Hypoplastic

Ventricle
Mediana Sutopo Liedapraja
PPDS Tahap 2A
Departemen Obstetri dan
Ginekologi
FKUI-RSCM

Hypoplastic Right
Ventricle
Complex cardiac
malformation
Prenatal sonographic
features: Pulmonary atresia
with an intact ventricular
septum, tricuspid atresia,
tricuspid regurgitation, and
hypoplasia of right
ventricular walls.
Significant disproportion in
size between two
ventricular cavities.

 Neonates will be ductal

dependent
Provide immediate pediatric
cardiology intervention
Prostaglandin infusion to
maintain PDA
Provide catheter based
intervention
Pulmonary valvotomy
Ductus arterosis stenting and
balloon atrial septostomy)

Differential Diagnosis
Fetal Echocardiography only one ventricle is clearly visible
DD/
- Hypoplastic left heart syndrome
- Right sided cardiac masses
- Pulmonary stenosis
- Univentricular heart
Doppler echocardiography absence of antegrade flow through
pulmonary valve ( confirm presence of complete pulmonary atresia).

Treatment
Grup A

Grup C

Grup B

( mild hypoplasia and

membranous
pulmonary atresia is
present)
Catheter based
approach
Laser assisted
valvotomy
Balloon pulmonary
valvuloplasty)

Severe hypoplasia
and infundibulum
completely atretic

Moderate hypoplasia

Ductal dependent

- Pulmonary valvotomy

- Prolonged
prostaglandin infusion
- PDA Stenting or
Blalock-Taussig shunt

Infundibular hypoplasia

(laser assisted or
radiofrequency)
-Stenting of the PDA
- Balloon atrial
septostomy

Hypoplastic Left
Ventricle

Hypoplastic Left Ventricle

Hypoplastic left heart syndrome
involving aortic atresia with or
without mitral atresia or stenosis.
Diagnosed as severe ventricular
hypoplasia before 20 wga
Malformation can include :
1. Congenital hypoplasia of the left
ventricular wall
2. Atresia of aortic and mitral valve
3. Coarctation or hypoplasia of aortic
arch

Hypoplastic Left Heart Syndrome

(HLHS)
Prenatal
May evolve during
prenatal life from
severe aortic stenosis
Result in decreased
right to left shunting
between atria
Hypoplasia of the left
ventricle

Postnatal
Left to right shunting
Returning oxygenated
blood from pulmonary vein
through a patent foramen
ovale into right atrium.
Right ventricle provide both
pulmonary and systemic
circulation
Mitral valve is hypoplastic
Tricuspid valve is often
large and regurgitant

HLHS often associated with other cardiac anomalies:

- Ventricular septal defect
- Aortic-arch interruption
- Transposition of the great vessels

CNS Abnormalities associated with HLHS:

- Microcephaly
- Holoprosencephaly
- Agenesis of the corpus callosum

Postnatal Therapy
Infusion prostaglandin E1
Surgical neonatal heart
transplantation
Norwood procedure ( 3 stages)
Survival after surgery 55-65%
Palliative

THANK YOU