Progressive stiffening and relatively slow growth of the dilated ascending aorta in long-term Fontan survivors―Serial assessment for 15 years
Progressive stiffening and relatively slow growth of the dilated ascending aorta in long-term Fontan survivors―Serial assessment for 15 years
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Fontan 长期幸存者的升主动脉进行性硬化和相对缓慢的生长——15 年的系列评估
DOI：
10.1016/j.ijcard.2020.04.071
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发表时间：
2020
影响因子：
3.5
通讯作者：
Kurosaki Kenichi
中科院分区：
文献类型：
--
作者：
Hayama Yohsuke;Ohuchi Hideo;Negishi Jun;Iwasa Toru;Sakaguchi Heima;Miyazaki Aya;Tsuda Etsuko;Kurosaki Kenichi
背景：升主动脉硬化、扩张可能是心血管死亡率的重要预测因子，在包括单心室在内的先天性心脏病患者中已有报道。然而，一系列的构象变化和决定因素，降低膨胀性在升主动脉还没有澄清。MethodsThis回顾性研究调查了115术后Fontan幸存者（中位年龄Fontan：3.7岁）。所有患者均在Fontan手术前和术后1、5、10和15年接受心导管检查。我们测量了血管造影片中升主动脉和降主动脉直径和刚度指数（β）的Z评分。我们还审查了患者的临床资料，血流动力学参数和运动能力，并将其与47名对照subjects.ResultsFontan幸存者的结果进行了比较，显示出显着较大的Z-评分和β的升主动脉从手术前到15年后比对照组，而值为降主动脉是可比的。根据趋势检验，升主动脉的Z评分降低，但β显著升高。多因素分析显示，Fontan术后15年升主动脉β值及其增加趋势与Fontan手术时年龄较大和心室舒张末期压升高相关。减少运动能力也与硬化的升aortic.ConclusionsFontan幸存者表现出渐进硬化和相对缓慢的增长扩张的升主动脉。升主动脉的进行性硬化可能与舒张功能障碍和运动能力降低相关，提示亚临床Fontan病理生理学终身管理的重要性。
BackgroundA stiffened, dilated ascending aorta may represent an important predictor of cardiovascular mortality, and has been reported in patients with congenital heart disease, including single ventricle. However, the serial conformational changes and determinants of reduced distensibility in ascending aorta have not been clarified.MethodsThis retrospective study investigated 115 postoperative Fontan survivors (median age at Fontan: 3.7 years). All patients underwent cardiac catheterization before and 1, 5, 10, and 15 years after the Fontan operation. We measured Z-scores for diameters and stiffness indexes (β) of the ascending aorta and descending aorta from angiograms. We also reviewed the clinical profiles, hemodynamic parameters, and exercise capacities of patients and compared them with results from 47 control subjects.ResultsFontan survivors displayed significantly larger Z-score and β of the ascending aorta from before to 15 years after surgery than controls, whereas values for the descending aorta were comparable. Z-score for the ascending aorta was decreased, but β was elevated significantly according to the trend test. In multivariable analysis, β of the ascending aorta at 15 years after Fontan operation and its increasing trend were associated with older age at Fontan operation and elevated ventricular end-diastolic pressure. Reduced exercise capacity also correlated with stiffening of the ascending aorta.ConclusionsFontan survivors showed progressive stiffening and relatively slow growth of the dilated ascending aorta. Progressive stiffening of the ascending aorta may be coupled to diastolic dysfunction and reduced exercise capacity, suggesting the importance of lifelong management of subclinical Fontan pathophysiology.