Abstract
Background: The aim was to determine the effect of pulmonary artery (PA) morphology on the branch pulmonary artery-regurgitation fraction (BPA-RF), the relationship of pulmonary insufficiency (PI) to BPA-RF and PA-distensibility, and factors (BPA-RF and PA-distensibility) associated with right ventricular function (RVF) in repaired tetralogy of Fallot (rTOF). Methods: A total of 182 rTOF patients (median age 17.1 years) were analyzed for length, angle of PA, BPA-RF, PI, and PA-distensibility, using magnetic resonance imaging. Results: The left PA had a significant greater RF than the right PA (median (interquartile range)): LPA 43.1% (32.6–51.5) and RPA 35.2% (24.7–44.7), p < 0.001. The LPA was shorter with a narrower angle than the RPA (p < 0.001). The anatomy of the branch-PA was not a factor for the greater LPA-RF (odds ratio, 95% confidence interval: CI, p-value): length 0.44 (0.95–2.00), p = 0.28; angle 0.63 (0.13–2.99), p = 0.56. There was a strong positive correlation between PI and BPA-RF-coefficients (95% CI), p-value: LPA 0.78% (0.70–0.86), p < 0.001; RPA 0.78% (0.71–0.84), p < 0.001 and between BPA-RF and distensibility-coefficients (95%CI), p-value: LPA 0.73% (0.37–1.09), p < 0.001; RPA 1.63% (1.22–2.03), p < 0.001, respectively. The adjusted BPA-RF did not predict RVF, RPA (p = 0.434), LPA (p = 0.268). Conclusions: PA morphology is not a significant factor for the differential BPA-RF. The vascular wall in rTOF patients responds to chronic increased intravascular volume by increasing distensibility. BPA-RF is not a determinant of RVF.
Highlights
Tetralogy of Fallot is one of the most common cyanotic heart diseases, and requires surgical repair during infancy or early childhood [1]
The purpose of this study is to assess the effect of pulmonary artery morphology on the differential branch pulmonary artery regurgitation fraction (BPA-RF), to test the relationship between pulmonary insufficiency and BPA-RF and vascular distensibility, and to determine factors associated with right ventricular function, using Magnetic resonance imaging (MRI)
One hundred and twelve patients were excluded for the following reasons: repaired tetralogy of Fallot (rTOF) with percutaneous or surgical pulmonary valve replacement (n = 22), rTOF with Rastelli operation (n = 2), significant pulmonary artery stenosis which was defined as a greater than 50% narrowing diameter compared with the adjacent vessel proximally (n = 63) [15], moderate to severe pulmonary valve stenosis which was defined as a peak velocity across the valve greater than 3 m/s (n = 1) [16], and incomplete MRI data (n = 24)
Summary
Tetralogy of Fallot is one of the most common cyanotic heart diseases, and requires surgical repair during infancy or early childhood [1]. Post-repair sequelae, including pulmonary insufficiency, right ventricular dilatation, biventricular dysfunction, and exercise intolerance, are common in patients with repaired tetralogy of Fallot (rTOF) [3,4]. Pulmonary insufficiency may cause flow changes in both left and right pulmonary arteries (LPA, RPA) and regurgitation in the branch pulmonary arteries. Magnetic resonance imaging (MRI) is a gold standard to evaluate pulmonary insufficiency and flow dynamics in patients with rTOF [6]. The aim was to determine the effect of pulmonary artery (PA) morphology on the branch pulmonary artery-regurgitation fraction (BPA-RF), the relationship of pulmonary insufficiency (PI) to BPA-RF and PA-distensibility, and factors (BPA-RF and PA-distensibility) associated with right ventricular function (RVF) in repaired tetralogy of Fallot (rTOF). Methods: A total of 182 rTOF patients (median age 17.1 years) were analyzed for length, angle of PA, BPA-RF, PI, and PA-distensibility, using magnetic resonance imaging.
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