Perforation of the atretic pulmonary valve. Long-term follow-up.
OBJECTIVE
We evaluated the long-term results of perforation of the pulmonary valve in patients with pulmonary atresia with an intact ventricular septum (PA-IVS).


BACKGROUND
Interventional perforation of the pulmonary valve is considered the elective first stage treatment for PA-IVS, particularly in patients with a tripartite right ventricle (RV) and normal coronary circulation. However, the long-term results of this procedure are lacking.


METHODS
Between January 1991 and December 2001, 39 newborns with a favorable form of PA-IVS underwent attempted perforation of the pulmonary valve. We evaluated the early and long-term outcomes.


RESULTS
Median tricuspid and pulmonary z values were -1.2 and -2.4, respectively. Perforation was successful in 33 patients. Among them, 17 needed neonatal surgery, 13 did not need any surgery, and 3 had elective surgery after the first month of life. There were two procedure-related deaths, seven nonfatal procedural complications, and four postsurgical deaths. Compared with patients needing neonatal surgery, those having no or elective surgery had a higher incidence of a tripartite RV and a higher median tricuspid z value (92% vs. 53%, p = 0.04 and -1.7 vs. -0.5, p = 0.03). At a median follow-up of 5.5 years (range 0.5 to 11.5), survival was 85% and freedom from surgery was 35%. Five patients, four of whom had neonatal surgery, underwent a partial cavo-pulmonary connection.


CONCLUSIONS
Our results show that this technique, although burdened by non-negligible mortality and morbidity, is effective in selected patients with a normal-sized RV. Preselection of patients allows interventional or surgical biventricular correction in the majority of cases.
Agnoletti
Gabriella
G
Service de Cardiologie Pédiatrique, Necker Enfants Malades, Paris, France. gabriella.agnoletti@nck.ap-hop-paris.fr
Piechaud
Jean François
JF
Bonhoeffer
Philipp
P
Aggoun
Yacine
Y
Abdel-Massih
Tony
T
Boudjemline
Younes
Y
Le Bihan
Christine
C
Bonnet
Damien
D
Sidi
Daniel
D
D006328:Cardiac Catheterization / Q000009:adverse effects; D006348:Cardiac Surgical Procedures / Q000009:adverse effects; D017558:Elective Surgical Procedures; D005500:Follow-Up Studies; D006346:Heart Septum; D006352:Heart Ventricles; D006801:Humans; D007231:Infant, Newborn; D011237:Predictive Value of Tests; D018633:Pulmonary Atresia / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D011664:Pulmonary Valve / Q000503:physiopathology; D013997:Time Factors; D016896:Treatment Outcome; D014261:Tricuspid Valve / Q000503:physiopathology; D014656:Vascular Surgical Procedures / Q000009:adverse effects