Cardiovascular Medicine Book

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Hypoplastic Left Heart Syndrome

Aka: Hypoplastic Left Heart Syndrome, Hypoplastic Left Heart, Hyoplasia of the Aortic Tract Complex, HLHS
  1. Definitions
    1. Hypoplastic Left Heart Syndrome
      1. Under-developed entire left heart (left atrium, left ventricle, aorta, aortic valve, mitral valve)
      2. Severe symptom onset as the ductus arteriosus closes
  2. Pathophysiology
    1. Ductus dependent lesion (along with reliance on right ventricle)
    2. Characteristic Anomalies
      1. Left ventricle hypoplasia
      2. Ascending aorta hypoplasia
      3. Aortic Stenosis (or hypoplasia or atresia)
      4. Mitral Stenosis (or hypoplasia or atresia)
  3. Associated Conditions
    1. Atrial Septal Defect (ASD) or Patent Foramen Ovale
    2. Patent Ductus Arteriousus
    3. Aortic Coarctation
  4. Management: Surgery
    1. Uniformly fatal without surgical correction
    2. See Single Ventricle Defect Management
      1. Includes Acute decompensation following first Norwood Procedure
  5. References
    1. Civitarese and Crane (2016) Crit Dec Emerg Med 30(1): 14-23
    2. Sloas and Orman in Majoewsky (2013) EM:Rap 13(9): 10-11
    3. Rao in Berger (2013) Pediatric Hypoplastic Left Heart Syndrome, Emedicine
      1. http://emedicine.medscape.com/article/890196-overview#showall

Hypoplastic Left Heart Syndrome (C0152101)

Definition (MSH) A condition caused by underdevelopment of the whole left half of the heart. It is characterized by hypoplasia of the left cardiac chambers (HEART ATRIUM; HEART VENTRICLE), the AORTA, the AORTIC VALVE, and the MITRAL VALVE. Severe symptoms appear in early infancy when DUCTUS ARTERIOSUS closes.
Definition (NCI) A rare congenital cardiovascular disorder characterized by severe underdevelopment of the left side of the heart. Signs and symptoms include tachypnea, cyanosis, dyspnea, and lethargy.
Concepts Disease or Syndrome (T047) , Congenital Abnormality (T019)
MSH D018636
ICD9 746.7
ICD10 Q23.4
SnomedCT 62067003
English Hypoplastic Left Heart Syndrome, Left Heart Syndrome, Hypoplastic, HLH - Hypoplast lt heart syndr, Hypoplastic lt heart syndrome, Hypoplastic left heart, HLHS - Hypoplas left heart syn, Left Heart Hypoplasia Syndrome, hypoplastic left heart syndrome (diagnosis), hypoplastic left heart syndrome, Hypoplas left heart synd, Hypoplastic Left Heart Syndrome [Disease/Finding], hypoplastic left heart, left hypoplastic heart, heart hypoplastic left, hypoplasia left heart, left heart hypoplastic, heart hypoplastic left syndrome, HLHS, HLHS1, HYPOPLASTIC LEFT HEART SYNDROME 1, Hypoplastic left heart syndrome, HLH - Hypoplastic left heart syndrome, HLHS - Hypoplastic left heart syndrome, Hypoplastic left heart syndrome (disorder), heart; hypoplasia, left heart (syndrome), heart; hypoplasia, left heart syndrome, hypoplasia; heart, left heart (syndrome), hypoplasia; heart, left heart syndrome, hypoplasia; left heart syndrome, hypoplastic left-heart; syndrome, left heart syndrome; hypoplasia, syndrome; heart, hypoplastic left, syndrome; hypoplastic left-heart
Italian Sindrome del cuore sinistro ipoplasico, Sindrome da ipoplasia del cuore sinistro, Sindrome del cuore sinistro ipoplastico
Swedish Hypoplastisk vänsterkammare
Japanese サシンテイケイセイショウコウグン, 左心低形成症候群, Hypoplastic Left Heart症候群, 左心室発育不全症候群, 左心形成不全症, 低形成左心症候群, 左(心)室低形成症候群, 左室低形成症候群, 左室形成不全症候群, 左心(室)形成不全症候群, 左心低形成症, 左心室低形成症候群
Czech syndrom hypoplazie levého srdce, Syndrom hypoplastického levého srdce
Finnish Vajaakehittyneen vasemman sydänpuoliskon oireyhtymä
French Syndrome d'hypoplasie du coeur gauche, Hypoplasie du coeur gauche
Russian LEVOGO SERDTSA GIPOPLASTICHESKII SINDROM, ГИПОПЛАСТИЧЕСКИЙ ЛЕВЫЙ СЕРДЕЧНЫЙ СИНДРОМ, GIPOPLASTICHESKII LEVYI SERDECHNYI SINDROM, ГИПОПЛАСТИЧЕСКИЙ СИНДРОМ ЛЕВЫХ ОТДЕЛОВ СЕРДЦА, GIPOPLASTICHESKII SINDROM LEVYKH OTDELOV SERDTSA, ЛЕВОГО СЕРДЦА ГИПОПЛАСТИЧЕСКИЙ СИНДРОМ
Spanish Síndrome de Hipoplasia del Corazón Izquierdo, Síndrome del Corazón Izquierdo Hipoplásico, síndrome de corazón izquierdo hipoplásico (trastorno), síndrome de corazón izquierdo hipoplásico, Síndrome de corazón izquierdo hipoplásico
Korean 발육부전성 좌심 증후군
Polish Zespół niedorozwoju lewego serca
Hungarian Hypoplastikus balszívfél syndroma
Norwegian Hypoplastisk venstre hjertekammer, Hypoplastisk venstre hjerte-syndrom
Portuguese Síndrome do Coração Esquerdo Hipoplástico, Síndrome de coração esquerdo hipoplásico, Síndrome do Coração Esquerdo Hipoplásico
Dutch hart; hypoplasie, linker hart (syndroom), hart; hypoplasie, linkerhartsyndroom, hypoplasie; hart, linker hart (syndroom), hypoplasie; hart, linkerhartsyndroom, hypoplasie; linkerhartsyndroom, hypoplastisch linker hart; syndroom, linkerhartsyndroom; hypoplasie, syndroom; hart, hypoplastisch linker, syndroom; hypoplastisch linker hart, hypoplastisch linkerhartsyndroom, Hypoplastisch-linkerhartsyndroom, Linkerhartsyndroom, hypoplastisch-, Syndroom, hypoplastisch-linkerhart-
German Linksherz-Hypoplasie Syndrom, Hypoplastisches Linksherzsyndrom, Linksherzsyndrom, hypoplastisches
Sources
Derived from the NIH UMLS (Unified Medical Language System)


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