PubMed · 16564286
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Frank M Midgley. 2006. Invited commentary.. https://doi.org/10.1016/j.athoracsur.2005.09.055
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Two patients with anomalous right subclavian artery proximal to aortic coarctation are reported for their rarity. The embryological basis and surgical implications are briefly discussed. The major surgical implication of this anomaly relates to the risk of spinal cord ischemia and in these cases, the repair was performed safely.
In male patients with congenital anomalies of the kidney and urinary tract, an increased incidence of a polymorphism in the angiotensin type 2 receptor gene (AT2R) has been identified. The AT2R has been shown to be involved in apoptosis, particularly during embryogenesis. The aim of this study was to examine the A-->1675G transition polymorphism in intron 1 of the AT2R gene that is located on the X chromosome in patients with coarctation of the aorta (CoA) with and without Ullrich-Turner syndrome (UTS). Screening of DNA samples was performed with restriction fragment length polymorphism analysis. Ninety-seven patients with CoA, 28 girls with UTS, 10 girls with UTS and CoA, and 96 control individuals were studied. There was no significant difference in the distribution of A and G-genotypes in any of the patient groups compared to controls. An A-->1675G transition in the AT2R gene seems not to be involved in the pathogenesis of aortic coarctation.
OBJECTIVE: To investigate the clinical features of coarctation of aorta (CoA). METHODS: The clinical data of 96 pediatric patients with CoA, 60 male and 36 female, aged 3.7 months (7 - 12 years), were analyzed. RESULTS: The male to female ratio was 1.7:1. Infants aged less than 6 months accounted for 60% (57/96). The proportion of CoA in all hospitalized patients with congenital heart diseases admitted in this hospital was 0.5% (5/924) in 1996, increased every year, and reached 4.3% (15/330) in 2005. The coarctation was situated at the distal end of the left subclavian artery opposite to or near the ductus arteriosus in 93 cases, between the left common carotid artery and left subclavian artery in 2 cases, and at the opening of the left subclavian artery in 1 case. Thirteen patients (4%) suffered only from CoA, 47 patients were complicated with patent ductus arteriosus (PDA, 47/96, 49%) and/or ventricular septum defect (VSD, 47/96, 49%), 22 of the 96 patients (23%) complicated with both PDA and VSD. Eighty-nine cases were diagnosed by echocardiography, however, echocardiography failed to diagnose CoA in 7 cases (7.3%). Fifty-five patients underwent surgical repair and 2 of them died with a mortality of 3.8%. Seven patients with large VSD and severe pulmonary hypertension underwent two-stage repair. Immediate post-operative echocardiography showed satisfactory outcome. CONCLUSION: The morbidity of CoA among Chinese is similar to that among the Western population. Most of the coarctation is situated at the distal end of the left subclavian artery opposite to or near the ductus arteriosus, and most of the cases are complicated by PDA and/or VSD. Echocardiography is the first choice in the diagnosis of CoA; however, angiography is still necessary in some cases. Primary radical operation is indicated for infants with CoA, but older patients, especially those complicated with VSD and severe pulmonary hypertension, should undergo two-stage procedure.