title: Pulmonary vascular disease in Down's syndrome with complete atrioventricular septal defect.
abstract: We sought to determine the predisposing factors of pulmonary vascular disease (PVD) in complete atrioventricular septal defect. Down's syndrome is considered a risk factor for PVD, but the progression of PVD differs in each case. Morphometric analysis in autopsied hearts showed that Rastelli type A morphology had a narrower left ventricular outlet and a wider right ventricular outlet than did type C. In 81 consecutive patients with Down's syndrome who underwent cardiac catheterization, we estimated the following variables: Rastelli subtypes, pulmonary vascular resistance, pulmonary-to-systemic flow ratio, patients' age, and operative outcome. The hemodynamic variables in those <1 year old did not differ between the groups with type A and type C. However, all 5 patients with fatal pulmonary hypertension in early infancy had type A morphology. The lung histology revealed that 3 of these patients already had irreversible PVD. At >/=1 year old, those with type A showed a significantly higher pulmonary vascular resistance (p <0.001) and a lower pulmonary to systemic flow ratio (p <0.05) than did those <1 year old. In contrast, neither of these variables in the type C group differed between those >/=1 and <1 year old. Moreover, those with type A had a greater risk of being contraindicated for surgical repair (p <0.05). We suspect, therefore, that type A morphology is an independent risk factor for PVD in those with Down's syndrome associated with this anomaly. This hemodynamic influence could become obvious once patients are >/=1 year old. It may also sometimes result in irreversible PVD even in early infancy.
authors.affiliation: Department of Pediatrics, The Sakakibara Heart Institute, Japan Research Promotion Society for Cardiovascular Disease, Tokyo, Japan. k.suzuki@moatokyo.or.jp
authors.forename: K
authors.initials: K
authors.lastname: Suzuki
authors.forename: S
authors.initials: S
authors.lastname: Yamaki
authors.forename: S
authors.initials: S
authors.lastname: Mimori
authors.forename: Y
authors.initials: Y
authors.lastname: Murakami
authors.forename: K
authors.initials: K
authors.lastname: Mori
authors.forename: Y
authors.initials: Y
authors.lastname: Takahashi
authors.forename: T
authors.initials: T
authors.lastname: Kikuchi
mesh_terms: D000293:Adolescent; D006328:Cardiac Catheterization; D002648:Child; D002675:Child, Preschool; D004314:Down Syndrome / Q000150:complications*; D005260:Female; D006345:Heart Septal Defects, Ventricular / Q000150:complications* / Q000473:pathology / Q000601:surgery; D006439:Hemodynamics; D006801:Humans; D006976:Hypertension, Pulmonary / Q000145:classification / Q000209:etiology* / Q000401:mortality; D007223:Infant; D008297:Male; D011652:Pulmonary Circulation; D012307:Risk Factors; D014655:Vascular Resistance