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Perforation of the atretic pulmonary valve. Long-term follow-up
Gabriella Agnoletti1, Jean François Piechaud, Philipp Bonhoeffer
1Service de Cardiologie Pédiatrique, Necker Enfants Malades, Paris, France. gabriella.agnoletti@nck.ap-hop-paris.fr
Insights
Pulmonary valve perforation is effective for selected patients with pulmonary atresia and intact ventricular septum (PA-IVS), enabling biventricular correction in most cases. Careful patient selection is key to successful outcomes and reduced need for further surgery.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Interventional Cardiology
Background:
- Pulmonary valve perforation is a primary treatment for pulmonary atresia with intact ventricular septum (PA-IVS).
- Long-term outcomes of this intervention, especially in patients with a tripartite right ventricle (RV) and normal coronary circulation, are not well-documented.
Purpose of the Study:
- To evaluate the long-term efficacy and safety of interventional pulmonary valve perforation in newborns with PA-IVS.
- To identify factors influencing outcomes and the need for subsequent surgical interventions.
Main Methods:
- Retrospective analysis of 39 newborns with PA-IVS undergoing attempted pulmonary valve perforation between 1991 and 2001.
- Assessment of early procedural outcomes, complications, mortality, and long-term survival and need for surgery.
Main Results:
- Successful perforation in 33 patients, with 17 requiring neonatal surgery and 13 avoiding surgery. Two procedure-related deaths and four postsurgical deaths occurred.
- Patients not needing immediate surgery had a higher incidence of tripartite RV and better tricuspid valve z-values.
- At 5.5 years median follow-up, survival was 85%, with 35% freedom from surgery. Five patients underwent partial cavopulmonary connection.
Conclusions:
- Pulmonary valve perforation is an effective treatment for selected PA-IVS patients with a normal-sized RV.
- Patient preselection is crucial for achieving successful interventional or surgical biventricular correction in the majority of cases.
- The procedure has significant associated mortality and morbidity, necessitating careful consideration.
Objectives:
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.