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Hypoplastic Left Heart Syndrome (HLHS) Present by 4A Ri  田馥綿
History <ul><li>1952, Lev's : congenital cardiac malformations associated with underdevelopment of the chambers on the lef...
Introduction  <ul><li>Marked hypoplasia of the left ventricle and ascending aorta </li></ul><ul><li>The aortic and mitral ...
 
 
Epidemiology <ul><li>CHD: 4.9 per 1,000 livebirths </li></ul><ul><li>HLHS: 1.8 per 10,000 livebirths </li></ul><ul><li>Acc...
Hypothesis <ul><li>A late phenomenon after embryogenesis, eg. Viral illness, hypoxemic event. </li></ul><ul><li>Diminished...
History <ul><li>Routine obstetrical ultrasound examination  </li></ul><ul><li>2-5%: respiratory symptoms and systemic cyan...
Clinical menifestations <ul><li>Heart failure: dyspnea, hepatomegaly, low C.O. </li></ul><ul><li>Weak or absent peripheral...
Prognosis & complications <ul><li>Morality: 90% in the 1st month, usually during 1st wk or two. </li></ul><ul><li>80-90% s...
Pre-op management <ul><li>Correct acidosis and hypoglycemia </li></ul><ul><li>Support systemic blood flow: PGE1 for PDA </...
Surgical therapy <ul><li>1979, Norwood: first successful palliation on a neonate. </li></ul><ul><li>3 stages: </li></ul><u...
Norwood procedure <ul><li>Atrial septectomy </li></ul><ul><li>Ductus arteriosus ligation </li></ul><ul><li>Form a neoaorta...
 
 
<ul><li>Hemi-Fontan: </li></ul><ul><li>Connect SVC to pulmonary arteries </li></ul><ul><li>Fontan: </li></ul><ul><li>Conne...
 
 
 
Complications s/p Norwood procedure <ul><li>Bleeding, low cardiac output syndrome, and arrhythmia  </li></ul><ul><li>Aorti...
Complications s/p Fontan procedure <ul><li>Transient superior vena cava syndrome </li></ul><ul><li>Persistent pleural or p...
Prognosis of surgical treatment <ul><li>Success rate (survival to discharge): </li></ul><ul><li>Stage1:75% </li></ul><ul><...
Quality-of-life outcomes <ul><li>Behavioural abnormalities, learning disabilities, lower intelligence scores </li></ul><ul...
Risk factors with HLHS <ul><li>Prematurity/low birth weight </li></ul><ul><li>Chromosomal and other extracardiac anomalies...
Summary  <ul><li>Early diagnosis, operative techniques for HLHS: progress in the care of these children </li></ul><ul><li>...
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  • Develop in Mid-to-late gestation the primary abnormality occurs during aortic and mitral valve development. During cardiac development, adequate flow of blood through a structure is largely responsible for the growth of that structure. With little or no blood flow because of aortic and mitral valve atresia, growth of the left ventricle does not occur 2.significant obstruction to pulmonary venous return (a congenitally small or absent patent foramen ovale) usually is present.
  • Most studies report neurodevelopmental disabilities in a significant number of patients who survive either staged surgical reconstruction or cardiac transplantation.
  • Increased alveolar oxygen decreases pulmonary vascular resistance, leading to increased pulmonary blood flow. Therefore, most infants should remain in room air with acceptable oxygen saturation (pulse oximeter) in the low 70s
  • 1.Poor peripheral and end-organ perfusion may represent poor cardiac output or pulmonary overcirculation, which may be treated by inotropic support or manipulation of relative pulmonary and systemic resistances. 2. Shunt complications, such as thrombosis, can occur. All patients are started on low-dose aspirin when they begin enteral nutrition
  • Prenatal diagnosis &amp; improved survival with Norwood--&gt; 需要開始 identify 何種 patient 數於 high risk of poor prognosis--&gt;affect postnatal management and surgical options (transplant or Norwood)
  • Transcript of "Hypoplastic Left Heart Syndrome.ppt"

    1. 1. Hypoplastic Left Heart Syndrome (HLHS) Present by 4A Ri 田馥綿
    2. 2. History <ul><li>1952, Lev's : congenital cardiac malformations associated with underdevelopment of the chambers on the left side and a small ascending aorta and arch. </li></ul><ul><li>1958, Noonan and Nadas: describe the morphologic features of combined aortic and mitral atresia </li></ul>
    3. 3. Introduction <ul><li>Marked hypoplasia of the left ventricle and ascending aorta </li></ul><ul><li>The aortic and mitral valves are atretic, hypoplastic, or stenostic </li></ul><ul><li>A large patent ductus arteriosus supplies blood to the systemic circulation </li></ul>
    4. 6. Epidemiology <ul><li>CHD: 4.9 per 1,000 livebirths </li></ul><ul><li>HLHS: 1.8 per 10,000 livebirths </li></ul><ul><li>Account for 3.8% of all CHD(8th) </li></ul>
    5. 7. Hypothesis <ul><li>A late phenomenon after embryogenesis, eg. Viral illness, hypoxemic event. </li></ul><ul><li>Diminished flow from </li></ul><ul><li>foramen ovale </li></ul><ul><li> LV hypoplasia </li></ul>
    6. 8. History <ul><li>Routine obstetrical ultrasound examination </li></ul><ul><li>2-5%: respiratory symptoms and systemic cyanosis at birth </li></ul><ul><li>First 24-48 hrs: cyanosis, tachypnea, respiratory distress (closure of ductus arteriosus) </li></ul>
    7. 9. Clinical menifestations <ul><li>Heart failure: dyspnea, hepatomegaly, low C.O. </li></ul><ul><li>Weak or absent peripheral pulse </li></ul><ul><li>Palpable RV parasternal lift, systolic murmur </li></ul>
    8. 10. Prognosis & complications <ul><li>Morality: 90% in the 1st month, usually during 1st wk or two. </li></ul><ul><li>80-90% survival rates for the first-stage Norwood operation </li></ul><ul><li>Heart transplantation </li></ul><ul><li>1/3: CNS abnormality </li></ul>
    9. 11. Pre-op management <ul><li>Correct acidosis and hypoglycemia </li></ul><ul><li>Support systemic blood flow: PGE1 for PDA </li></ul><ul><li>Avoid excessive pul. blood flow </li></ul>
    10. 12. Surgical therapy <ul><li>1979, Norwood: first successful palliation on a neonate. </li></ul><ul><li>3 stages: </li></ul><ul><li>1. The Norwood procedure (first 2 wks) </li></ul><ul><li>--reconstruction of Aorta </li></ul><ul><li>2. Hemi-Fontan (4-6 months) </li></ul><ul><li>3. Fontan (18-36 months) </li></ul><ul><li>--separate pulmonary and systemic flow </li></ul>
    11. 13. Norwood procedure <ul><li>Atrial septectomy </li></ul><ul><li>Ductus arteriosus ligation </li></ul><ul><li>Form a neoaorta </li></ul><ul><li>Aortopulmonary shunt </li></ul>
    12. 16. <ul><li>Hemi-Fontan: </li></ul><ul><li>Connect SVC to pulmonary arteries </li></ul><ul><li>Fontan: </li></ul><ul><li>Connect IVC to pulmonary arteries </li></ul>
    13. 20. Complications s/p Norwood procedure <ul><li>Bleeding, low cardiac output syndrome, and arrhythmia </li></ul><ul><li>Aortic arch obstruction at the site of surgical anastomosis </li></ul><ul><li>Progressive cyanosis caused by limited blood flow through the shunt </li></ul>
    14. 21. Complications s/p Fontan procedure <ul><li>Transient superior vena cava syndrome </li></ul><ul><li>Persistent pleural or pericardial effusion </li></ul>
    15. 22. Prognosis of surgical treatment <ul><li>Success rate (survival to discharge): </li></ul><ul><li>Stage1:75% </li></ul><ul><li>Stage2:95% </li></ul><ul><li>Stage3:70% </li></ul><ul><li>5-year survival: 70% </li></ul>
    16. 23. Quality-of-life outcomes <ul><li>Behavioural abnormalities, learning disabilities, lower intelligence scores </li></ul><ul><li>Multifactors: </li></ul><ul><li>1. Possible associated abnormalities of CNS </li></ul><ul><li>2. Hemodynamic instability in the pre-op period </li></ul><ul><li>3. Intra-op perfusion --> neurologic injury </li></ul>
    17. 24. Risk factors with HLHS <ul><li>Prematurity/low birth weight </li></ul><ul><li>Chromosomal and other extracardiac anomalies </li></ul><ul><li>Additional intracardiac lesions/anatomic variants </li></ul><ul><li>Obstructed pulmonary venous return </li></ul>
    18. 25. Summary <ul><li>Early diagnosis, operative techniques for HLHS: progress in the care of these children </li></ul><ul><li>Define the optimal strategies to improve survival and quality of life </li></ul>
    19. 26. Thanks for your attention!!!
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