CERCLAGE DE L'ARTERE PULMONAIRE DANS LE TRAITEMENT DES COEURS UNIVENTRICULAIRES. RESULTATS ET IMPLICATIONS THERAPEUTIQUES POUR LES DERIVATIONS CAVOPULMONAIRES

Eighty-six children under one year of age with univentricular hearts associated with increased pulmonary flow underwent pulmonary artery banding as the first stage of palliative therapy. Fifteen patients also had treatment of coarctation of the aorta at the same time and an atrial septal defect was treated in 13 patients. Twenty-seven patients underwent a Fontan procedure secondarily. The mean follow-up was 42.6 ± 45.5 months; 8 patients were lost to follow-up. Twenty-three patients died during the whole of the study period. The global actuarial survival rate was 69.6 ± 5% at 5 years. The 3 year survival rale was 56 ± 12% in patients with anatomical right ventricles compared with 74.4 ± 5.7% and 69.5 ± 7.3% at 3 and 5 years respectively in those with anatomical left ventricles (p < 0.01). The presence of coarctation of the aorta reduced the 5 year actuarial survival rate to 25.4 ± 12.8% (p < 0.01). Subaortic stenosis either at the time of initial presentation or occurring during pulmonary banding was associated with a 5 year survival of 58.3 ± 13.7% (p < 0.01). Uni- and multivariate analysis demonstrated poor prognostic risk factors. On univariate analysis, they were the residual mean pulmonary pressures after banding, coarctation of the aorta, subaortic stenosis and a restrictive atrial septal defect. Independent risk factors on multivariate analysis were the residual pulmonary pressures after banding, coarctation of the aorta, the necessity of operation in the neonatal period and the need for reoperation for reason other than for a cavopulmonary connection. The feasability of a Fontan procedure was reduced when subaortic stenosis was observed at any time. Only 17.5% of patients were free of reoperation during this study for whom banding remained the only palliative procedure. In conclusion, a programme of cavopulmonary connection should be envisaged from the initial presentation of these patients. The different stages of treatment with this objective in mind should aim to preserve myocardial function and keep pulmonary resistances low. Therefore, pulmonary artery banding should be rejected in cases with coarctation of the aorta and/or subaortic stenosis. Similarly, early conversion with a Glenn type bidirectional anastomosis allows adaptation of myocardial function for a secondary total cavopulmonary connection.