[Tetralogy of fallot with absent pulmonary valve].
BACKGROUND
Absent pulmonary valve with ventricular septal defect is a rare cardiac malformation. The aim of our study is to specify the anatomic characteristics and the clinical and echocardiographic features of this cardiac malformation and to discuss its management.


METHODS
We report 8 cases of absent pulmonary valve with ventricular septal defected collected over a period of 24 years. The diagnosis was made during the first year of life in all cases in the presence of respiratory symptoms and/or cyanosis. It was confirmed by cardiac catheterization in 4 cases and echocardiography in 4 cases.


RESULTS
Pulmonary vascular obstructive disease, related to aortopulmonary collateral vessels, was noted in one patient who died at the age of 20 years. One patient was lost to follow up and 6 patients were operated. Closure of the ventricular septal defect, widening of the pulmonary tract and insertion of a pulmonary valve were performed in the 6 cases. Reduction procedure of the pulmonary arteries was performed in 3 cases. One death related to early postoperative infective endocarditis was noted. At a mean follow up of 2 years, the 5 survivors are going well with no significant pulmonary stenosis.


CONCLUSIONS
Although named "tetralogy of Fallot with absent pulmonary valve", absent pulmonary valve with ventricular septal defect is different from tetralogy of Fallot by aneurysmal dilatation of the pulmonary arteries which may compress the bronchial tree and lead to respiratory symptoms that can be severe with respiratory distress. Pulmonary arterioplasty eliminate airways obstruction and its results are satisfactory. The need for insertion of a pulmonary valve is debatable.
Service de Cardiologie Pédiatrique, Hôpital la Rabta, Tunis.
Lilia
L
Chaker
Ali
A
Cherif
Nabil
N
Triki
Nabil
N
Hermi
Syrine
S
Abid
Fatma
F
Ouarda
Hela
H
Msaad
Rachid
R
Mechmeche
Abdelfatteh
A
Abid
Fekria
F
Abid
D005260:Female; D006350:Heart Valve Prosthesis; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011664:Pulmonary Valve / Q000002:abnormalities* / Q000601:surgery; D012189:Retrospective Studies; D013771:Tetralogy of Fallot / Q000175:diagnosis* / Q000601:surgery