Hypoplastic Left Heart Syndrome.ppt
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Transcript of Hypoplastic Left Heart Syndrome.ppt
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Hypoplastic Left Heart Syndrome (HLHS)
Present by 4A Ri 田馥綿
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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
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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
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Epidemiology
CHD: 4.9 per 1,000 livebirths HLHS: 1.8 per 10,000 livebirths Account for 3.8% of all CHD(8th)
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Hypothesis
A late phenomenon after embryogenesis, eg. Viral illness, hypoxemic event.
Diminished flow from foramen ovale LV hypoplasia
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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)
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Clinical menifestations Heart failure: dyspnea, hepatomegaly, lo
w C.O. Weak or absent peripheral pulse Palpable RV parasternal lift, systolic mur
mur
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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
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Pre-op management
Correct acidosis and hypoglycemia Support systemic blood flow: PGE1 for P
DA Avoid excessive pul. blood flow
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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
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Norwood procedure
Atrial septectomy Ductus arteriosus ligation Form a neoaorta Aortopulmonary shunt
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Hemi-Fontan: Connect SVC to pulmonary arteries Fontan: Connect IVC to pulmonary arteries
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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
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Complications s/p Fontan procedure Transient superior vena cava
syndrome Persistent pleural or pericardial
effusion
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Prognosis of surgical treatment
Success rate (survival to discharge):
Stage1:75%
Stage2:95%
Stage3:70%
5-year survival: 70%
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Quality-of-life outcomes
Behavioural abnormalities, learning disabilities, lower intelligence scores
Multifactors:1. Possible associated abnormalities of CNS2. Hemodynamic instability in the pre-op perio
d3. Intra-op perfusion --> neurologic injury
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Risk factors with HLHS
Prematurity/low birth weight Chromosomal and other extracardiac an
omalies Additional intracardiac lesions/anatomic
variants Obstructed pulmonary venous return
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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
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Thanks for your attention!!!