Diastolic function in repaired tetralogy of Fallot at rest and during stress: assessment with MR imaging.
OBJECTIVE
To prospectively assess, with magnetic resonance (MR) imaging, right ventricular (RV) diastolic function after repair of tetralogy of Fallot (TOF) at rest and during pharmacologic stress and to study relationship between main pulmonary artery end-diastolic forward flow (EDFF) (indicative of restrictive RV physiology) and clinical status.


METHODS
Institutional medical ethics committee approval and patient or parent informed consent were obtained. Patients with TOF corrected through the transatrial-transpulmonary approach underwent MR imaging at rest and during dobutamine stress and maximal exercise testing. Two-dimensional (2D) cine volumetric data were acquired. Flow measurements were performed with a standard 2D flow-sensitized sequence. MR imaging flow curves for tricuspid and pulmonary valves were combined into RV time-volume change curves, from which indexes of RV filling were derived. Patient results were compared with published data in control subjects. Student t tests, Mann-Whitney U tests, analysis of covariance, and paired and one-sample t tests were used.


RESULTS
Thirty-six patients (mean age at repair, 0.9 year +/- 0.5 [standard deviation]; median age at study inclusion, 17 years [range, 7-23 years]; 26 male and 10 female patients) were included. Abnormalities in RV filling included impaired relaxation (prolonged deceleration time, P = .002; smaller early filling fraction, P = .02) in the entire group compared with published data in healthy control subjects and signs of restriction to RV filling (smaller atrial filling fraction and higher early filling/atrial filling peak ratio, P < .05 for both) in patients with EDFF (n = 24) compared with patients without EDFF (n = 12). Stress response was abnormal in patients with EDFF, who developed impaired RV relaxation not appreciated at rest. Patients with EDFF had more severe pulmonary regurgitation (P < .05) and poorer exercise performance (P < .001).


CONCLUSIONS
In patients with TOF corrected with currently widely accepted surgical strategies, pulmonary artery EDFF relates to worse clinical state at mid- to long-term follow-up. Dobutamine stress imaging may unmask abnormalities in RV diastolic filling not appreciated with rest imaging alone.
van den Berg
Jochem
J
Department of Pediatric Cardiology, Sophia Children's Hospital, Erasmus Medical Center, Dr Molewaterplein 60, 3015 GJ Rotterdam, the Netherlands.
Wielopolski
Piotr A
PA
Meijboom
Folkert J
FJ
Witsenburg
Maarten
M
Bogers
Ad J J C
AJ
Pattynama
Peter M T
PM
Helbing
Willem A
WA
D000293:Adolescent; D000328:Adult; D002648:Child; D003971:Diastole / Q000502:physiology; D005080:Exercise Test; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D011665:Pulmonary Valve Insufficiency / Q000150:complications*; D013771:Tetralogy of Fallot / Q000150:complications* / Q000601:surgery; D018497:Ventricular Dysfunction, Right / Q000175:diagnosis* / Q000209:etiology / Q000503:physiopathology