title: Differential Impact of Pulmonary Regurgitation on Patients With Surgically Repaired Pulmonary Stenosis Versus Tetralogy of Fallot.
abstract: Patients with repaired pulmonary stenosis (PS) or tetralogy of Fallot (TOF) both develop pulmonary regurgitation (PR) leading to right ventricular (RV) dilatation and dysfunction. We aimed to characterize differential effects of chronic PR in these populations. Patients with surgically repaired PS were matched 1:2 by age and PR fraction with patients with TOF. Patients with previous pulmonary valve replacement were excluded. Cardiovascular magnetic resonance data were compared; peak longitudinal and circumferential systolic strain by feature tracking were compared to evaluate differential contribution of the RV sinus and outflow tract, respectively. PS (n = 24, 41 ± 13 years old) and TOF (n = 47, 39 ± 13 years old) patients did not differ in RV end-diastolic volume (153 ± 45 vs 154 ± 45 ml/m(2), p = 0.99) or diastolic function. However, patients with PS had preserved RV ejection fraction (54.3 ± 4.4% vs 48.0 ± 7.1%, p <0.0001). Differences were greater in RV circumferential (-15.8 ± 3.3 vs -11.8 ± 3.4, p <0.0001) than longitudinal strain (-18.0 ± 3.8 vs -15.9 ± 3.8, p = 0.04), with particular decrease in the infundibulum (-17.4 ± 7.5 vs -6.8 ± 6.3, p <0.0001). Late gadolinium enhancement in the RV outflow tract was more frequent in patients with TOF (70.2% vs 45.8%, p = 0.001). In conclusion, surgical repair of PS leads to similar RV dilatation and diastolic dysfunction compared to patients with TOF, but differential effects on ventricular systolic function, largely related to differences in the outflow tract. With different patterns of scarring and ventricular remodeling, further study is needed to clarify whether criteria for pulmonary valve replacement in patients with PS should differ from those with TOF.
authors.affiliation: Division of Pediatric Cardiology, Department of Pediatrics and Communicable Diseases, University of Michigan, Ann Arbor, Michigan. Electronic address: mjoynt@med.umich.edu.
authors.forename: Michael R
authors.initials: MR
authors.lastname: Joynt
authors.affiliation: Division of Pediatric Cardiology, Department of Pediatrics and Communicable Diseases, University of Michigan, Ann Arbor, Michigan.
authors.forename: Sunkyung
authors.initials: S
authors.lastname: Yu
authors.affiliation: Division of Pediatric Cardiology, Department of Pediatrics and Communicable Diseases, University of Michigan, Ann Arbor, Michigan; Section of Pediatric Radiology, Department of Radiology, University of Michigan, Ann Arbor, Michigan.
authors.forename: Adam L
authors.initials: AL
authors.lastname: Dorfman
authors.affiliation: Section of Pediatric Radiology, Department of Radiology, University of Michigan, Ann Arbor, Michigan; Division of Cardiothoracic Radiology, Department of Radiology, University of Michigan, Ann Arbor, Michigan.
authors.forename: Maryam
authors.initials: M
authors.lastname: Ghadimi Mahani
authors.affiliation: Division of Cardiothoracic Radiology, Department of Radiology, University of Michigan, Ann Arbor, Michigan.
authors.forename: Prachi P
authors.initials: PP
authors.lastname: Agarwal
authors.affiliation: Division of Pediatric Cardiology, Department of Pediatrics and Communicable Diseases, University of Michigan, Ann Arbor, Michigan; Section of Pediatric Radiology, Department of Radiology, University of Michigan, Ann Arbor, Michigan.
authors.forename: Jimmy C
authors.initials: JC
authors.lastname: Lu
mesh_terms: D000293:Adolescent; D000328:Adult; D006348:Cardiac Surgical Procedures; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D019028:Magnetic Resonance Imaging, Cine; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011665:Pulmonary Valve Insufficiency / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology; D011666:Pulmonary Valve Stenosis / Q000150:complications / Q000175:diagnosis / Q000601:surgery*; D012189:Retrospective Studies; D013318:Stroke Volume; D013771:Tetralogy of Fallot / Q000150:complications / Q000175:diagnosis / Q000601:surgery*; D020257:Ventricular Remodeling / Q000502:physiology*; D055815:Young Adult