Abstract
Background: Repaired Tetralogy of Fallot (rTOF) patients may have residual lesions such as main (MPA) and branch pulmonary artery stenosis (BPAS). While MPA stenosis is well studied, few data are available on BPAS in rTOF. We aimed to describe pulmonary perfusion in a large paediatric cohort of rTOF and its impact on right ventricular and outflow-tract hemodynamics using 4D flow CMR. Methods: 130 consecutive patients (mean age at CMR 14.3 ± 4.6 years) were retrospectively reviewed. 96 patients had transannular patch without valve preservation while 34 patients had conserved annulus or valved conduit. A pulmonary blood flow ratio (right pulmonary artery (RPA)/left pulmonary artery (LPA)) between 0.75 and 1.56 was considered normal. Results: Asymmetric pulmonary perfusion was present in 59/130 patients (45%), with 54/59 (91%) having left lung hypoperfusion (blood flow ratio >1.56). RPA/LPA perfusion ratio in the whole cohort was independently associated with the LPA Z-score (−0.053, p = 0.007), the RPA regurgitant fraction (RF) (0.013, p = 0.011) and previous LPA stenting (0.648, p = 0.004). Decreasing LPA % perfusion (and conversely increasing RPA % perfusion) was significantly associated with higher MPA diameter Z-score (−0.06, p = 0.007). On multivariate analysis, MPA Z-score was independently associated with pulmonary RF (0.48, p
Highlights
Tetralogy of Fallot (TOF) is the most common cyanotic heart defect (CHD), accounting for 3%–5% of all infants born with CHD [1]
The advent of cardiac surgery has led to improved survival to adulthood with about 17 per 100,000 adults currently living with repaired TOF [2,3]
Patients were divided in two groups according to previous surgical treatment: Group 1-transannular patch without valve preservation; Group 2-surgical repair with conserved valve function
Summary
Tetralogy of Fallot (TOF) is the most common cyanotic heart defect (CHD), accounting for 3%–5% of all infants born with CHD [1]. Not cured, and many have residual hemodynamic lesions such as pulmonary regurgitation (PR), residual right ventricular outflow tract (RVOT) or main pulmonary artery (MPA) stenosis, and branch pulmonary artery stenosis (BPAS). Past investigations found that patients with significant residual unilateral BPAS seem to have reduced exercise tolerance compared to other rTOF patients [6–10] These studies suggested that BPAS may exacerbate pulmonary regurgitation (PR). Repaired Tetralogy of Fallot (rTOF) patients may have residual lesions such as main (MPA) and branch pulmonary artery stenosis (BPAS). In patients with transannular patch repair, asymmetric pulmonary flow was an independent predictor of right ventricular ejection fraction (RVEF) (−3.66, p = 0.04). Conclusions: Pulmonary perfusion asymmetry is frequent in rTOF and is associated with abnormal right ventricular and outflow-tract hemodynamics, including MPA dilatation and decreased RVEF in patients after transannular patch
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