TETRALOGY OF FALLOT WITH ABSENT PULMONARY VALVE - ECHOCARDIOGRAPHIC MORPHOMETRIC FEATURES OF THE RIGHT-SIDED STRUCTURES AND THEIR RELATIONSHIP TO PRESENTATION AND OUTCOME
Mt. Donofrio et al., TETRALOGY OF FALLOT WITH ABSENT PULMONARY VALVE - ECHOCARDIOGRAPHIC MORPHOMETRIC FEATURES OF THE RIGHT-SIDED STRUCTURES AND THEIR RELATIONSHIP TO PRESENTATION AND OUTCOME, Journal of the American Society of Echocardiography, 10(5), 1997, pp. 556-561
Respiratory symptoms in tetralogy of Fallot with absent pulmonary valv
e are believed to be due to bronchial compression secondary to dilated
pulmonary arteries; however, not all patients are born compromised. E
chocardiographic morphometry of the right-sided structures was investi
gated to determine the possible relationship between anatomy and clini
cal presentation. Twenty-five patients were identified, and 15 had pre
operative echocardiograms. Patients were divided into two groups: thos
e with respiratory distress (group I, n = 9) and those without (group
II, n = 6). No difference was noted in branch pulmonary artery diamete
rs between groups; however, the pulmonary valve/aortic valve ratio, re
flecting the dimension of the narrowest pathway from the right ventric
le, was larger in group I (0.74 +/- 0.15 versus 0.60 +/- 0.07, p < 0.0
5). Pulmonary, valve diameter correlated with main and right pulmonary
artery diameters. We conclude that patients with tetralogy of Fallot
with absent pulmonary valve and respiratory compromise have a greater
pulmonary valve/aortic valve ratio but do not have greater dilatation
of proximal branch pulmonary arteries. This suggests that the pathophy
siology is not due solely to compression of the bronchi but is also re
lated to the blood flow dynamics in the pulmonary vessels.