Absent pulmonary valve syndrome is a rare congenital heart defect where the pulmonary valve is either completely absent or has rudimentary tissue. This causes the pulmonary arteries to dilate massively and compress the trachea during fetal development. After birth, affected infants often experience respiratory distress or complications like pneumonia. Surgical repair is needed to close the ventricular septal defect and restore competence to the pulmonary valve to address both the cardiac issues and pulmonary complications.

1. ABSENT PULMONARY VALVE
SYNDROME
Dr.Sherif Sabet
PSCC

2. Absent Pulmonary Valve Syndrome is Fascinating
and unique variety of structural heart disease!
Moss and Adams’

3. PATHOLOGY
 Tetralogy of Fallot with an absent pulmonary valve
occurs in approximately 2% of patients with TOF.
 The pulmonary valve leaflets are either completely
absent or have an uneven rim of rudimentary valve tissue
present
 Rudimentary pulmonary valve is typically both stenotic
and regurgitant with aneurysmally dilated pulmonary
arteries, and a large malaligned outlet ventricular septal
defect (VSD)

4. The massive pulmonary artery aneurysm
develops during fetal life.
It compresses the developing trachea and
bronchi throughout fetal life.

5. Airway obstruction and respiratory distress during
infancy.
Pulmonary complications (e.g.,atelectasis,
pneumonia), rather than the intracardiac defect, are the
usual causes of death.

6. Clinical Manifestations
Mild cyanosis
Signs of CHF may develop after the
newborn period
Respiratory symptoms

7. To-and-fro murmur (with “sawing-wood”
sound) at the upper and mid-left sternal
Borders
The S2 is loud and single

8. The ECG shows RAD and RVH
Chest radiography images reveal a
noticeably dilated main PA and hilar Pas
Echocardiography
CT or MRI scan

9. MANAGEMENT
 After the pulmonary symptoms appear,
neither surgical nor medical management
has good results.
 Symptomatic neonates should have
corrective surgery on an urgent basis. Even
asymptomatic children should have elective
surgery in the first 3 to 6 month of life.

10. Complete primary repair is the procedure of
choice. VSD is closed through right
ventriculotomy
(across the pulmonary annulus).
Alternatively, a valved conduit may be used to
restore competence of the pulmonary valve,
and the aneurysmal PAs are plicated

12. REAL CTA CASES
Absent Pulmonary Valve Syndrome

13. EPARTERIAL HYPARTERIAL

15. CASE #1
13 MONTH-OLD BOY
S/P TOF REPAIR

17. Huge RPA
Huge MPA
Severe PS
Aneurysmal RVOT

19. Posterior

20. CASE #2
8 MONTH-OLD GIRL

24. Case 3
35 Days girl

30. Home Message:
Absent Pulmonary Valve Syndrome
Pulmonary valve is both stenotic and regurgitant.
The massively dilated pulmonary arteries compresses
the trachea from the fetal life
Should be corrected soon after diagnosis.

31. Thank You
Dr.Sherif
Sabet