Congenital

1. HLHS
2019

2. • Atresia of aortic valve
• Or hypolasia or atresia of ao arch
• May be with mitral hypoplasia
• Small LV and LA
• Big RV and RA
• ASD, PDA
• VSD in 10 %

3. • High PVR >>> R-L shunt at PDA level
• ASD L to R
• Severe systemic hypoperfusion with decrease PVR or duct
closure or with narrow ASD
• Clinically shock cyanosis PO acc P2 (single)
• ECG >>> RVH
• Xray PVC
• Echo : small LV, LA
• big RV
• ASD
• PDA
• RETROGRADE AA FLOW

4. hypoplastic left heart syndrome
(HLHS).
• This echocardiographic still
frame shows a long-axis view
of the aortic arch in a patient
with hypoplastic left heart
syndrome (HLHS). The
ascending aorta is markedly
hypoplastic, serving only to
deliver blood in a retrograde
fashion to the coronary
arteries. An echo-bright
coarctation shelf is seen at the
insertion of the ductus
arteriosus.

5. • This echocardiographic still
frame shows a 4-chamber
view of the heart in a
patient with hypoplastic left
heart syndrome (HLHS). A
large right ventricle (RV)
and hypoplastic left
ventricle (star) are seen.
Right atrium = RA. Left
atrium = LA.

6. Blood gas
• Normal Pco2
• Systemic hypoxia (mild)
• Low PH
• Decrease saturation in left side and increased
in right side

7. Date of download:
5/1/2014
Copyright © The American College of Cardiology.
All rights reserved.
From: Cardiac Magnetic Resonance and the Need for Routine Cardiac Catheterization in Single Ventricle
Patients Prior to Fontan: A Comparison of 3 Groups: Pre-Fontan CMR Versus Cath Evaluation
J Am Coll Cardiol. 2012;60(12):1094-1102. doi:10.1016/j.jacc.2012.06.021
CMR Imaging of Single Ventricle Patients Prior to Fontan
(A) Steady-state free precession cine in a 3-chamber (left) and short-axis view (right) of a patient with hypoplastic left heart
syndrome. (B) Three-dimensional gadolinium images of the patient in A from anterior (left) and posterior (right) angles
demonstrating the aortopulmonary anastomosis (Ao-PA), the superior vena cava to right pulmonary artery anastomosis (SVC-RPA)
of a bidirectional Glenn, and the branch pulmonary artery. CMR = cardiac magnetic resonance; DAo = descending aorta; RA = right
atrium; RV = right ventricle.
Figure Legend:

8. Teatment
• PG
• Vent , V ass device
• Atrial spetostmy
• Norwood then BDG>> Fontan

9. prerequest for Norwood
• Good RV function no
increase in Pul pressure or
PVR
• No pulmonary obstruction
• Competent Tricuspid valve

10. The Sano Modification of the Norwood involves the placement of a conduit (light blue
tube below) between the pulmonary artery and the right ventricle instead of the
Modified Blalock-Taussig Shunt.

11. Staged reconstruction is the treatment of choice for hypoplastic
left heart syndrome. Reconstruction takes place in three stages.
• The first stage, known as a Norwood
operation, is undertaken as soon as possible
after birth. This stage calls for a homograft1
to be used to enlarge the rudimentary aortic
arch and then join it to the pulmonary trunk.
This is known as an anastomosis. The
pulmonary trunk will have been
disconnected from the left and right
pulmonary arteries. The newly created blood
vessel functions as the patient's aorta. The
right ventricle then becomes a common
ventricle, pumping blood through the aorta
to the rest of the body.
• ASD enlarge
• Lig the duct
• VR - RARV--. Prox PA --.> Neo
AO- DA and Body and shunt 
distal PA --- > Lung -- LA - RA

12. Staged reconstruction is the treatment of choice for
hypoplastic left heart syndrome.Stage II
• superior vena cava connected
to pulmonary artery
2 - takedown of Blalock-
Taussig shunt
De-oxygenated blood from the
superior vena cava is routed
directly to the lungs. A mix of
oxygenated and de-
oxygenated blood is sent to
the body through the
anastomosed aorta and
pulmonary trunk.
• Lower body VR - RA RV
Neo Aorta
• Upper body VR- PA- LA
RA- RV Neo aorta

13. Staged reconstruction is the treatment of choice for
hypoplastic left heart syndrome.
• Stage 3 of hypoplastic left heart
syndrome reconstruction
(Fenestrated Fontan)
1 - conduit joining inferior vena
cava and superior vena cava to
right pulmonary artery
2 - artificial wall containing small
hole De-oxygenated blood from
the superior vena cava and
inferior vena cava is routed
directly to the lungs. Oxygenated
blood is sent to the body through
the anastomosed aorta and
pulmonary trunk.
• Venous retun- RPA-- Lung -
LA  ASD-RA - RV- PP
trunk- Neo aorta

14. The Sano Modification of the Norwood involves the placement of a conduit (light blue
tube below) between the pulmonary artery and the right ventricle instead of the
Modified Blalock-Taussig Shunt.

15. • Hypoplastic LV

16. Fontan Norwood

17. hypoplastic left heart syndrome
(HLHS).
• This echocardiographic still
frame shows a long-axis view
of the aortic arch in a patient
with hypoplastic left heart
syndrome (HLHS). The
ascending aorta is markedly
hypoplastic, serving only to
deliver blood in a retrograde
fashion to the coronary
arteries. An echo-bright
coarctation shelf is seen at the
insertion of the ductus
arteriosus.

18. • This echocardiographic still
frame shows a 4-chamber
view of the heart in a
patient with hypoplastic left
heart syndrome (HLHS). A
large right ventricle (RV)
and hypoplastic left
ventricle (star) are seen.
Right atrium = RA. Left
atrium = LA.

19. Hypoplastic LV after Fontan