J. Hals et al., PULMONARY VASCULAR-RESISTANCE IN COMPLETE ATRIOVENTRICULAR SEPTAL-DEFECT - A COMPARISON BETWEEN CHILDREN WITH AND WITHOUT DOWNS-SYNDROME, Acta paediatrica, 82(6-7), 1993, pp. 595-598
Invasive data obtained in children with complete atrioventricular sept
al defect over the last three years are presented to determine the age
at which pulmonary vascular obstructive disease develops. Comparisons
were made between children with (n = 21) and without Down's syndrome
(n = 12). The investigation was restricted to patients less than one y
ear of age at initial catheterization. Patients with complicated assoc
iated heart defects were excluded. Patients with Down's syndrome had a
higher ratio of pulmonary to systemic vascular resistance than childr
en without Down's syndrome in the basal situation. This difference alm
ost disappeared after 100% oxygen had been given to patients with elev
ated pulmonary vascular resistance, indicating that hypoxia and/or hyp
oventilation is of importance, especially in children with Down's synd
rome. Fixed elevated pulmonary vascular resistance was found in 11% of
Down patients under one year, with the youngest patient being 5.5 mon
ths old. One child without Down's syndrome may have had fixed elevated
pulmonary vascular resistance. It is concluded that all children with
complete atrioventricular septal defect should be evaluated and opera
ted on at a very young age.