Long-term Follow Up in D-Transposition of Great Arteries After Arterial Switch Operation: focusing on the aortic root dilatation and its pharmacological therapy

Autor: Kun-Lang Wu, 吳焜烺
Rok vydání: 2013
Druh dokumentu: 學位論文 ; thesis
Popis: 101
The incidence of d-transposition of great arteries (d-TGA) in Taiwan was 0.36/1000 from the national database 2000-2006, and was the second common cyanotic congenital heart disease in Taiwan. The first arterial switch operation (ASO) for the neonate in our institution was successfully performed in 1990. Based on the long-term follow up data of these TGA patients after ASO, we sought to follow up the long-term result, investigate the chance of aortic root dilatation (ARD) and the clinical correlates as the risk factors. Recent data from mouse models of Marfan syndrome suggest that aortic-root enlargement is caused by excessive signaling by transforming growth factor β (TGF-β) that can be mitigated by treatment with TGF-β antagonists, including angiotensin II–receptor blockers (ARBs).We evaluated the clinical response to ARBs in our patients who had severe aortic-root enlargement. There were 103 hospital survivors (79 males and 24 females) with d-TGA who received an ASO between 1990 and 2006 who constituted the study cohort. The ASO was performed at a median age of 12 days. Ten of them received the ASO at ages older than 6 months of the age. Prior to the ASO, palliative surgery had been performed in 16 patients. The total follow up amounted to 1284 patient-years and the mean follow up was 13.02 years. There was only one late death. Serial echocardiograms were reviewed for the size of neo-aortic root and neo-aortic regurgitation (AR). Among them, aortic root dilatation (ARD, an aortic root z score >3) was noted in 60 (58%) patients during the study period. The probability free from ARD was 71.8 %, 62.9%,55%, 44% and 37%,at 1, 2, 5, 10 and 15 years, respectively. The median time from ASO to ARD for the patients in whom it developed was 1.09 years (range, 16 days to 17.11 years). The risk factors associated with ARD were Taussig–Bing abnormality (p =0.02), body weight >7 kilograms at ASO (p=0.02), age > 6 months at ASO (P=0.01) as well as the coexisting at least moderate AR(p=0.001) and left ventricular outflow tract obstruction(p=0.01).Multivariate analysis revealed only at the least moderate AR was the only independent predictor associated with the risk of developing ARD(p=0.005, Odds ratio=2.2). AR was noted in 73 patients (70.8%); moderate in 9 (8.73%) and severe in 4 (3.8%). While AR tended to be higher with increasing degree of neo-aortic root dilatation. (P3) was common and the probability free from ARD was only 55% 5 years after ASO, particularly in those repaired at older age. ARB seems helpful to reduce the progression rate of ARD and will be a promising therapeutic regimen in repaired TGA patients. Running title: arterial switch operation, transposition of great arteries, aortic root dilatation, angiotensin II–receptor blockers, Angiotensin-converting enzyme inhibitors Keywords：Angiotensin II–receptor blockers；Aortic root dilatation；Aortic valve regurgitation；Arterial switch operation；D-transposition of great arteries
Databáze: Networked Digital Library of Theses & Dissertations
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