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| | __NOTOC__ |
| {{Infobox_Disease | | | {{Infobox_Disease | |
| Name = Hypoplastic left heart syndrome | | | Name = Hypoplastic left heart syndrome | |
| Image = Hypoplastic left heart syndrome.svg | | | Image = Hypoplastic left heart syndrome.svg | |
| Caption = Diagram of a healthy heart and one suffering from Hypoplastic left heart syndrome | | | Caption = Diagram of a healthy heart and one suffering from Hypoplastic left heart syndrome | |
| Width = 248 |
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| DiseasesDB = 31507 |
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| ICD10 = {{ICD10|Q|23|4|q|20}} |
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| ICD9 = {{ICD9|746.7}} |
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| ICDO = |
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| OMIM = 241550 |
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| MedlinePlus = 001106 |
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| eMedicineSubj = ped |
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| eMedicineTopic = 1131 |
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| MeshName = Hypoplastic+Left+Heart+Syndrome |
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| MeshNumber = C14.240.400.625 |
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| }} | | }} |
| {{WikiDoc Cardiology Network Infobox}} | | {{Hypoplastic left heart syndrome}} |
| {{Search infobox}}
| | '''For patient information click [[Hypoplastic left heart syndrome (patient information)|here]]''' |
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| '''Associate Editors-In-Chief:''' {{CZ}}; [[User:KeriShafer|Keri Shafer, M.D.]] [mailto:kshafer@bidmc.harvard.edu] | | {{CMG}}; '''Associate Editor(s)-In-Chief:''' [[Priyamvada Singh|Priyamvada Singh, M.B.B.S.]][mailto:psingh13579@gmail.com], {{CZ}}, [[User:KeriShafer|Keri Shafer, M.D.]] [mailto:kshafer@bidmc.harvard.edu]; '''Assistant Editor(s)-In-Chief:''' [[Kristin Feeney|Kristin Feeney, B.S.]][mailto:kfeeney@elon.edu] |
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| {{Editor Join}} | | {{SK}} HLHS |
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| ==Overview== | | ==[[Hypoplastic left heart syndrome overview|Overview]]== |
| [[Hypoplastic left heart syndrome]] (also known as HLHS), is a rare [[congenital heart defect]] in which the left side of the [[heart]] is severely underdeveloped. In babies with HLHS, the [[aorta]] and [[left ventricle]] are very small, and the [[aortic valve|aortic]] and [[mitral valve]]s are either too small to allow sufficient blood flow or are atretic (closed) altogether. As [[blood]] returns from the [[lung]]s to the [[left atrium]], it must pass through an [[atrial septal defect]] to the right side of the heart. | |
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| In a healthy human, the left side of the heart receives [[oxygen]]-rich blood from the lungs and pumps it out to the rest of the body; with these structures underdeveloped, they cannot circulate blood to other organs, and the right ventricle must pump blood to both the lungs, as it would normally, and to the rest of the body, a situation which cannot be sustained for long.
| | ==[[Hypoplastic left heart syndrome historical perspective|Historical Perspective]]== |
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| In cases of HLHS, the right side of the heart often must pump blood to the body through a [[patent ductus arteriosus|patent]] [[ductus arteriosus]]. As the ductus arteriosus closes a few days after birth, blood flow is severely restricted and eventually cut off, leading to dangerously low circulation and eventually to shock.
| | ==[[Hypoplastic left heart syndrome pathophysiology|Pathophysiology]]== |
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| ==Treatment== | | ==[[Hypoplastic left heart syndrome causes|Causes]]== |
| Without treatment, HLHS is fatal, but with intervention within a few days after birth, an infant may survive. A [[pediatric]] [[cardiac surgeon]] may perform a series of operations or a full [[heart transplant]]. In the meantime, the ductus may be kept open to allow blood flow using medication containing [[prostaglandin]]. Because these operations are complex and need to be individualized for each patient, a cardiologist must assess all medical and surgical option on a case-by-case basis.
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| The two methods for treatment of HLHS are transplantation and a 3-stage surgical procedure. The 3-stage procedure is a palliative procedure (not a cure), as the child's circulation is made to work with only two of the heart's four chambers. The first step is the [[Norwood procedure]] or the Sano shunt. The first step makes the right ventricle pump blood to the whole body (which the left ventricle normally does) AND to the lungs. During this time the baby may be medically fragile and have feeding problems because the heart is working very hard. The second stage, the Glenn or Hemi-Fontan, allows greater blood flow to the upper body and reduces some of the workload from the right ventricle. The final procedure, the Fontan ([[Fontan procedure]]) allows blood to automatically flow to the lungs (rather than being pumped there) and allows the right ventricle to only pump blood out to the body. The Norwood procedure is generally performed within a week of birth, the second stage at 3-6 months of age, and the Fontan at 18 months to 4 years of age. There are two types of Fontan: the Lateral Tunnel Fontan, and the Extracardiac Fontan.
| | ==[[Hypoplastic left heart syndrome differential diagnosis|Differentiating Hypoplastic left heart syndrome from other Diseases]]== |
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| ==Prognosis== | | ==[[Hypoplastic left heart syndrome epidemiology and demographics|Epidemiology and Demographics]]== |
| While infants successfully treated for HLHS have a good chance of survival, they may experience chronic health problems for the rest of their lives. The 3-stage surgeries were developed in the early 1980's with no survivors prior to that time. Therefore, the earliest survivors are in their early 20's and the long term prognosis is unknown. However, the advances in surgical and medical techniques have helped increase the survival rate dramatically since the surgeries were first developed.
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| As is true for patients with other types of heart defects involving malformed valves [http://www.mayoclinic.com/health/endocarditis/DS00409/DSECTION=4], HLHS patients run a high risk of [[endocarditis]], and must be monitored by a cardiologist for the rest of their lives to check on their heart function.
| | ==[[Hypoplastic left heart syndrome natural history|Natural History, Complications, and Prognosis]]== |
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| ==Pathological Findings== | | ==Diagnosis== |
| [http://www.peir.net Images courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology] | | [[Hypoplastic left heart syndrome history and symptoms|History and Symptoms]] | [[Hypoplastic left heart syndrome physical examination|Physical Examination]] | [[Hypoplastic left heart syndrome laboratory tests|Laboratory Findings]] | [[Hypoplastic left heart syndrome electrocardiogram | Electrocardiogram]] | [[Hypoplastic left heart syndrome chest x ray|Chest X Ray]] | [[Hypoplastic left heart syndrome echocardiography or ultrasound|Echocardiography]] | [[Hypoplastic left heart syndrome other imaging findings|Other Imaging Findings]] |
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| <div align="left">
| | ==Treatment== |
| <gallery heights="175" widths="175">
| | [[Hypoplastic left heart syndrome medical therapy|Medical Therapy]] | [[Hypoplastic left heart syndrome surgery|Surgery]] | [[Hypoplastic left heart syndrome primary prevention|Primary Prevention]] | [[Hypoplastic left heart syndrome cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Hypoplastic left heart syndrome future or investigational therapies|Future or Investigational Therapies]] |
| Image:Hypoplastic left ventricle 1.jpg|
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| Image:Hypoplastic left ventricle 2.jpg|
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| </gallery>
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| </div>
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| | ==Case Studies== |
| | [[Hypoplastic left heart syndrome case study one|Case #1]] |
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| <div align="left">
| | ==Related Chapters== |
| <gallery heights="175" widths="175">
| | *[[Congenital heart disease]] |
| Image:Hypoplastic left ventricle 3.jpg|
| | *[[Norwood procedure]] |
| Image:Hypoplastic left ventricle 4.jpg|
| | *[[Tetralogy of fallot]] |
| </gallery>
| | *[[Edward Bove]] |
| </div>
| | *[[Hypoplastic right heart syndrome]] |
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| | {{WikiDoc Help Menu}} |
| | {{WikiDoc Sources}} |
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| <div align="left">
| | [[Category:Cardiology]] |
| <gallery heights="175" widths="175">
| | [[Category:Disease]] |
| Image:Hypoplastic left ventricle 5.jpg|
| | [[Category:Neonatology]] |
| Image:Hypoplastic left ventricle.jpg|
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| </gallery>
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| </div>
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| ==External links==
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| * [http://heartcenter.seattlechildrens.org/conditions_treated/hypoplastic_left_heart.asp Hypoplastic Left Heart Syndrome information] from Seattle Children's Hospital Heart Center
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| * [http://www.tchin.org/ Congenital Heart Information Network]
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| * [http://www.hlhsinfo.homestead.com/ HLHS Information Page] - created for parents expecting a child with HLHS. Includes questions to ask the physicians and insurance company.
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| * [http://www.caheartconnection.org/ California Heart Connection] - support network for those with congenital heart defects including Hypoplastic Left Heart Syndrome (HLHS.) Free online support group
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| ==Sources==
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| *[http://www.card-ag.org Card-AG], The Cardiologycal Working Group of the University Pediatric Clinic Munster
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| *[http://www.americanheart.org/presenter.jhtml?identifier=1353 Hypoplastic Left Heart Syndrome], American Heart Association
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| *Information on HLHS from the [http://www.cincinnatichildrens.org/health/heart-encyclopedia/anomalies/hlhs.htm Cincinnati Children's Hospital Medical Center], accessed 9 July, 2006
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| [[Category:Congenital heart disease]] | | [[Category:Congenital heart disease]] |
| | | [[Category:Pediatrics]] |
| *Hypoplastic Left Heart Syndrome - [http://www.med.umich.edu/cvc/mchc/parhyp.htm University of Michigan Congenital Heart Center]
| | [[Category:Mature chapter]] |
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| {{Congenital malformations and deformations of circulatory system}}
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| {{SIB}}
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| [[de:Hypoplastisches Linksherz-Syndrom]] | |
| [[nn:Hypoplastisk venstre hjarte-syndrom]]
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| [[uk:Синдром гіпоплазії лівих відділів серця]]
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| [[Category:Cardiology]] | |
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| {{WikiDoc Help Menu}}
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| {{WikiDoc Sources}}
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