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J Thorac Cardiovasc Surg 2003;125:1083-1090
© 2003 The American Association for Thoracic Surgery

Surgery for Congenital Heart Disease

Nitric oxide synthase expression by pulmonary arteries: A predictive marker of Fontan procedure outcome?

From the Service de Chirurgie Cardiaque,^a Hôpital Necker-Enfants Malades, Service d'Anatomo-Pathologie,^b and Hôpital Européen Georges Pompidou and Laboratoire d'Immunologie Pulmonaire,^c UFR Biomédicale des Saints Pères, Faculté Paris V, Paris, France.

This work was supported by APPCC (Paris, France) and SESERAC (Paris, France).

Received for publication April 17, 2002. Revisions requested May 30, 2002; revisions received July 25, 2002. Accepted for publication Aug 15, 2002. Address for reprints: Marilyne Lévy, MD, Hôpital Necker-Enfants Malades, 149 rue de Sevres, 75015 Paris, France (E-mail: marilyne.levy{at}nck.ap-hop-paris.fr).

Objectives: We retrospectively analyzed lung biopsy specimens from patients who underwent the Fontan procedure to identify predictive markers of outcome.
Methods: We studied the intra-acinar pulmonary arteries present in lung biopsy specimens from 17 patients undergoing the Fontan procedure. We evaluated both their morphology and their expression of endothelial nitric oxide synthase and endothelin 1. We compared these data with those of 6 patients who died of no pulmonary cause (control group).
Results: Eight patients had a good surgical outcome (group 1). Their distal arteries were thin and weakly expressed endothelin 1 and endothelial nitric oxide synthase. The procedure failed in 9 patients (group 2). Their distal arteries displayed muscle extension with an increased wall thickness (P < .01 vs group 1). Their endothelin 1 expression remained low (not significant vs group 1). By contrast, endothelial nitric oxide synthase was markedly overexpressed (P < .001 vs group 1).
Conclusion: Distal pulmonary arteries of patients in whom the Fontan procedure failed exhibited a markedly increased wall thickness and a clear endothelial nitric oxide synthase overexpression. In addition to giving clues to the pathogenesis of the procedure's failure, our study might help to define reliable predictive markers of its outcome.

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