title: Complete repair in total atrioventricular canal defect with cyanosis.
abstract: Atrioventricular septal defects account for 4% of congenital cardiac malformations and over 50% of cardiac defects seen in Down syndrome. Clinical presentation is marked by congestive heart failure early in infancy. Cyanosis is rarely found in infants and suggests irreversible pulmonary hypertension or associated cardiac defects as tetralogy of Fallot, double outlet right ventricle, Ebstein anomaly, persistent left superior vena cava draining in the left atrium (Barbero Marcial, personal communication). Children with Down's syndrome is particularly difficult to assess because they often suffer from upper airways obstruction, which may contribute to the increased pulmonary vascular resistance determined at cardiac catheterization. This association of factors becomes a challenge for operability and, we will report one such case.
authors.affiliation: Instituto do Coração, Hospital das Clínicas, FMUSP, São Paulo, SP. ctanama@terra.com.br
authors.forename: Carla
authors.initials: C
authors.lastname: Tanamati
authors.forename: Renata Lure
authors.initials: RL
authors.lastname: Suguimoto
authors.forename: Edmar
authors.initials: E
authors.lastname: Atik
authors.forename: Solange Gimenez
authors.initials: SG
authors.lastname: Copolla
authors.forename: Filomena Regina
authors.initials: FR
authors.lastname: Galles
authors.forename: Vera Demarchi
authors.initials: VD
authors.lastname: Aiello
authors.forename: Miguel Lorenzo
authors.initials: ML
authors.lastname: Barbero-Marcial
mesh_terms: D003490:Cyanosis / Q000209:etiology*; D004314:Down Syndrome / Q000150:complications*; D005260:Female; D006333:Heart Failure / Q000209:etiology*; D006343:Heart Septal Defects / Q000150:complications / Q000601:surgery*; D006801:Humans; D007223:Infant; D012720:Severity of Illness Index; D016896:Treatment Outcome