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Hypoplastic Left Ventricle

Hypoplastic Left Ventricle

1. Anatomy

A. Hypoplastic LV and ascending aorta
B. Aortic atresia/stenosis
C. Mitral atresia/stenosis
D. Restrictive inter-atrial communication
E. Coarctation in 80%

2. Clinical Features/Diagnosis

A. Newborn with cyanosis, tachypnea
B. Most common cause cardiac death < 1 month
C. Echocardiography makes diagnosis
D. Catheterization rarely needed

3. Medical Support

A. PGE-1 for ductal patency
B. Maintain hematocrit at 45%
C. Balance pulmonary blood flow
D. O2 saturation 70-75% (FIO2 0.18 - 0.21)
E. Maintain PCO2 40 - 50
F. NO atrial septostomy

4. Staged Surgical Therapy

A. Norwood procedure as neonate
1) Atrial septectomy
2) Controlled shunt
3) Aortic reconstruction
B. Bi-directional Glenn
1) 4-8 months
C. Fontan procedure
1) 12-24 months

5. Results of Staged Surgical Therapy

A. 10-30% mortality stage I Norwood
B. 5% mortality Glenn
C. 5% mortality Fontan
D. Actuarial survival 50-60% at 1 year
E. Functional results variable

6. Transplantation

A. Bi-ventricular repair - 1 operation
B. 20-30% waiting list mortality
C. Lifelong immunosuppression
D. 75% 2 year actuarial survival

7. Summary

A. Uniformly lethal untreated
B. Medical stabilization for all
C. Staged reconstruction
1) High yearly mortality
2) Applicable in nearly all
3) Approach of choice in many centers
D. Transplantation
E. Low yearly mortality
F. Donor limited