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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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 H%#> ?@+