Perforation of the atretic pulmonary valve. Long-term follow-up

J Am Coll Cardiol. 2003 Apr 16;41(8):1399-403. doi: 10.1016/s0735-1097(03)00167-0.


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.

MeSH terms

  • Cardiac Catheterization* / adverse effects
  • Cardiac Surgical Procedures* / adverse effects
  • Elective Surgical Procedures
  • Follow-Up Studies
  • Heart Septum
  • Heart Ventricles
  • Humans
  • Infant, Newborn
  • Predictive Value of Tests
  • Pulmonary Atresia / mortality
  • Pulmonary Atresia / physiopathology
  • Pulmonary Atresia / surgery*
  • Pulmonary Valve / physiopathology
  • Time Factors
  • Treatment Outcome
  • Tricuspid Valve / physiopathology
  • Vascular Surgical Procedures* / adverse effects