II. 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)

III. Associated Conditions

  1. Atrial Septal Defect (ASD) or Patent Foramen Ovale
  2. Patent Ductus Arteriousus
  3. Aortic Coarctation

IV. Management: Acute decompensation following first Norwood Procedure

  1. Apply oxygen, but aim Oxygen Saturation to 75-85%
  2. Oxygen results in vasodilation of pulmonary vessels and shunts flow away from systemic circulation
    1. Excessive oxygen risks serious Hypotension, shock or death
  3. Emergent pediatric cardiology or cardiothoracic surgery Consultation
    1. Risk of lethal shunt closure requiring immediate management

V. Management: Surgery

  1. Uniformly fatal without surgical correction
  2. Procedures
    1. Heart Transplantation or
    2. Norwood Procedure (3 stage, very complex repair)
      1. Establishes permanent flow between right ventricle and aorta
      2. High mortality in the first 24-48 hours of procedure
      3. Stage 1: Norwood (aorto-pulmonary artery shunt, most unstable after this stage)
      4. Stage 2: Fontan or Glenn
      5. Stage 3: Fontan
  3. Post-operative decompensation management (post-Norwood)
    1. At risk for severe decompensation even with mild viral infections
    2. Present with increased systemic vascular resistance (cold, cyanotic digits) and Metabolic Acidosis
    3. Treatment options
      1. Avoid excessive Supplemental Oxygen (Oxygen Saturation goal 75-85%) to maintain systemic perfusion
      2. Consider small fluid Normal Saline bolus trials (5-10 ml/kg)

VI. 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

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Ontology: 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