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ORIGINAL ARTICLE

New surgical techniques for treatment of pulmonary atresia with ventricular septal defect and pulmonary arteries anomalies including the so-called tipo IV truncus

Miguel Barbero-Marcial; Edmar Atik; Miguel Ratti; Luiz Junya Kajita; Arlindo Riso; Geraldo Verginelli; Delmont Bittencourt; Fúlvio Pileggi; Adib D Jatene

DOI: 10.1590/S0102-76381987000100003

ABSTRACT

Fourty-two patients with pulmonary atresia and interventricular septal defect were submited to a partial or total correction, between January 1975 and October 1986, with a range of 2 months to 18 years of age. Three groups were identified: Group A: 34 patients with all bronco-pulmonary segments connected to pulmonary arteries (PA); Group B: 6 patients with some of the bronco-pulmonary segments connected to PA's; Group C: 2 patients with all bronco-pulmonary segments connected to systemic pulmonary collaterals. One to three stages were utilized to perform the proper correction. In Group A 17 were treated in one stage, with 3 deaths; 9 were submitted to a first stage where the pulmonary arteries were reconstructed with an additional Blalock-Taussig (BT) shunt, with 1 death. In two of them a second stage for total correction was performed, with no death. In the remaining 8 patients the collateral circulation was ligated and a BT shunt performed, with 1 hospital death. In Group B the first stage joining the intra or extra-pulmonary collaterals was obtained in 6 cases, without death. In two of them a total correction was performed, with 1 death. In group C a three stage operation was done in 1 patient. In the first stage an intermediate arterial segment was constructed between the lobar arteries and the BT shunt. In the second stage the contra-lateral collaterals were joined. The third stage was the establishment of the continuity between the right ventricle and the reconstructed pulmonary circulation with a good result. In the other patient of this group, until now, only the first stage was done. Serial hemodynamic studies were carried out in 32 patients. These proposed techniques demonstrate the possibility of total correction with appropriate righ-to-left ventricular pressure relation.

RESUMO

Entre janeiro de 1975 e outubro de 1986, 42 pacientes com atresia pulmonar e comunicação interventricular, com idade entre 2 e 18 anos, foram submetidos a correção parcial, ou total. Foram divididos em: tipo A) com todos os segmentos broncopulmonares conectados às artérias pulmonares (AP's), 34 pacientes; tipo B) com alguns dos segmentos broncopulmonares conectados às AP's, 6 pacientes; tipo C) com todos os segmentos broncopulmonares conectados às colaterais sistêmico-pulmonares, 2 pacientes. A correção foi planejada em uma a três etapas. No tipo A, 17 foram corrigidos em uma etapa, com três óbitos; em 9, na primeira etapa, as AP's foram reconstruídas e o Blalock (BT), realizado, tendo ocorrido um óbito. Em 2, a segunda etapa de correção total foi realizada, sem óbitos. No tipo B, a primeira etapa de unificação das colaterais intra ou extra-hilares foi realizada em 6 casos, sem óbitos; em 2, a segunda etapa da correção total foi realizada, com um óbito. No tipo C, 2 pacientes foram operados; 1 em três etapas; a primeira constou de construção de segmento arterial intermediário entre as artérias lobares e o BT; a segunda compreendeu unificação das colaterais contralaterais e a terceira, restabelecimento da continuidade ventrículo direito - circulação pulmonar; o paciente teve boa evolução. No segundo caso, a correção foi realizada após somente uma intervenção prévia. A evolução foi satisfatória. Estudos hemodinâmicos seriados foram realizados em 32 pacientes. As técnicas propostas permitem obter condições para correção total com adequada relação pós-operatória das pressões ventrículo direito - ventrículo esquerdo.
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