Aortic root pathology has been described in patients with Tetralogy of Fallot, although the most common reason for repeat surgery in the adult after TOF repair relates to problems in the right ventricular outflow trac...Aortic root pathology has been described in patients with Tetralogy of Fallot, although the most common reason for repeat surgery in the adult after TOF repair relates to problems in the right ventricular outflow tract, the aortic root is often forgotten. Objective: We sought to determine those patients with known Fallot tetrallogy at risk for progressive dilatation of the thoracic aorta and explore the common predictors present in this patient group. Methods and Results: A multicenter observational study which enrolled 100 patients (50 surgically repaired and 50 before surgical repair of TOF) with standardized reassessment of echocardiographic parameters and multislice CT angiography of the heart and great vessels data. The data were reviewed and analyzed according to the demographic, morphological, surgical and clinical details. We used standard nomograms and Z score for aortic root dimensions at the level of aortic annulus, sino-tubular junction and sinus of Valsalva based on body surface area. For surgically repaired patients, all the measured diameters across aortic annulus, STJ & sinus of Valsalva were larger in the dilated unrepaired group with mean & median of 24.63 (3.99) & 25 (15 - 35), 27.2 (4.26) & 27 (17 - 40), 35.97 (4.59) & 36 (24 - 45) mm respectively compared to a mean & median of 13.2 (2.62) & 13 (9 - 17), 14.53 (2.90) & 14 (10 - 19), 20.53 (3.40) & 21 (14 - 25) mm respectively in the not dilated unrepaired group with significant statistical difference (p value < 0.0001). Also Z score among unrepaired dilated TOF patients was larger in comparison to the non dilated unrepaired group with significant statistical difference (p value < 0.0001). For unrepaired patients, all the measured diameters across aortic annulus, STJ & sinus of Valsalva were larger in the dilated unrepaired group with mean & median of 24.63 (3.99) & 25 (15 - 35), 27.2 (4.26) & 27 (17 - 40), 35.97 (4.59) & 36 (24 - 45) mm respectively compared to a mean & median of 13.2 (2.62) & 13 (9 - 17), 14.53 (2.90) & 14 (10 - 19), 20.53 (3.40) & 21 (14 - 25) mm respectively in the not dilated unrepaired group with significant statistical difference (p value < 0.0001). Also Z score among unrepaired dilated TOF patients at the level of annulus, STJ & sinus of Valsalva was larger in comparison to the non dilated unrepaired group with significant statistical difference (p value < 0.0001). Conclusions: The first important finding of this study is the occurrence of significant aortic root dilatation in 22% of patients after intra-cardiac repair of TOF. Older age at repair, long shunt to repair interval and residual ventricular septal defect are the most common variables associated with aortopathy and aortic regurgitation in such group of patients. The second important finding is the occurrence of aortic root dilatation in 70% of patients before surgical repair of TOF;whereas male sex and TOF with pulmonary atresia appeared to be the most common variables associated with aortopathy and aortic regurgitation in this group of patients.展开更多
文摘Aortic root pathology has been described in patients with Tetralogy of Fallot, although the most common reason for repeat surgery in the adult after TOF repair relates to problems in the right ventricular outflow tract, the aortic root is often forgotten. Objective: We sought to determine those patients with known Fallot tetrallogy at risk for progressive dilatation of the thoracic aorta and explore the common predictors present in this patient group. Methods and Results: A multicenter observational study which enrolled 100 patients (50 surgically repaired and 50 before surgical repair of TOF) with standardized reassessment of echocardiographic parameters and multislice CT angiography of the heart and great vessels data. The data were reviewed and analyzed according to the demographic, morphological, surgical and clinical details. We used standard nomograms and Z score for aortic root dimensions at the level of aortic annulus, sino-tubular junction and sinus of Valsalva based on body surface area. For surgically repaired patients, all the measured diameters across aortic annulus, STJ & sinus of Valsalva were larger in the dilated unrepaired group with mean & median of 24.63 (3.99) & 25 (15 - 35), 27.2 (4.26) & 27 (17 - 40), 35.97 (4.59) & 36 (24 - 45) mm respectively compared to a mean & median of 13.2 (2.62) & 13 (9 - 17), 14.53 (2.90) & 14 (10 - 19), 20.53 (3.40) & 21 (14 - 25) mm respectively in the not dilated unrepaired group with significant statistical difference (p value < 0.0001). Also Z score among unrepaired dilated TOF patients was larger in comparison to the non dilated unrepaired group with significant statistical difference (p value < 0.0001). For unrepaired patients, all the measured diameters across aortic annulus, STJ & sinus of Valsalva were larger in the dilated unrepaired group with mean & median of 24.63 (3.99) & 25 (15 - 35), 27.2 (4.26) & 27 (17 - 40), 35.97 (4.59) & 36 (24 - 45) mm respectively compared to a mean & median of 13.2 (2.62) & 13 (9 - 17), 14.53 (2.90) & 14 (10 - 19), 20.53 (3.40) & 21 (14 - 25) mm respectively in the not dilated unrepaired group with significant statistical difference (p value < 0.0001). Also Z score among unrepaired dilated TOF patients at the level of annulus, STJ & sinus of Valsalva was larger in comparison to the non dilated unrepaired group with significant statistical difference (p value < 0.0001). Conclusions: The first important finding of this study is the occurrence of significant aortic root dilatation in 22% of patients after intra-cardiac repair of TOF. Older age at repair, long shunt to repair interval and residual ventricular septal defect are the most common variables associated with aortopathy and aortic regurgitation in such group of patients. The second important finding is the occurrence of aortic root dilatation in 70% of patients before surgical repair of TOF;whereas male sex and TOF with pulmonary atresia appeared to be the most common variables associated with aortopathy and aortic regurgitation in this group of patients.
