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Hypoplastic
VentricleMediana Sutopo Liedapraja
PPDS Tahap 2ADepartemen Obstetri dan
Gineolo!i
"#$%&'S(M
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Hypoplastic RightVentricle•Complex cardiacmalformation
• Prenatal sonographicfeatures: Pulmonary atresiawith an intact ventricular
septum, tricuspid atresia,tricuspid regurgitation, andhypoplasia of rightventricular walls.
• Signicant disproportion insi!e "etween twoventricular cavities.
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$i'erential $iagnosis
•
(etal )chocardiography
only one ventricle is clearly visi"le
$$*
- Hypoplastic left heart syndrome
- Right sided cardiac masses
-Pulmonary stenosis
- +niventricular heart
$oppler echocardiography a"sence of antegrade ow throupulmonary valve - conrm presence of complete pulmonary a
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reatment
/rup C
Severe hypoplasiaand infundi"ulumcompletely atretic
$uctal dependent
/rup %
- mild hypoplasia andmem"ranouspulmonary atresia ispresent&
Catheter "asedapproach
0aser assistedvalvotomy
1alloon pulmonaryvalvuloplasty&
2 Prolongedprostaglandin infusion2 P$% Stenting or1laloc32aussig shunt
/rup 1
4oderate5nfundi"u
2 Pulmona
-laser ass
radiofre62Stenting
2 1alloon septostom
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Hypoplastic Le)tVentricle
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• Hypoplastic left heart syndrome involving aortic atresia with or
without mitral atresia or stenosis.• $iagnosed as severe ventricular
hypoplasia "efore 78 wga
4alformation can include :
9. Congenital hypoplasia of the leftventricular wall
7. %tresia of aortic and mitral valve
. Coarctation or hypoplasia of aorticarch
Hypoplastic Le)t Ventricle
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Hypoplastic 0eft Heart Syndro-H0HS&
• 4ay evolve duringprenatal life fromsevere aortic stenosis
• Result in decreasedright to left shunting"etween atria
• Hypoplasia of the leftventricle
Prenatal
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Postnatal
• 0eft to right shunting
• Returning oxygenated"lood from pulmonary veinthrough a patent foramenovale into right atrium.
• Right ventricle provide "oth
pulmonary and systemiccirculation
• 4itral valve is hypoplastic
• ricuspid valve is oftenlarge and regurgitant
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H0HS often associated with other cardiac anomalies:
- Ventricular septal defect
- %ortic2arch interruption
- ransposition of the great vessels
C#S %"normalities associated with H0HS:
- 4icrocephaly- Holoprosencephaly
- %genesis of the corpus callosum
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Postnatal Therapy
•
5nfusion prostaglandin )9• Surgical neonatal heart
transplantation
#orwood procedure - stages&
Survival after surgery ;;2
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