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Anatomical classification and surgical repair of the pulmonary atresia with ventricular septal defect

OBJECTIVE: To analyze the morphological aspects, the surgical results obtained according to the number of procedures, and the mortality in each group of Barbero-Marcial´s classification of the pulmonary atresia with ventricular septal defect. MATERIAL E METHODS: From January 1990 to November 1999, 73 patients submitted to cardiac catheterization and detailed pulmonary angiographic study before the first surgical intervention were analyzed. The anatomical characteristics of the pulmonary arteries and major aorticopulmonary collaterals, as the surgical techniques of definitive, palliative and "definitive palliative" were studied. The causes of mortality were also described. RESULTS: Nineteen patients had all the pulmonary segments supplied by pulmonary arteries (group A), 45 patients had pulmonary segments supplied by pulmonary arteries and major aorticopulmonary collaterals (group B) and 9 had only major aorticopulmonary collaterals (group C). The group A has the major incidence of definitive surgical repair, the group B more palliative procedures and the group C more "definitive palliative" procedures (p< 0.0001). There is no statistically significant difference of mortality among the groups (p=0.860), although the mortality of group B and C was more than twice that of group A. CONCLUSIONS: Barbero-Marcial´s classification allows to estimate the probability of definitive, palliative or "definitive palliative" treatment, as well as the risk of mortality in patients with pulmonary atresia with ventricular septal defect.

Heart defects; Heart defects; Pulmonary atresia; Pulmonary atresia; Pulmonary atresia


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