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Restrictive versus nonrestrictive physiology following repair of tetralogy of Fallot: is there a difference? J Am Soc Echocardiogr 2013 Jul;26(7):746-55

Date

04/30/2013

Pubmed ID

23623591

DOI

10.1016/j.echo.2013.03.019

Scopus ID

2-s2.0-84879418368 (requires institutional sign-in at Scopus site)   27 Citations

Abstract

BACKGROUND: Long-term outcome in repaired tetralogy of Fallot (TOF) is related to chronic pulmonary insufficiency (PI), right ventricular (RV) dilation, and deterioration of RV function. The aim of this study was to characterize clinical differences between restrictive and nonrestrictive RV physiology in young patients with repaired TOF.

METHODS: Patients were prospectively enrolled from February 2008 to August 2009. Each had a clinic visit, brain natriuretic peptide assessment, exercise test, cardiac magnetic resonance study, and echocardiographic examination with assessment of regional myocardial mechanics. Consistent antegrade diastolic pulmonary arterial flow with atrial contraction identified restrictive RV physiology.

RESULTS: Twenty-nine patients (median age, 12 years; range, 8-33 years; nine male patients) were studied. Twelve had restrictive RV physiology. The median time since initial TOF repair was 12 years (range, 5-27 years). Restrictive physiology appeared more prevalent after transannular patch repair and was not influenced by other demographic features. The restrictive group had more PI (46% vs 28%, P = .002), larger RV end-diastolic volumes (128 vs 98 mL/m(2), P = .046), but similar ejection fractions, brain natriuretic peptide levels, New York Heart Association classes, and exercise capacity. RV basal and mid free wall peak diastolic strain rate differed between groups, negatively correlating with exercise time and positively correlating with PI in patients with restrictive physiology.

CONCLUSIONS: Restrictive RV physiology correlates with a larger right ventricle and increased PI after TOF repair but does not negatively affect other markers of myocardial health. Diastolic regional RV myocardial mechanics, particularly diastolic velocity and peak diastolic strain rate, differ for postoperative TOF patients with restrictive and nonrestrictive RV physiology; longitudinal study is necessary to understand the relationship of regional myocardial mechanics and patients' clinical status.

Author List

Samyn MM, Kwon EN, Gorentz JS, Yan K, Danduran MJ, Cava JR, Simpson PM, Frommelt PC, Tweddell JS

Authors

Joseph R. Cava MD, PhD Associate Professor in the Pediatrics department at Medical College of Wisconsin
Peter C. Frommelt MD Adjunct Professor in the Pediatrics department at Medical College of Wisconsin
Margaret Mary Samyn MD Professor in the Pediatrics department at Medical College of Wisconsin
Pippa M. Simpson PhD Adjunct Professor in the Pediatrics department at Medical College of Wisconsin
Ke Yan PhD Associate Professor in the Pediatrics department at Medical College of Wisconsin




MESH terms used to index this publication - Major topics in bold

Adolescent
Adult
Cardiomyopathy, Restrictive
Child
Echocardiography
Exercise Test
Female
Humans
Magnetic Resonance Imaging
Male
Postoperative Complications
Prospective Studies
Statistics, Nonparametric
Tetralogy of Fallot
Treatment Outcome