Anomalous coronary arteries arising from the pulmonary trunk is a rare but potentially fatal condition. We report the clinical presentation, surgical treatment and long-term follow-up of seven surgical cases of anomalous left coronary and one case of anomalous right coronary artery arising from the pulmonary artery. Age ranged from 7 months to 13 years (average: 5.09+/-3.7 years) and weight ranged from 7 to 50 kg (average: 19.9+/-8.8 kg). Follow-up was 100% complete: average 78 months (S.D.: 52.7 months). Direct reimplantation was the surgical technique in six cases, Takeuchi procedure in one case and subclavian artery interposition in one case. Concomitant mitral valve repair was undertaken in two cases. In two children the coronary artery anomaly was diagnosed and treated only after a first surgery for other congenital heart anomaly. Left ventricle ejection fraction was restored in those cases of pre-operative dysfunction. Mortality was not observed and all children are asymtomatic and free of reoperation.

The two-patch technique, associated with ventriculotomy and a transjunctional pulmonary patch is safe and efficient when correcting atrioventricular septal defect associated with tetralogy of Fallot, resulting in good mid-to-long term clinical outcomes.

Purpose -To evaluate if the presence of right bundle branch block (RBBB), without structural myocardial abnormalities (SMA) can generate fragmented potentials (FP) and spectral turbulence on signal-averaged electrocardiogram (SAECG).Methods -Twelve children with atrial septal defect (ASD) and incomplete right bundle branch block (IRBBB without SMA (group I) Objetivo -Em ausência de alterações estruturais miocárdicas (AEM), avaliar se o bloqueio de ramo direito (BRD) gera potenciais fragmentados (PF) e turbulência espectral (TE) no eletrocardiograma de alta resolução (ECGAR). Métodos -Doze crianças com comunicação interatrial (CIA) e bloqueio incompleto do ramo direito (BIRD) sem AEM (Grupo I), foram comparadas com 17 crianças com tetralogia de Fallot (TF) operada, BCRD e AEM, 5 com extra sístoles ventriculares e 2 com taquicardia ventricular sustentada (Grupo II). Todas fizeram ECGAR nos domínios do tempo (DT) e da freqüência (DF), com cinco variáveis analisadas. Resultados -Os pacientes do grupo I tiveram as variáveis normais apesar do BIRD. No grupo II, 4 das cinco variáveis foram anormais, sugerindo a presença de PF e TE atribuíveis a AEM inerentes à malformação e ao ato cirúrgico.Conclusão -Na CIA o BIRD não complicado de AEM não gera PF e TE, não constituindo fator de risco para taquicardia ventricular sustentada. Palavras

We report our results on surgical treatment of complete atrioventricular septal defects using the two-patch technique. Forty patients with complete atrioventricular septal defects were operated on in the period from November 1995 to January 2004 and retrospectively analyzed. The age at the time of surgery ranged from 4 months to 20 years (average=18.8+/-37 months). Their weights ranged from 3 to 39 kg (average=7.6+/-5.8 kg). Associated tetralogy of Fallot was present in 20% of the cases (8 patients). Monitoring was complete until January 2007, corresponding to a follow-up ranging from 36 to 135 months (average=74+/-33.7 months). The surgical mortality rate was 2.5% and the hospital mortality rate was 5%. A third patient died from a brain abscess two years after surgery. Over the long-term, two patients needed further operations: one was submitted to mitral plasty due to severe residual mitral insufficiency, one year later; the other underwent a resection of a sub-aortic membrane after three years. Differences were evaluated using the Student-t or Mann-Whitney tests. Surgical treatment of complete atrioventricular septal defect using the two-patch technique results in low morbidity and mortality in early-to-mid term follow-up.