[Complete atrioventricular septal defect. Anatomo-functional correlation between patients with and without Down's syndrome]. / Defeito total do septo atrioventricular. Correlação anatomofuncional entre pacientes com e sem síndrome de Down.

PURPOSE: To compare clinical course, causes and symptoms beginning enset time in children with complete atrioventricular canal with and without Down's syndrome. METHODS: Records of 80 patients < 2 years of age, were reviewed. There were 55 (69%) with Down's syndrome-group I (GI) and 25 (31%) without-group II (GII). Age at synpton enset intensity, functional class, clinical repercussion and anatomic variations in patients undergoing corrective surgery were evaluated. RESULTS: Mean age at symptoms onset was similar for the two groups (50 +/- 75 days). Class II (NYHA) was more frequent in GI (31 patients-56.5%) and class III-IV (NYHA) in GII (19 patients-76%) p < 0.005. Clinical repercussion evaluation showed that congestive heart failure was present in 34 (62%) patients of GI and 21 (84%) of GII; and, pulmonary hypertension was in 21 (38%) patients of GI and 4 (16%) patients of GII p < 0.04. Mean pulmonary arterial pressure of 50 mmHg or more was present in 68% of children with Down's syndrome and in 35% of GII. Clinical course until surgical correction was down hill in 33 (60%) from GI and 21 (84%) from GII p < 0.03. Seventy seven patients underwent surgical correction. CAVC type A of the Rastelli classification was predominant in both groups, GI 37 (67%)-GII 25 (100%). There or more severe valvar morphologic lesions in group II (38%) than in group I (8%). CONCLUSION: There seems to be a pulmonary vascular hyperreactivity predominance in Down's children and cardiac insufficiency signs in the normal genetic group.

[Tetralogy of Fallot associated with left atrial isomerism]. / Tetralogia de Fallot associada a isomerismo atrial esquerdo.

The association of tetralogy of Fallot with atrial isomerism has been rarely reported. Eight cases (five with left isomerism and three with right isomerism) are known. This paper reports two other cases of tetralogy of Fallot with left atrial isomerism. The syndrome's defects were disguised and without clinical expression because of the presence of the right ventricular outlet obstruction of tetralogy of Fallot. These diagnostic elements, not recognized in one of the patients previous to surgical correction of tetralogy of Fallot, were present: junctional rhythm, bronchial isomerism, partial anomalous pulmonary vein connection, agenesy of inferior vena cava and abdominal heterotaxy; their identification previous to surgical correction of tetralogy of Fallot, is necessary for an adequate surgical management.