Progressive stiffening and relatively slow growth of the dilated ascending aorta in long-term Fontan survivors―Serial assessment for 15 years. (1st October 2020)

• Record Type: Journal Article
• Authors: Hayama, Yohsuke; Ohuchi, Hideo; Negishi, Jun; Iwasa, Toru; Sakaguchi, Heima; Miyazaki, Aya; Tsuda, Etsuko; Kurosaki, Kenichi
• Is Part Of: International journal of cardiology. Volume 316(2020)
• Page Start: 87
• Page End: 93
• Publication Date: 2020-10-01
• Subjects: AAo ascending aorta -- annulus the aortic annulus -- CHD congenital heart disease -- DAo descending aorta -- eGFR estimated glomerular filtration rate -- F0 before Fontan operation -- F1 1 year after Fontan operation -- F5 5 years after Fontan operation -- F10 10 years after Fontan operation -- F15 15 years after Fontan operation -- INR international normalized ratio -- MELD-XI modified model for end-stage liver disease excluding INR -- sinus sinus of Valsalva -- STJ sinotubular junction -- Z-X Z-score for X diameter -- β-X stiffness index of X; Fontan operation -- Single ventricle -- Ascending aorta -- Arterial stiffness -- Diastolic dysfunction; Cardiology -- Periodicals; Electronic journals; 616.12
• DOI: 10.1016/j.ijcard.2020.04.071
• Languages: English
• ISSNs: 0167-5273

Abstract

Abstract: Background: A 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. Methods: This 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. Results: Fontan 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. Conclusions: Fontan 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. Graphical abstract: Unlabelled Image Highlights: A stiffened, dilated ascending aorta represents aortopathy in single ventricle. Ascending aorta grew relatively slowly and its dilation gradually attenuated. In contrast, stiffness of ascending aorta was elevated in 15-year Fontan survivors. This stiffening was associated with diastolic dysfunction and exercise intolerance. Our findings emphasize importance of lifelong management in Fontan survivors.