Sengupta Aditya, Lee Ji M, Gauvreau Kimberlee, Colan Steven D, Del Nido Pedro J, Mayer John E, Nathan Meena
Department of Cardiac Surgery, Boston Children's Hospital, Boston, Mass.
Department of Cardiology, Boston Children's Hospital, Boston, Mass; Department of Biostatistics, Harvard School of Public Health, Boston, Mass.
J Thorac Cardiovasc Surg. 2023 Dec;166(6):1718-1728.e4. doi: 10.1016/j.jtcvs.2023.04.014. Epub 2023 May 8.
We sought to characterize the natural history of aortic root dilatation and aortic regurgitation in tetralogy of Fallot (TOF).
A single-center review of patients who underwent TOF repair from January 1960 to December 2022 was performed. Morphology was categorized as TOF-pulmonary stenosis or TOF-variant (including TOF-pulmonary atresia and TOF-pulmonary atresia-major aortopulmonary collateral arteries). Echocardiographically determined diameters and derived z scores were measured at the annulus, sinus of Valsalva, and sinotubular junction immediately before TOF repair and throughout follow-up. Linear mixed-effects models assessed trends in dimensions over time.
Of 2205 patients who underwent primary repair of TOF at a median age of 4.9 months (interquartile range, 2.3-20.5 months) and survived to discharge, 1608 (72.9%) patients had TOF-pulmonary stenosis and 597 (27.1%) patients had TOF-variant. At a median postoperative follow-up of 14.4 years (interquartile range, 3.3-27.6 years; range, 0.1-62.6 years), 313 (14.2%) patients had mild or greater aortic regurgitation and 34 (1.5%) patients required an aortic valve or root intervention. The overall mean rates of annular, sinus of Valsalva, and sinotubular junction growth were 0.5 ± 0.2, 0.6 ± 0.3, and 0.7 ± 0.5 mm/year, respectively. Root z scores remained stable with time. At baseline, patients with TOF-variant had larger diameters and z scores at the annulus, sinus of Valsalva, and sinotubular junction, compared with patients with TOF-pulmonary stenosis (all P values < .05). Over time, patients with TOF-variant demonstrated relatively greater annular (P = .020), sinus of Valsalva (P < .001), and sinotubular junction (P < .001) dilatation. Patients with ≥75th percentile root growth rates had a higher incidence of mild or greater aortic regurgitation (P < .001), moderate or greater aortic regurgitation (P < .001), and aortic valve repair or replacement (P = .045).
Patients with TOF-variant are at comparatively greater risk of pathologic root dilatation over time, warranting closer longitudinal follow-up.
我们试图描述法洛四联症（TOF）患者主动脉根部扩张和主动脉反流的自然病程。
对1960年1月至2022年12月期间接受TOF修复手术的患者进行单中心回顾性研究。形态学分类为TOF-肺动脉狭窄或TOF变异型（包括TOF-肺动脉闭锁和TOF-肺动脉闭锁-主要体肺侧支动脉）。在TOF修复术前及整个随访过程中，通过超声心动图测量瓣环、主动脉窦和窦管交界处的直径及衍生的z评分。线性混合效应模型评估各维度随时间的变化趋势。
2205例接受TOF一期修复手术的患者，中位年龄为4.9个月（四分位间距，2.3 - 20.5个月），存活至出院，其中1608例（72.9%）为TOF-肺动脉狭窄，597例（27.1%）为TOF变异型。术后中位随访14.4年（四分位间距，3.3 - 27.6年；范围，0.1 - 62.6年），313例（14.2%）患者出现轻度或更严重的主动脉反流，34例（1.5%）患者需要进行主动脉瓣或根部干预。瓣环、主动脉窦和窦管交界处的总体平均生长速率分别为0.5±0.2、0.6±0.3和0.7±0.5 mm/年。根部z评分随时间保持稳定。基线时，与TOF-肺动脉狭窄患者相比，TOF变异型患者在瓣环、主动脉窦和窦管交界处的直径和z评分更大（所有P值<0.05）。随着时间推移，TOF变异型患者的瓣环（P = 0.020）、主动脉窦（P < 0.001）和窦管交界处（P < 0.001）扩张相对更明显。根部生长速率≥第75百分位数的患者，轻度或更严重主动脉反流（P < 0.001）、中度或更严重主动脉反流（P < 0.001）以及主动脉瓣修复或置换（P = 0.045）的发生率更高。
随着时间推移，TOF变异型患者发生病理性根部扩张的风险相对更高，需要更密切的长期随访。
