• Shivani M Bhatt, Yan Wang, Okan U Elci, Elizabeth Goldmuntz, Michael McBride, Stephen Paridon, and Laura Mercer-Rosa.
• Division of Cardiology, Department of Pediatrics, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania. Electronic address: bhatts@email.chop.edu.
• J Am Soc Echocardiogr. 2019 Jan 1; 32 (1): 135-144.

BackgroundPulmonary insufficiency (PI) and right ventricular (RV) dysfunction are long-term complications in patients with repaired tetralogy of Fallot (rTOF). The aim of this study was to investigate RV contractile reserve and changes in PI that occur during exercise in patients with rTOF and the associations of these changes with exercise performance using stress echocardiography.MethodsSubjects with rTOF (n = 32) and healthy control subjects (n = 10) were prospectively enrolled and underwent rest and peak exercise echocardiography during standard cardiopulmonary exercise test protocol on a cycle ergometer or treadmill. RV contractile reserve was defined as the change in RV global longitudinal strain from rest to peak exercise. PI was assessed with the diastolic-to-systolic time-velocity integral ratio and diastolic/systolic velocity ratio from pulmonary artery Doppler interrogation. Exercise measures included heart rate reserve, percentage predicted maximum oxygen consumption, percentage predicted maximum work, and oxygen pulse.ResultsRV contractile reserve was impaired in patients with rTOF compared with control subjects, with a significant drop in the absolute value of RV global longitudinal strain from 17% (range, 8%-27%) at rest to 13% (range, 5%-28%) at peak exercise. Similarly, PI decreased at peak exercise, with decreases in diastolic-to-systolic time-velocity integral and diastolic/systolic velocity ratios. Reduction in PI was directly associated with percentage predicted maximum oxygen consumption, percentage predicted maximum work, and greater oxygen pulse. Heart rate reserve was directly associated with percentage predicted maximum oxygen consumption and percentage predicted maximum work. RV contractile reserve was not associated with any exercise parameters.ConclusionsPatients with rTOF have an abnormal myocardial response to exercise with impaired RV contractile reserve compared with control subjects. Heart rate reserve and reduction in PI at peak exercise are associated with better exercise performance and appear to be significant contributors to exercise performance in rTOF. Measures to improve chronotropic health in rTOF should be explored.Copyright © 2018 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.

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