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CLINICAL STUDY: CONGENITAL HEART DISEASE

Perforation of the atretic pulmonary valve

long-term follow-up

Gabriella Agnoletti, MD, PhD^*,*, Jean François Piechaud, MD, Philipp Bonhoeffer, MD^*, Yacine Aggoun, MD^*, Tony Abdel-Massih, MD^*, Younes Boudjemline, MD^*, Christine Le Bihan, MD^*, Damien Bonnet, MD, PhD^* and Daniel Sidi, MD, PhD^*

^* Service de Cardiologie Pédiatrique, Necker Enfants Malades, Paris, France
Institut Hospitalier Jacques Cartier, Paris, France

Manuscript received July 10, 2002; revised manuscript received November 27, 2002, accepted January 16, 2003.

^* Reprint requests and correspondence: Dr. Gabriella Agnoletti, Service de Cardiologie Pédiatrique, Groupe Hospitalier Necker Enfants Malades, 149 rue de Sèvres, 75743 Paris, France.
gabriella.agnoletti{at}nck.ap-hop-paris.fr

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.

Abbreviations and Acronyms   ASD   atrial septal defect   BT   Blalock-Taussig   NYHA   New York Heart Association   PA-IVS   pulmonary atresia with intact ventricular septum   PCPC   partial cavo-pulmonary connection   PG   prostaglandin   RV   right ventricle/ventricular   RVOT   right ventricular outflow tract

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