title: [Complete atrioventricular septal defect. Anatomo-functional correlation between patients with and without Down's syndrome].
abstract: OBJECTIVE
  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%).
  
  
  CONCLUSIONS
  There seems to be a pulmonary vascular hyperreactivity predominance in Down's children and cardiac insufficiency signs in the normal genetic group.
authors.affiliation: Instituto do Coração do Hospital das Clínicas, FMUSP.
authors.forename: L M
authors.initials: LM
authors.lastname: Ferrín
authors.forename: E
authors.initials: E
authors.lastname: Atik
authors.forename: N M
authors.initials: NM
authors.lastname: Ikari
authors.forename: T C
authors.initials: TC
authors.lastname: Martins
authors.forename: M B
authors.initials: MB
authors.lastname: Marcial
authors.forename: M
authors.initials: M
authors.lastname: Ebaid
mesh_terms: D004314:Down Syndrome / Q000150:complications* / Q000503:physiopathology; D005260:Female; D006344:Heart Septal Defects, Atrial / Q000150:complications* / Q000601:surgery; D006345:Heart Septal Defects, Ventricular / Q000150:complications* / Q000601:surgery; D006439:Hemodynamics; D006801:Humans; D006976:Hypertension, Pulmonary / Q000503:physiopathology; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D012189:Retrospective Studies
vernacular_title: Defeito total do septo atrioventricular. Correlação anatomofuncional entre pacientes com e sem síndrome de Down.