Preliminary studies on sleep of patients with congenital heart disease and
Eisenmenger's syndrome (ES) at our institution demonstrated nocturnal worse
ning arterial unsaturation, which appeared to be a body position-related ph
enomenon. To investigate the potential effect of body position on gas excha
nge in ES, we carried out a prospective study of 28 patients (mean age, 34.
8 +/- 11.7 yr) with established ES due to congenital heart disease. in ever
y patient, arterial blood gases were performed during both sitting and supi
ne positions under three different conditions: room air, while breathing 10
0% oxygen, and after breathing oxygen at a flow rate of 3 L/min through nas
al prongs. Alveolar oxygen pressure (Pa-o2) for the calculation of alveolar
-arterial oxygen tension differences (AaPo(2)) was derived from the alveola
r gas equation using Pace, and assuming R = 1. We used paired t test, repea
ted-measures two-way ANOVA with Bonferroni's test, and regression analysis.
From sitting to supine position on room air, there was a significant decre
ase in Pa-o2 (from 52.5 +/- 7.5 to 47.5 +/- 5.5 mm Hg; p < 0.001) and Sa(o2
) (from 86.7 +/- 4.6 To 83.3 +/-: 4.9%; p < 0.001), both of which were corr
ected by nasal O-2 (to 68.2 +/- 21 mm Hg and to 92 +/- 4%, respectively, p
< 0.005). Pa-co2 and pH remained unchanged. The magnitude of the change In
Pa-o2 correlated with the change in AaPo(2) on room air (r = 0.77; p < 0.01
) but not with the change in AaPo(2) on 100% oxygen. It is concluded that i
n adult patients with ES there is a significant decrease in Pao(2) and Sao(
2) when they change from the sitting to the supine position. A ventilation-
perfusion ((V) over dot/(Q) over dot) distribution abnormality and/or a dif
fusion limitation phenomenon rather than an increase: in true shunt may be
the mechanisms responsible for this finding. The response to nasal O-2 We o
bserved warrants a trial with long-term nocturnal oxygen therapy in these p
atients.