Hypoplastic Left Heart Syndrome (HLHS)enparentshttps://kidshealth.org/EN/images/headers/Cardiology_Hypoplastic_Left_Heart_Syndrome_enHD_2.jpgHypoplastic left heart syndrome (HLHS) is a birth defect of a baby’s heart. The left side of the heart doesn’t grow as it should, making it smaller and weaker than normal. hypoplastic left heart syndrome, HLHS, heart defect, atrial septal defect, ASD, patent ductus arteriosus, PDA, left side of the heart, heart conditions, cardiology, cardiologist, heart transplants, hypoplastic, left heart, ductus arteriosus, foramen ovale, ventricles, valves, heart surgery, mitral valve , aortic valve, prostaglandin, norwood, Norwood procedure, Glenn, Glenn shunt operation, Fontan, Fontan operation, hypoplastic left heart syndrome surgery, single ventricle, single ventricle defect, hybrid procedure10/10/201711/22/201911/22/2019Michael A. Bingler, MD11/05/20183f36bb72-0bef-42bc-b6de-c0fddf263c16https://kidshealth.org/ws/RadyChildrens/en/parents/hypoplastic-heart.html/<h3>What Is Hypoplastic Left Heart Syndrome (HLHS)?</h3> <p>Hypoplastic left heart syndrome (HLHS) is a rare birth defect of a baby's heart. The left side of the heart doesn't grow as it should, making it smaller and weaker than normal.</p> <p>The left side of the heart is supposed to pump blood out to the body. But a baby's heart with HLHS can't do the job. This makes a baby very sick. Without medicines and a series of three surgeries to rebuild the heart, babies with HLHS won't survive.</p> <p>The left side of the heart can't be fixed, so the goal of the surgeries is to rebuild parts of the heart and "redirect" the way blood flows to and from the body.</p> <p>Soon after birth, a baby with HLHS needs around-the-clock care in a hospital for the first weeks or months of life.</p> <h3>How Does the Heart Work?</h3> <p>The heart is a muscle that pumps blood. It acts like two pumps in one. The pumps are called ventricles and each has an important job:</p> <ul> <li>The <a class="kh_anchor">right ventricle</a> gets blood from the body and pumps it out the <a class="kh_anchor">pulmonary artery</a> to the lungs. <br /><br />In the lungs, blood fills up with oxygen and gets rid of carbon dioxide.</li> <li>The <a href="https://kidshealth.org/ws/RadyChildrens/en/parents/dictionary-left-ventricle.html/">left ventricle</a> gets the blood from the lungs and pumps it out through the <a href="https://kidshealth.org/ws/RadyChildrens/en/parents/dictionary-aorta.html/">aorta</a> to the body. <br /><br />Because it pumps blood to the entire body, the left ventricle is a stronger pump than the right ventricle.</li> </ul> <p><img class="right" title="" src="https://kidshealth.org/EN/images/illustrations/HLHSestab_415X415_enIL.jpg" alt="Illustration: How the heart pumps blood" /></p> <h3>What Happens in Hypoplastic Left Heart Syndrome?</h3> <p>With hypoplastic left heart syndrome:</p> <ul class="kh_longline_list"> <li>The left ventricle and aorta are too small to pump enough blood to the body.</li> <li>The right ventricle, which is only supposed to pump blood to the lungs, pumps blood to the lungs <em>and</em> the body through an extra pathway called a <a href="https://kidshealth.org/ws/RadyChildrens/en/parents/patent-ductus-arteriosus.html/">patent ductus arteriosis (PDA)</a>. That means the right ventricle gets overworked.<img class="right" title="" src="https://kidshealth.org/EN/images/illustrations/HLHSCloseASDandPDA_415X415_enIL.jpg" alt="Illustration: Babies with HLHS rely on two important pathways, an ASD and a PDA." /></li> <li>Babies with HLHS are almost always born with an <a href="https://kidshealth.org/ws/RadyChildrens/en/parents/asd.html/">atrial septal defect (ASD)</a>. This is a hole in the upper part of the heart. It lets blood with oxygen mix with blood low on oxygen. Then the right ventricle pumps the blood to the lungs and the body.</li> </ul> <p><img class="right" title="" src="https://kidshealth.org/EN/images/illustrations/HLHSbloodflow_415X415_enIL.jpg" alt="Illustration: How a heart with HLHS pumps blood" /></p> <h3>What Are the Signs &amp; Symptoms of Hypoplastic Left Heart Syndrome?</h3> <p>Within a few hours to a day or so after birth, a newborn with undiagnosed HLHS may have:</p> <ul> <li>trouble breathing</li> <li>blue or grayish coloring of the skin and nails</li> <li>difficulty feeding</li> <li>lethargy (very little activity)</li> <li>weak pulses in the arms and legs</li> <li>few wet diapers</li> </ul> <h3>What Causes Hypoplastic Left Heart Syndrome?</h3> <p>HLHS is a birth defect that happens when a baby is growing in the womb. No one knows exactly what causes it, but it could have a mix of causes, including a baby's&nbsp;<a href="https://kidshealth.org/ws/RadyChildrens/en/parents/about-genetics.html/">genes (DNA)</a>.</p> <h3>How Is Hypoplastic Left Heart Syndrome Diagnosed?</h3> <p>Hypoplastic left heart syndrome can be diagnosed with:</p> <ul> <li>a prenatal <a href="https://kidshealth.org/ws/RadyChildrens/en/parents/prenatal-ultrasound.html/">ultrasound</a> scan</li> <li><a href="https://kidshealth.org/ws/RadyChildrens/en/parents/xray-exam-chest.html/">chest X-ray</a></li> <li><a href="https://kidshealth.org/ws/RadyChildrens/en/parents/ekg.html/">electrocardiogram</a></li> <li><a href="https://kidshealth.org/ws/RadyChildrens/en/parents/echo.html/">echocardiogram</a></li> <li><a href="https://kidshealth.org/ws/RadyChildrens/en/parents/pulse-oximetry.html/">pulse oximetry</a></li> </ul> <h3>How Is Hypoplastic Left Heart Syndrome Treated?</h3> <p>HLHS is first treated with medicines, and then a series of <strong>three surgeries</strong>. Without surgery, a baby won't survive. Treatment for HLHS starts as soon as the baby is born.</p> <h4>Medicines</h4> <p>Immediate treatment includes a medicine called <strong><a class="kh_anchor">prostaglandin</a>.</strong> Prostaglandin keeps the PDA open so oxygen can get to the body.</p> <p>Some babies need other medicines to help balance how much blood goes to the lungs and how much goes to the body.</p> <p>Also if the ASD is too small, it might be made bigger. This is done with a <a href="https://kidshealth.org/ws/RadyChildrens/en/parents/cardiac-catheter.html/">cardiac catheterization</a> procedure or by surgery.</p> <h4>The Surgeries</h4> <p>Because the left ventricle and aorta are too small, the goals of the surgeries are to:</p> <ul class="kh_longline_list"> <li>Use the right ventricle to pump oxygen to the body.</li> <li>Send the blood coming back from the body straight to the lungs without having to pass through the heart.</li> </ul> <p>Surgeries are done in stages as a baby grows. They are (in order):</p> <ol> <li><strong><a href="https://kidshealth.org/ws/RadyChildrens/en/parents/norwood.html/">Norwood Procedure</a></strong></li> <li><strong><a href="https://kidshealth.org/ws/RadyChildrens/en/parents/glenn.html/">Glenn Procedure</a></strong></li> <li><strong><a href="https://kidshealth.org/ws/RadyChildrens/en/parents/fontan.html/">Fontan Procedure</a></strong></li> </ol> <h3>Looking Ahead</h3> <p>Being actively involved in your child's care plan can help you feel more in control.</p> <ul class="kh_longline_list"> <li>Take things one day at a time.</li> <li>Look to your care team to guide you on caring for your child, getting ready for surgery, recovery, and more. If you have questions, ask.</li> <li>Learn from others. Find a support group for families dealing with a heart problem or talk to other parents whose kids have had heart surgery. Support groups include: <ul> <li>the <a href="https://npcqic.org/">National Pediatric Cardiology Quality Improvement Collaborative (NPCQJC)</a></li> <li><a href="http://www.sistersbyheart.org/">Sisters By Heart</a></li> <li>the <a href="http://conqueringchd.org/">Pediatric Congenital Heart Association (PCHA)</a></li> </ul> </li> <li>Accept help when you can, and lean on those around you for support.</li> <li>Keep a journal to record your questions for the medical team.</li> <li>Partner with your care team to face the challenges ahead.</li> </ul>Síndrome de corazón izquierdo hipoplásicoEl síndrome de corazón izquierdo hipoplásico es un defecto de nacimiento poco frecuente que afecta al corazón del bebé.https://kidshealth.org/ws/RadyChildrens/es/parents/hypoplastic-heart-es.html/95b99b5d-10d3-4b66-a981-f4d62b4585b0
Birth DefectsSome birth defects are minor and cause no problems; others cause major disabilities. Learn about the different types of birth defects, and how to help prevent them.https://kidshealth.org/ws/RadyChildrens/en/parents/birth-defects.html/eeaa74ff-3f65-4df3-8757-9df2d014c2ee
Ebstein AnomalyEbstein anomaly is a rare heart defect that affects the tricuspid valve. It can cause problems that range from very mild to very serious.https://kidshealth.org/ws/RadyChildrens/en/parents/ebstein-anomaly.html/a783cf1a-8445-4909-a91b-8cd9f04e1683
Heart MurmursHeart murmurs are very common, and most are no cause for concern and won't affect a child's health.https://kidshealth.org/ws/RadyChildrens/en/parents/murmurs.html/9e6ab8dd-2a20-40ab-8625-3e956311e737
Heart and Circulatory SystemThe heart and circulatory system are our body's lifeline, delivering blood to the body's tissues. Brush up on your ticker with this body basics article.https://kidshealth.org/ws/RadyChildrens/en/parents/heart.html/52398b6a-54a6-4272-a569-42ed5b12aeac
Interrupted Aortic Arch (IAA)An interrupted aortic arch (IAA) is a rare heart condition in which the aorta doesn’t form completely. Surgery must be done within the first few days of a baby’s life to close the gap in the aorta. https://kidshealth.org/ws/RadyChildrens/en/parents/aortic-arch.html/d892fcbc-6205-4d08-83cb-903e58b78f39
Patent Ductus Arteriosus (PDA)The ductus arteriosus is a blood vessel that connects two major arteries before birth and normally closes after a baby is born. If it stays open, the result is a condition called patent ductus arteriosus (PDA).https://kidshealth.org/ws/RadyChildrens/en/parents/patent-ductus-arteriosus.html/1220a363-ed10-4541-94c6-ecb923902cd8
Patent Foramen Ovale (PFO)The foramen ovale is a normal opening between the upper two chambers of an unborn baby’s heart. It usually closes soon after the baby’s birth — when it doesn't, it's called a patent foramen ovale. https://kidshealth.org/ws/RadyChildrens/en/parents/pfo.html/27e4037d-e150-4b0f-a5b9-07375901eff3
When Your Child Needs a Heart TransplantIf your child needs a heart transplant, you're probably feeling lots of emotions. Fortunately, many kids who undergo heart transplants go on to live normal, healthy lives.https://kidshealth.org/ws/RadyChildrens/en/parents/heart-transplant.html/0c94f853-a992-413c-b67f-2ed97ac42ec6
kh:age-allAgesOrAgeAgnostickh:age-babyZeroToOnekh:clinicalDesignation-cardiothoracicSurgerykh:genre-articlekh:primaryClinicalDesignation-cardiologySingle Ventricle Defectshttps://kidshealth.org/ws/RadyChildrens/en/parents/single-ventricle-defects/803dc5d9-9021-4e70-aed9-292391bfefa7Heart & Blood Vesselshttps://kidshealth.org/ws/RadyChildrens/en/parents/medical/heart/e9ef0549-4392-4778-974d-753019ce4b8bHeart Conditionshttps://kidshealth.org/ws/RadyChildrens/en/parents/hearthealth/heartconditions/ba7116cf-3c46-4896-8429-8be5c439795ehttps://kidshealth.org/EN/images/illustrations/HLHSestab_415X415_enIL.jpghttps://kidshealth.org/EN/images/illustrations/HLHSCloseASDandPDA_415X415_enIL.jpghttps://kidshealth.org/EN/images/illustrations/HLHSbloodflow_415X415_enIL.jpg