1. One & A Half Ventricle Repair
Seoul National University Hospital
Department of Thoracic & Cardiovascular Surgery

2. One & A Half Ventricle Repair
Rationale
In an attempt to avoid the high early mortality and extending the limits of biventricular repair for patients with borderline pulmonary ventricle, or late complications after Fontan operation while still achieving separate pulmonary and systemic circulation

3. Introduction
Long-term outcomes of the Fontan procedure have been less ideal.
Late problems of ventricular hypertrophy, dilation, and eventual failure are time-dependant and appear to be inevitable.
Avoidance of the Fontan physiology is preferred.
At the same time, biventricular repair in patients with borderline(inadequate) pulmonary ventricles can result in poorer early and late survival.
One and a half ventricle repair would be logical if it improves patient survival and functional status.

4. One & A Half Ventricle Repair
Term
A superior vena cava to pulmonary artery anastomosis
can be created as an adjunct to biventricular repair.
Aim
By diverting the superior vena caval flow directly to the lungs, pulmonary ventricular volume load is reduced by approximately one third.

5. Inappropriate Pulmonary Ventricle
1. Small tricuspid annulus
. Less than 75% of the diameter of IVC
. Tricuspid z-value less than –2
. Pulmonary ventricular volume less than 70~ 80%
2. Normal or dilated tricuspid valve annulus
. Hypertrophic RV exposed to long-lasting
hypertension & cyanosis
. Dilated RV & poorly contracting due to chronic PR
3. A pulmonary ventricle, guarded by a AV valve,
even if hypoplastic or diminutive

6. Anatomic Selection Criteria for Small Pulmonary Ventricle
Tricuspid Pulmonary Operation
z-Value Ventricular
Volume
> > 80% Biventricular repair
< < 80% ½ repair & CPA
< < 50% ½ repair & CPA
Atrial fenestration
< -10 ? < 30% Fontan operation

7. Principles of Partial Biventricular Repair
1. To create a modified in-series circulation with
no left-to-right, or right-to-left shunting
2. Systemic ventricle pumping a single cardiac
output
3. Pulmonary circulation receiving full cardiac
output through the pulmonary ventricle &
superior cavo-pulmonary connection

8. Advantages of Partial Biventricular Repair
1. The Fontan operation remains a suboptimal form
of definite palliation.
2. Maintenance of equal and separate pulmonary &
systemic circulation
3. Incorporating the pulmonary ventricle into the
circulation with a reduced volume load
4. Exercise tolerance, rhythm status comparing the
Fontan procedure, & effect of cerebral function of
pulsatile & mildly elevated pressure in SVC

9. Indications for Partial Biventricular Repair
1. Small physiologic right ventricle
2. Acute & chronic right ventricular dysfunction
3. Facilitation of biventricular repair without
hypoplasia or functional impairment of the
pulmonary ventricle
4. Simplifying the operation requiring a complex
intraatrial baffle for atrial isomerism, single atrium
& bilateral SVC

10. Decisions for Biventricular or One & a Half Ventricular Repair
1. Influencing factors for biventricular repair
1) Size of ventricle
2) Morphology of ventricle
3) Function of ventricle
4) Demensions and function of the inflow and outflow
2. Indications for one and a half ventricular repair
1) Small right ventricle
2) Chronic right ventricular dysfunction
3) Acute right ventricular dysfunction
4) Facilitation of repair without hypoplasia or functional
impairment of pulmonary ventricle

11. Decision Making for Pulmonary Ventricles
Assessing pulmonary ventricular morphology
Missing or defective component of right ventricle
Measurement of ventricular volume (Simpson method)
The tricuspid annulus
Functional assessment of pulmonary ventricle
Functional analysis of right ventricle by Echo, Angio, MRI
Ventricular wall thickness & diastolic filling
Dilated ventricle
Pulmonary afterload
Elevated PVR is not indicated.

12. Simplified Physiologic Indications (Mavroudis, 1999)
Volume unloading
Reduced ventricular contraction
Correction of cyanosis (LSVC to LA)
Pressure considerations

13. One & a Half Ventricle Repair
Atrial isomerism complex
AVSD with or without TOF
Atrioventricular & ventriculoarterial discordance
Double-inlet left ventricle
Double-outlet right ventricle
D-transposition of great arteries
Ebstein’s anomalies
Pulmonary atresia with intact ventricular septum
Pulmonary stenosis
Tetralogy of Fallot
VSD with or without straddling atrioventricular valves

14. One & a Half Ventricle Repair
Arterial Switch Operation
Arterial Switch Operation , VSD closure and BCPC
in patient with TGA, VSD , small RV cavity

15. One & a Half Ventricle Repair
BCPC
small RV
Arterial Switch Operation , VSD closure and BCPC in patient with TGA, VSD , small RV cavity

16. Intermediate Outcomes
Rare complications of Fontan operation
Atrial arrhythmia
Ventricular failure
Pulmonary arteriovenous fistula
Protein losing enteropathy
Presence of additional pulmonary blood flow
Prolonged pleural effusion or chylothorax
Limited morning periorbital edema
Pseudoaneurysm of SVC
Pulmonary regurgitation & excessive pulsatility