ABSENT PULMONARY VALVE

SYNDROME

Dr.Sherif Sabet

PSCC

Absent Pulmonary Valve Syndrome is Fascinating

and unique variety of structural heart disease!

Moss and Adams’

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)

The massive pulmonary artery aneurysm

develops during fetal life.

It compresses the developing trachea and

bronchi throughout fetal life.

Airway obstruction and respiratory distress during

infancy.

Pulmonary complications (e.g.,atelectasis,

pneumonia), rather than the intracardiac defect, are the

usual causes of death.

Clinical Manifestations

Mild cyanosis

Signs of CHF may develop after the

newborn period

Respiratory symptoms

To-and-fro murmur (with “sawing-wood”

sound) at the upper and mid-left sternal

Borders

The S2 is loud and single

The ECG shows RAD and RVH

Chest radiography images reveal a

noticeably dilated main PA and hilar Pas

Echocardiography

CT or MRI scan

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.

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

REAL CTA CASES

Absent Pulmonary Valve Syndrome

EPARTERIAL HYPARTERIAL

CASE #1

13 MONTH-OLD BOY

S/P TOF REPAIR

Huge RPA

Huge MPA

Severe PS

Aneurysmal RVOT

Posterior

CASE #2

8 MONTH-OLD GIRL

Case 3

35 Days girl

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.

Thank You

Dr.Sherif Sabet