Pulmonary artery size and function after Fontan operation at a young age.
OBJECTIVE
To assess pulmonary artery (PA) size, flow variables, and wall shear stress (WSS) in patients after Fontan operation at a young age.


METHODS
Flow in the branch PA was obtained with phase contrast velocity-encoded cardiovascular magnetic resonance imaging in 14 patients before and after low-dose dobutamine stress (7.5 microg/kg/min) and in 17 healthy controls at rest.


RESULTS
At rest, stroke index, total flow, average, and peak flow rate were all statistically significantly lower in patients than in controls (P<0.001). With stress-testing, all variables increased in patients (P<0.001), apart from stroke index, which did not change. At rest, branch PA area did not differ between patients and controls. Distensibility was lower in patients than in controls (P<0.001). With stress-testing, area and distensibility did not change. At rest, WSS was lower in patients than in controls (P<0.001). WSS increased with stress-testing (P<0.001), but not to the same levels as during resting conditions of the control group.


CONCLUSIONS
PA size is normal long-term after Fontan operation at a young age. Flow variables, distensibility, and WSS are significantly lower compared to healthy controls, and do not show adequate reactions with stress-testing, which is suggestive of pulmonary artery endothelial and/or vascular dysfunction.
Robbers-Visser
Daniëlle
D
Department of Pediatrics, Division of Cardiology, Erasmus MC-Sophia Children's Hospital, Rotterdam, The Netherlands.
Helderman
Frank
F
Strengers
Jan L
JL
van Osch-Gevers
Lennie
L
Kapusta
Livia
L
Pattynama
Peter M
PM
Bogers
Ad J
AJ
Krams
Rob
R
Helbing
Willem A
WA
D000293:Adolescent; D002648:Child; D004280:Dobutamine; D005080:Exercise Test; D005260:Female; D018729:Fontan Procedure; D006352:Heart Ventricles / Q000002:abnormalities* / Q000473:pathology / Q000601:surgery*; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D009929:Organ Size; D011379:Prognosis; D011651:Pulmonary Artery / Q000473:pathology*; D016896:Treatment Outcome; D055815:Young Adult
D004280:Dobutamine