Hypoplastic Left Ventricle
1. Anatomy
Hypoplastic LV and ascending aorta
Aortic atresia/stenosis
Mitral atresia/stenosis
Restrictive inter-atrial communication
Coarctation in 80%
Hypoplastic LV
2. Clinical Features/Diagnosis
Newborn with cyanosis, tachypnea
Most common cause cardiac death < 1 month
Echocardiography makes diagnosis
Catheterization rarely needed
3. Medical Support
PGE-1 for ductal patency
Maintain hematocrit at 45%
Balance pulmonary blood flow
O2 saturation 70-75% (FIO2 0.18 - 0.21)
Maintain PCO2 40 - 50
NO atrial septostomy
4. Staged Surgical Therapy
Norwood procedure as neonate
Atrial septectomy
Controlled shunt
Aortic reconstruction
Bi-directional Glenn
4-8 months
Fontan procedure
12-24 months
Image Norwood 1
Image Norwood 2
Image Norwood 3
5. Results of Staged Surgical Therapy
10-30% mortality stage I Norwood
5% mortality Glenn
5% mortality Fontan
Actuarial survival 50-60% at 1 year
Functional results variable
6. Transplantation
Bi-ventricular repair - 1 operation
20-30% waiting list mortality
Lifelong immunosuppression
75% 2 year actuarial survival
Procedure 1
Procedure 2
Procedure 3
Procedure 4
7. Summary
Uniformly lethal untreated
Medical stabilization for all
Staged reconstruction
High yearly mortality
Applicable in nearly all
Approach of choice in many centers
Transplantation
Low yearly mortality
Donor limited
