart syndrome is one type of congenital heart defect. Congenital means present at birth. Because a baby with this defect needs surgery or other procedures soon after birth, HLHS is considered a critical congenital heart defect (CCHD). […] Babies born with hypoplastic left heart syndrome usually are seriously ill soon after birth. Hypoplastic left heart syndrome symptoms include: Grayish-blue skin color (cyanosis), Rapid, difficult breathing, Poor feeding, Cold hands and feet, Being unusually drowsy or inactive. […] In a baby with hypoplastic left heart syndrome, if the natural connections between the heart’s left and right sides are allowed to close, he or she may go into shock and may die.

• #27 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 with hypoplastic left heart syndrome usually get symptoms shortly after birth: […] The symptoms of HLHS may look like other health conditions and heart problems. Have your child see his or her healthcare provider for a diagnosis. […] Without surgery, babies with hypoplastic left heart syndrome will not live longer than a few days or weeks. […] Babies usually get symptoms shortly after birth. […] Babies with HLHS will not survive without surgery. […] Most babies will need a series of at least 3 surgeries during their first 2 to 3 years of life. […] After the surgeries, children will need special care and be monitored for complications.

• #28 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. […] In a child with hypoplastic left heart syndrome, the structures on the left side of the heart are very underdeveloped. […] Newborns with hypoplastic left heart syndrome will have lower-than-normal oxygen saturations. This is because all the blood from the lungs mixes in the single right ventricle before being pumped out of the lungs and body. […] Cyanosis, or blue coloration of the skin, may be the first clue to a serious cardiac condition. […] Sleepiness, poor feeding, and worsening respiratory distress may be seen as the ductus arteriosus closes. […] Without treatment, hypoplastic left heart syndrome is fatal. This happens within the first hours or days of life.

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

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

  Babies born with hypoplastic left heart syndrome (HLHS) usually are very sick soon after birth. Symptoms of HLHS include: […] Without treatment, a baby with this condition may go into shock. Symptoms of shock include: […] Get emergency medical help if your baby has: […] 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.

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

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

  Hypoplastic left heart syndrome symptoms may include: […] Newborns with HLHS dont always show symptoms immediately. Instead, symptoms may develop within a few hours or days of birth. […] Without treatment, hypoplastic left heart syndrome is fatal days or weeks after birth. […] With treatment, the prognosis depends on the complexity of your childs heart deformity. […] About 20% to 60% of babies with hypoplastic left heart syndrome survive their first year of life. After that, the survival rate for the next five, 10 and 15 years is about 40%.

• #31 Hypoplastic Left Heart Syndrome – MD Searchlight

  https://mdsearchlight.com/heart-health/hypoplastic-left-heart-syndrome/

  Only two-thirds of children with Hypoplastic Left Heart Syndrome (HLHS) survive past the age of 5. Furthermore, about 1% of patients who undergo the Fontan procedure (a surgery used in treating this condition) pass away each year. […] The mortality rate for the initial palliation stage is around 15%, with subsequent stages Hemi-Fontan/Glenn and Fontan presenting significantly lower mortality rates at 1.8% and 1.0% respectively.

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

  https://healthlibrary.metroplus.org/ReturningtoCare/90,P01798

  Babies with hypoplastic left heart syndrome usually get symptoms shortly after birth: […] The symptoms of HLHS may look like other health conditions and heart problems. Your child needs to see a healthcare provider for a diagnosis. […] 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. […] Call your child’s healthcare provider if your child’s symptoms get worse or if new symptoms develop.

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

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

  In hypoplastic left heart syndrome, the left side of the heart including the aorta, aortic valve, left ventricle and mitral valve is underdeveloped. […] The baby often seems normal at birth but will need medical attention within a few days as the ductus closes. […] This heart defect is usually fatal within the first days or months of life without treatment. […] Babies with this syndrome have: Poor color and mottled skin, Rapid and difficult breathing, Difficulty feeding. […] Left untreated, children with hypoplastic left heart syndrome usually die in early infancy. […] The long-term outlook is only beginning to be known, as this heart defect used to be fatal. […] Over time, the function of the right ventricle may worsen, and heart transplantation may be needed. […] Problems encountered over time, or known morbidities include: Congestive heart failure, Arrhythmias, Protein losing enteropathy (PLE), Plastic bronchitis, Fontan-related liver disease, Chronic kidney disease, Endocrine abnormalities (bone disease, growth failure), Thromboembolic events, Neurodevelopmental concerns.

• #34 Hypoplastic left heart syndrome – Wikipedia

  https://en.wikipedia.org/wiki/Hypoplastic_left_heart_syndrome

  Prognosis is dependent upon the health of the child, as there is an increased demand on respiratory and heart rate in infants during common childhood illnesses. This fragile population has little cardiac reserve to accommodate these demands and provide hemodynamic stability during illnesses. […] Children with HLHS and other comparable single-ventricle conditions, as a group, have poorer neurodevelopmental outcomes than their healthy peers. Deficits in language, executive functioning, and higher rates of anxiety and depression disorders have been demonstrated.

• #35 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. […] 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. […] Most children with hypoplastic left heart syndrome need several surgeries. […] 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.

• #36 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.

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

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

  The perioperative survival rates reported for the Norwood operation have ranged from 47% to 85%. […] Survival has been shown to be worse in patients with significant noncardiac congenital anomalies, low birth weight and severe obstruction to pulmonary venous return. […] Concerns about the long term outcome of infants with HLHS include the adequate preservation of the right ventricle and valvular function when exposed to systemic pressure. […] 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.

• #38 Hypoplastic Left Heart Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK554576/

  A continuous infusion of prostaglandin E1 is necessary to maintain ductal patency. […] Before the 1980s, infants with this condition received comfort care and typically did not survive long after birth. […] However, due to significant medical and surgical advancements, current treatment options include a staged surgical approach that may start with the Norwood or Sano procedure during the neonatal period. […] If the infant survives the first stage of surgical or hybrid treatment (ie, the Norwood/hybrid), 2 palliative surgical operations typically follow: the hemi-Fontan or bidirectional Glenn shunt, followed by the Fontan procedures. […] Neonates with HLHS are entirely dependent on a patent ductus arteriosus (PDA) to sustain systemic and coronary perfusion. […] Closure of the PDA results in severe systemic hypoperfusion and rapid cardiovascular collapse, necessitating continuous infusion of prostaglandin E1 to maintain ductal patency. […] Early recognition, stabilization, and a staged surgical approach are critical to improving survival and outcomes in neonates with HLHS.

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