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    Hypoplastic Left Heart Syndrome

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    Khaled S. Harb
    Hypoplastic Left Heart Syndrome (HLHS) is a congenital heart defect where the left ventricle and ascending aorta are severely underdeveloped. It requires staged surgical repairs, starting with the Norwood procedure in the first days of life to reconstruct the aorta, followed by the hemi-Fontan and Fontan procedures by age 3 to separate pulmonary and systemic blood flow. While surgical techniques have improved outcomes, HLHS still carries a high risk of mortality and morbidities like neurodevelopmental delays. Ongoing research aims to further enhance survival and quality of life for children with this critical condition.

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    Hypoplastic Left Heart Syndrome

    Uploaded by

    Khaled S. Harb
    174 views26 pages
    Hypoplastic Left Heart Syndrome (HLHS) is a congenital heart defect where the left ventricle and ascending aorta are severely underdeveloped. It requires staged surgical repairs, starting with the Norwood procedure in the first days of life to reconstruct the aorta, followed by the hemi-Fontan and Fontan procedures by age 3 to separate pulmonary and systemic blood flow. While surgical techniques have improved outcomes, HLHS still carries a high risk of mortality and morbidities like neurodevelopmental delays. Ongoing research aims to further enhance survival and quality of life for children with this critical condition.

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    HLHS

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    Hypoplastic Left Heart Syndrome (HLHS) is a congenital heart defect where the left ventricle and ascending aorta are severely underdeveloped. It requires staged surgical repairs, starting with the Norwood procedure in the first days of life to reconstruct the aorta, followed by the hemi-Fontan and Fontan procedures by age 3 to separate pulmonary and systemic blood flow. While surgical techniques have improved outcomes, HLHS still carries a high risk of mortality and morbidities like neurodevelopmental delays. Ongoing research aims to further enhance survival and quality of life for children with this critical condition.

    Copyright:

    © All Rights Reserved
    Download as PPT, PDF, TXT or read online from Scribd
    Download as ppt, pdf, or txt
    174 views26 pages

    Hypoplastic Left Heart Syndrome

    Uploaded by

    Khaled S. Harb
    Hypoplastic Left Heart Syndrome (HLHS) is a congenital heart defect where the left ventricle and ascending aorta are severely underdeveloped. It requires staged surgical repairs, starting with the Norwood procedure in the first days of life to reconstruct the aorta, followed by the hemi-Fontan and Fontan procedures by age 3 to separate pulmonary and systemic blood flow. While surgical techniques have improved outcomes, HLHS still carries a high risk of mortality and morbidities like neurodevelopmental delays. Ongoing research aims to further enhance survival and quality of life for children with this critical condition.

    Copyright:

    © All Rights Reserved
    Download as PPT, PDF, TXT or read online from Scribd
    Download as ppt, pdf, or txt
    of 26

    Hypoplastic Left Heart

    Syndrome (HLHS)

    Present by 4A Ri 田馥綿
    History
     1952, Lev's : congenital cardiac
    malformations associated with
    underdevelopment of the chambers
    on the left side and a small ascending
    aorta and arch.
     1958, Noonan and Nadas: describe
    the morphologic features of combined
    aortic and mitral atresia
    Introduction
     Marked hypoplasia of the left ventricle
    and ascending aorta
     The aortic and mitral valves are
    atretic, hypoplastic, or stenostic
     A large patent ductus arteriosus
    supplies blood to the systemic
    circulation
    Epidemiology
     CHD: 4.9 per 1,000 livebirths
     HLHS: 1.8 per 10,000 livebirths
     Account for 3.8% of all CHD(8th)
    Hypothesis
     A late phenomenon after
    embryogenesis, eg. Viral illness,
    hypoxemic event.
     Diminished flow from
    foramen ovale
     LV hypoplasia
    History
     Routine obstetrical ultrasound
    examination
     2-5%: respiratory symptoms and
    systemic cyanosis at birth
     First 24-48 hrs: cyanosis, tachypnea,
    respiratory distress (closure of ductus
    arteriosus)
    Clinical menifestations
     Heart failure: dyspnea, hepatomegaly,
    low C.O.
     Weak or absent peripheral pulse
     Palpable RV parasternal lift, systolic
    murmur
    Prognosis & complications
     Morality: 90% in the 1st month,
    usually during 1st wk or two.
     80-90% survival rates for the first-
    stage Norwood operation
     Heart transplantation
     1/3: CNS abnormality
    Pre-op management
     Correct acidosis and hypoglycemia
     Support systemic blood flow: PGE1
    for PDA
     Avoid excessive pul. blood flow
    Surgical therapy
     1979, Norwood: first successful palliation
    on a neonate.
     3 stages:
    1. The Norwood procedure (first 2 wks)
    --reconstruction of Aorta
    2. Hemi-Fontan (4-6 months)
    3. Fontan (18-36 months)
    --separate pulmonary and systemic flow
    Norwood procedure
     Atrial septectomy
     Ductus arteriosus ligation
     Form a neoaorta
     Aortopulmonary shunt
     Hemi-Fontan:
    Connect SVC to pulmonary arteries
     Fontan:
    Connect IVC to pulmonary arteries
    Complications s/p Norwood
    procedure

     Bleeding, low cardiac output


    syndrome, and arrhythmia
     Aortic arch obstruction at the site of
    surgical anastomosis
     Progressive cyanosis caused by
    limited blood flow through the shunt
    Complications s/p Fontan procedure
     Transient superior vena cava
    syndrome
     Persistent pleural or pericardial
    effusion
    Prognosis of surgical treatment
     Success rate (survival to discharge):
    Stage1:75%
    Stage2:95%
    Stage3:70%
     5-year survival: 70%
    Quality-of-life outcomes
     Behavioural abnormalities, learning
    disabilities, lower intelligence scores
     Multifactors:
    1. Possible associated abnormalities of CNS
    2. Hemodynamic instability in the pre-op
    period
    3. Intra-op perfusion --> neurologic injury
    Risk factors with HLHS
     Prematurity/low birth weight
     Chromosomal and other extracardiac
    anomalies
     Additional intracardiac
    lesions/anatomic variants
     Obstructed pulmonary venous return
    Summary
     Early diagnosis, operative techniques
    for HLHS: progress in the care of
    these children
     Define the optimal strategies to
    improve survival and quality of life
    Thanks for your attention!!!

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