1. TOF with Absent Pulmonary Valve
Seoul National University Hospital
Department of Thoracic & Cardiovascular Surgery

2. TOF with Absent Pulmonary Valve
1. Definition
A subset of TOF determined largely by vestigial,
severely hypoplasic, nonfunctioning pulmonary
leaflets at the junction of RV & pulmonary trunk
2. History
1) Royer & Wilson : 1st description in 1908
2) Kurtz : 2nd report in 1927

3. TOF with Absent Pulmonary Valve
Morphology
1. Pulmonary valve
Myxomatous nubbins of valve tissue, severely hypoplastic,
Both nonfunctioning & only minimally stenotic
2. RVOT
Often dilated and elongated
3. Pulmonary trunk
Central portion of RPA & LPA are often aneurysmally dilated
Dilation into hilar portion, then tracheobronchial compression
Beyond hilar portion, pulmonary arteries are normal in size

4. TOF with Absent Pulmonary ValvePA
Pulmonary Valve
Pulmonary annulus

5. Clinical Features & Diagnosis
1) Severe pulmonary regurgitation and somewhat increased
pulmonary blood flow
2) Low pulmonary artery pressure & similar peak pressure in both
ventricles due to narrowing annulus & large VSD
3) Presentation is dependent on the severity of pulmonary arterial
dilatation, Qp increased and tracheobronchial compression
2. Diagnostic criteria
1) Physical examination
Overactive heart, cardiomegaly, raised venous pressure
2) Chest radiography
Supracardiac mediastinal widening , atelectasis or overinflation
3) Other studies include ECG, echocardiography, aortography

6. Natural History 1. Incidence 5% of TOF born with a large VSD + PS
2. 50% die in the 1st year of life if untreated, and most
in the few months of life, from the respiratory distress
Such patients also have heart failure with large shunt
with decreased systolic function.
3. Patients who survive infancy, generally do well for
time being, and ultimately die from intractable right
heart failure.

7. Operative Treatment 1. Indications 2. Techniques
1) Urgently needed for small babies who present with
severe respiratory distress
2) If infants responds well this therapy (prone, head-up),
operation is deferred selectively to 3 -5 years electively.
2. Techniques
1) VSD closure and insertion of homograft beyond infancy
2) Reduction pulmonary angioplasty with corrective repair
is preferred in neonates and infants
3) Pulmonary arterioplasty to takes pressure off underlying
tracheobronchial tree.

8. Operative Procedure
Ventriculotomy and resection of dilated portions of main &
branch pulmonary arteries.
Reconstruction of the right and left pulmonary arteries &
insertion of a homograft

9. Operative Procedure
Placement of a homograft with a tube graft extension
from the diaphragmatic surface of the right ventricle

10. TOF with Absent Pulmonary Valve
General management principles
1. Preoperative
1) Sternotomy
2) Prone position
3) Adequate management
2. Postoperative
1) Prone position in head-up
2) Avoid barotrauma
3) Avoid hyperinflation by air trapping
4) Short inspiratory phase (1:E>1:4)

11. Operative Results 1. Survival ; a high probability of hospital death
after repair in young infants, currently
emphasizing pulmonary arterioplasty
Early death
Poor preoperative conditions
Severe respiratory problems
Time-related survival
Similar to those with TOF
2. Incremental risk factors for death
Allograft valve conduit