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Background: Aortic aneurysm is common in patients with coarctation of aorta (COA), but it is unclear whether the risk of aortic aneurysms is due to COA or related to the presence of other risk factors such as bicuspid aortic valve (BAV) and hypertension., Objectives: The purpose of this study was to assess the relationship among COA, BAV, and thoracic aortic aneurysms., Methods: A total of 867 patients with COA (COA group) were matched 1:1:1 to 867 patients with isolated BAV (BAV group) and 867 patients without structural heart disease (SHD) (no-SHD group). The COA group was further subdivided into a COA+BAV subgroup (n = 304 [35%]), and COA with tricuspid aortic valve (TAV) (COA+TAV subgroup [n = 563 (65%)]). Aortic dimensions were assessed at baseline and at 3, 5, and 7 years., Results: Compared with the no-SHD group, the COA+BAV subgroup had larger aortic root diameter (37 mm [Q1-Q3: 30-43 mm] vs 32 mm [Q1-Q3: 27-35 mm]; P < 0.001) and mid ascending aorta dimeter (34 mm [Q1-Q3: 29-40 mm] vs 28 mm [Q1-Q3: 24-31 mm]; P = 0.008). Similarly, the BAV group had larger aortic root diameter (37 mm [Q1-Q3: 30-42 mm] vs 32 mm [Q1-Q3: 27-35 mm]; P < 0.001), and mid ascending aorta dimeter (35 mm [Q1-Q3: 30-40 mm] vs 28 mm [Q1-Q3: 24-31 mm]; P < 0.001). Compared with the COA+TAV subgroup, the COA+BAV subgroup and BAV group were associated with larger aortic root and mid ascending aorta diameter at baseline and follow-up. The risk of acute aortic complications was low in all groups., Conclusions: These findings suggest that BAV (and not COA) was associated with ascending thoracic aorta dimensions, and that patients with COA+TAV were not at a greater risk of developing ascending aortic aneurysms as compared with patients without SHD., Competing Interests: Funding Support and Author Disclosures The MACHD registry is supported by the Al-Bahar Research grant. Dr Egbe is supported by National Heart, Lung, and Blood Institute grants (R01 HL158517, R01 HL160761, and R01 HL162830). All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2024 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Objectives: This study sought to compare outcomes after surgical valvuloplasty and balloon dilation of the aortic valve in neonates and infants., Background: Surgical techniques of aortic valve repair have improved and there is today controversy on the best approach to treat neonatal congenital aortic valve stenosis., Methods: Retrospective review of data and follow-up of 123 consecutive neonates and infants (35 females, 88 males) undergoing intervention for congenital aortic stenosis., Results: From 1977 to 2009, 123 consecutive neonates (<30 days) and infants (31 days to 1 year) underwent relief of congenital aortic stenosis. Median age at procedure was 27 days (6 to 76 days). Twenty-year survival was 80 ± 7%. Fifty-four patients required a re-intervention and freedom from re-intervention was 55 ± 6% at 10 years and 40 ± 6% at 20 years. By multivariate analysis, having the relief of stenosis by balloon valvuloplasty and undergoing initial treatment as a neonate were predictive of re-intervention. Freedom from re-intervention at 5 years was 27% after balloon valvuloplasty versus 65% after surgery. At latest follow-up, an additional 16 patients had moderate or severe stenosis and 8 had regurgitation. Freedom from re-intervention or stenosis was 39 ± 5% at 15 years. By multivariate analysis, balloon valvuloplasty (p < 0.001) and treatment as a neonate (p = 0.003) were again predictive of stenosis or re-intervention. Thirty-five patients ultimately needed a valve replacement. Significant predictor of the requirement of valve replacement was unicuspid aortic valve (p < 0.001). Freedom from valve replacement was 55 ± 7% at 20 years., Conclusions: Surgical valvuloplasty remains the best approach to treat neonates and infants with congenital aortic stenosis. After surgery, a higher proportion of patients remain free of re-intervention than after interventional catheterization and the relief of their stenosis lasts longer., (Copyright © 2013 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Objectives: We sought to determine the prevalence and predisposing condition for aortic wall complications in adults with either repaired or non-repaired coarctation of the aorta., Background: Aortic wall complications may develop in adults with coarctation of the aorta, despite successful surgical repair in childhood., Methods: A total of 235 adults with coarctation (mean age 27 +/- 13 years) were retrospectively reviewed. Treatment had been performed by surgery in 181 patients (group I) or by balloon angioplasty or stenting in 28 patients (group II). No previous intervention had been carried out in 26 patients with mild coarctation at diagnosis (group III)., Results: Forty-four aortic wall complications were found in 37 patients (16%). There were no differences among the three groups with respect to total complications (15%, 18%, and 15%, respectively), ascending aortic aneurysms (9%, 11%, and 12%), or descending aortic aneurysms (4% in all three groups). Multivariate analysis did not show a significant relationship between previous repair, type of repair, age at repair, residual Doppler pressure gradient, or systemic hypertension and the occurrence of aortic complications. Only aging (risk ratio [RR] 1.4 per decade of age, 95% confidence interval [CI] 1.1 to 1.8, p = 0.002) and bicuspid aortic valve (RR 3.2, 95% CI 1.3 to 7.5, p = 0.005) were significantly related to these complications., Conclusions: Aortic wall complications are frequent in adults with coarctation of the aorta beyond that attributable to associated hemodynamic derangement or previous repair. The only independent risk factors appear to be advanced age and bicuspid aortic valve.

Objectives: To evaluate the usefulness of balloon angioplasty for relief of native aortic coarctation, we reviewed our experience with this procedure, with special emphasis on follow-up results., Background: Controversy exists with regard to the role of balloon angioplasty in the treatment of native aortic coarctation., Methods: During an 8.7-year period ending September 1993, 67 neonates, infants and children underwent balloon angioplasty for native aortic coarctation. A retrospective review of this experience with emphasis on long-term follow-up forms the basis of this study., Results: Balloon angioplasty produced a reduction in the peak-to-peak coarctation gradient from 46 +/- 17 (mean +/- SD) to 11 +/- 9 mm Hg (p < 0.001). No patient required immediate surgical intervention. At intermediate-term follow-up (14 +/- 11 months), catheterization (58 patients) and blood pressure (2 patients) data revealed a residual gradient of 16 +/- 15 mm Hg (p > 0.1). When individual results were scrutinized, 15 (25%) of 60 had recoarctation, defined as peak gradient > 20 mm Hg. Recoarctation was higher (p < 0.01) in neonates (5 [83%] of 6) and infants (7 [39%] of 18) than in children (3 [8%] of 36), respectively. Two infants in our early experience had surgical resection with excellent results. Three patients had no discrete narrowing but had normal arm blood pressure and had no intervention. The remaining 10 patients had repeat balloon angioplasty with reduction in peak gradient from 52 +/- 13 to 9 +/= 8 mm Hg (p < 0.001). Reexamination 31 +/- 18 months after repeat angioplasty revealed a residual gradient of 3 to 19 mm Hg (mean 11 +/- 6). Three (5%) of 58 patients who underwent follow-up angiography developed an aneurysm. Detailed evaluation of the femoral artery performed in 51 (88%) of 58 patients at follow-up catheterization revealed patency of the femoral artery in 44 (86%) of 51 patients. Femoral artery occlusion, complete in three (6%) and partial in four (8%), was observed, but all had excellent collateral flow. Blood pressure, echocardiography-Doppler ultrasound and repeat angiographic or magnetic resonance imaging data 5 to 9 years after angioplasty revealed no new aneurysms and minimal (2%) late recoarctation., Conclusions: On the basis of these data, it is concluded that balloon angioplasty is safe and effective in the treatment of native aortic coarctation; significant incidence of recoarctation is seen in neonates and infants; repeat balloon angioplasty for recoarctation is feasible and effective; and the time has come to consider balloon angioplasty as a therapeutic procedure of choice for the treatment of native aortic coarctation.

Objectives: The purpose of this study was to test observations that might aid prenatal prediction of the presence of coarctation of the aorta in newborn infants with and without other forms of heart disease., Background: Previous reports have suggested that abnormal growth of the aortic arch in utero may be identifiable as a marker for the diagnosis of coarctation., Methods: We reviewed the prenatal echocardiograms and postnatal outcome of 20 infants (gestational age at initial study 18 to 36 weeks) with coarctation of the aorta established postnatally, to identify echocardiographic findings that would most facilitate the prenatal diagnosis of coarctation. Associated cardiac lesions included double-inlet left ventricle anatomy (n = 5), double-outlet right ventricle (n = 4), abnormal aortic valve (n = 5), unbalanced atrioventricular canal (n = 3), and membranous ventricular septal defect (n = 1). Chromosomal abnormalities included XO karyotype (n = 1), trisomy 18 (n = 1), and trisomy 21 (n = 1)., Results: Hypoplasia determined by measurement of the distal aortic arch was the most frequently observed finding among the fetuses with coarctation. In 12 of 15 fetuses with a well visualized transverse arch at initial prenatal study, the diameter of the transverse arch was < or = 3rd percentile for gestational age as compared with that in a normal group of fetuses. Ten of 10 fetuses with adequate images of the isthmus had isthmus hypoplasia at prenatal study with a diameter < or = 3rd percentile for gestational age. On serial study in six of seven, including three fetuses with normal distal arch measurements at initial study, the distal arch became progressively more hypoplastic for gestational age. In three there was no growth of the transverse arch or isthmus on serial study, and in three there was reversal of flow from antegrade to retrograde through the distal arch., Conclusions: In our study, quantitative hypoplasia of the isthmus and transverse arch was the most consistent observation and therefore the most definitive antenatal sign of postnatal coarctation. The potential for progression of distal arch hypoplasia necessitates serial study in fetuses with associated cardiac and noncardiac lesions.

The incidence of congenital heart disease in the children of 219 probands was determined. Each of these probands had one of four selected defects: atrial septal defect, coarctation of the aorta, aortic valve stenosis or complex dextrocardia. Of their children, 8.8% had substantial congenital cardiac defects. This is a much higher incidence than that reported in most comparable studies. The difference is highly significant statistically and therefore is likely to be genuine. The cause of the high recurrence is probably environmental.