The Fontan surgery is a palliative surgery for univentricular congenital heart disease. On the longterm, heart failure, pulmonary arterial hypertension (PAH) and exudative enteropathy may lead tothe Fontan failure.A clinical adjustment has been realized by focusing secondarily on adult patients. The advantage, insome cases of total anomalous pulmonary venous return, of the bidirectional cavopulmonary (BCP),which is an intermediate stage of Fontan, has been described.In order to prepare future clinical studies, we have discussed about Brain Natriuretic Peptide inpostoperative Fontan surgery. We have demonstrated the reliability of a new technology of pulseoxymetry in cyanotic children and the unreliability of a new device of impedance for non-invasivemonitoring of cardiac outpout in comparison with pulmonary arterial catheter.A viable chronic animal model of bidirectional cavo-pulmonary (BCP) has been developed. Itshowed that the antegrade pulsatile flow prevents the formation of pulmonary arteriovenous fistulas(PAVMs) ,improves hematosis and attenuates the development of PAH clinically supporting themaintenance of this flow when creating a BCP.Moreover, the loss of pulsatility while reducing the shear stress causes a decrease in e-NOSsynthase and a dependent vaso-relaxing altered response of the pulmonary arterial endothelium. Themicropulsatility limits these deleterious effects. These results imply potential therapies against thePAH of Fontan surgery, maintaining an accessory pulmonary blood flow and the pharmaceuticalvaso-relaxing non-endothelium dependent modulation.
